DR MUHAMMED ASLAMMBBS MD PULMONARY MEDICINEfacebookcommedicalppt

Interstitial Lung Disease(ILD) or

Diffuse Parenchymal Lung Disease (DPLD)

OUTLINE

bull Introduction

bull Classification

bull Epidemiology

bull Pathogenesis

bull Clinical features

bull Investigation

bull Treatment

INTRODUCTION

bull Interstitial Lung Disease refers to a broad range of conditions that have common clinical physiological and radiological features

bull ILD is not one disease but several diseases that do not necessarily share a common histopathological or pathophysiological basis

INTRODUCTIONbull By strict definition Interstitial lung disease involves

abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip

INTRODUCTION

bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment

bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term

CLASSIFICATION OF DPLD

bull DPLD Two large groups

(1)Idiopathic DPLD ( no known cause )

(2) Those with identifiable cause or occurs secondary to other diseases

IDIOPATHIC ILD

1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis

obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease

(RBILD)

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

OUTLINE

bull Introduction

bull Classification

bull Epidemiology

bull Pathogenesis

bull Clinical features

bull Investigation

bull Treatment

INTRODUCTION

bull Interstitial Lung Disease refers to a broad range of conditions that have common clinical physiological and radiological features

bull ILD is not one disease but several diseases that do not necessarily share a common histopathological or pathophysiological basis

INTRODUCTIONbull By strict definition Interstitial lung disease involves

abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip

INTRODUCTION

bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment

bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term

CLASSIFICATION OF DPLD

bull DPLD Two large groups

(1)Idiopathic DPLD ( no known cause )

(2) Those with identifiable cause or occurs secondary to other diseases

IDIOPATHIC ILD

1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis

obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease

(RBILD)

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

INTRODUCTION

bull Interstitial Lung Disease refers to a broad range of conditions that have common clinical physiological and radiological features

bull ILD is not one disease but several diseases that do not necessarily share a common histopathological or pathophysiological basis

INTRODUCTIONbull By strict definition Interstitial lung disease involves

abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip

INTRODUCTION

bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment

bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term

CLASSIFICATION OF DPLD

bull DPLD Two large groups

(1)Idiopathic DPLD ( no known cause )

(2) Those with identifiable cause or occurs secondary to other diseases

IDIOPATHIC ILD

1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis

obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease

(RBILD)

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

INTRODUCTIONbull By strict definition Interstitial lung disease involves

abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip

INTRODUCTION

bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment

bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term

CLASSIFICATION OF DPLD

bull DPLD Two large groups

(1)Idiopathic DPLD ( no known cause )

(2) Those with identifiable cause or occurs secondary to other diseases

IDIOPATHIC ILD

1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis

obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease

(RBILD)

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

INTRODUCTION

bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment

bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term

CLASSIFICATION OF DPLD

bull DPLD Two large groups

(1)Idiopathic DPLD ( no known cause )

(2) Those with identifiable cause or occurs secondary to other diseases

IDIOPATHIC ILD

1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis

obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease

(RBILD)

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

CLASSIFICATION OF DPLD

bull DPLD Two large groups

(1)Idiopathic DPLD ( no known cause )

(2) Those with identifiable cause or occurs secondary to other diseases

IDIOPATHIC ILD

1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis

obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease

(RBILD)

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

IDIOPATHIC ILD

1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis

obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease

(RBILD)

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

THOSE WITH IDENTIFIABLE CAUSE

1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma

2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

CLASSIFICATION

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

EPIDEMIOLOGY

bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is

estimated to be 10 times that of clinical recognized disease

bull IPF is the most common form representing at least 30 percent of the incident cases

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

IPF - PATHOGENESIS VIDEO

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

NORMAL DPLD

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough

Pleuritic chest pain Wheeing Hemoptysis

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS

bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis

bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

ONSET OF SYMPTOMS

1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis

2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes

3 Chronic presentation (months to years) ndash eg IPF

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

ILD-HISTORY

bullSmokingbullMedication history- Amiodorane Methotrexate

bullOccupational historybullEnvironmental exposure history

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic

industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and

theuse of protective devices should be elicited

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

ENVIRONMENTAL EXPOSURE HISTORY

bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

ILD-SIGNS

bull Crackles - Dry velcro end inspiratory predominantly bibasilar

bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies

bull Cor pulmonale featuresbull Clubbing ndash IPF

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

EXTRA PULMONARY -SIGNS

bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum

bull Subcutaneous nodules bull Proximal muscle

weakness bull Arthritis

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

INVESTIGATIONS

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

CHEST X RAY

bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)

bull HRCT is more sensitivebull Combinations of ground

glass changes reticulonodular shadowing honeycomb cysts and traction

bronchiectasis

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

PULMONARY FUNCTION TESTING

bull Objective assessment of Resp Symptoms

bull Grading the severity

bull Monitoring the response to therapy

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

PULMONARY FUNCTION TESTING

bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC

bull Reduced diffusing capacity (DLCO)

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

LABORATORY INVESTIGATIONS

bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis

bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease

activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

OTHER INVESTIGATIONS

bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special

stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG

BIOPSY

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents

(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma

(3) to palliate the manifestations of these diseases

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

CORTICOSTERIODS

bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months

to a maintenance dose

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

IMMUNO SUPRISSIVE AGENTS

Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

N-ACETYL CYSTEIN

bullAntioxidant

bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

PIRFENIDONE

bullAntifibroticbullReduces acute exacerbations and reduction in FVC

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

OXYGEN THERAPY

bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g

bull Improves exerc i se to le rance

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

CONCLUSION

bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent

further destruction of the pulmonary parenchyma

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36

Thank you

• Outline
• introduction
• Introduction
• Introduction (2)
• Classification of dpld
• Idiopathic ILD
• Those with identifiable cause
• classification
• Epidemiology
• Ipf - PATHOgenesis VIDEO
• Slide 12
• Respiratory SYMPTOMS
• Non Respiratory symptoms associated with different dplds
• ONSET OF SYMPTOMS
• ILD-HISTORY
• Occupational history
• Environmental exposure history
• ILD-SIGNS
• Extra Pulmonary -SIGNS
• INVESTIGATIONS
• Chest X Ray
• High-resolution Computed topography (HRCT)
• PULMONARY FUNCTION TESTING
• PULMONARY FUNCTION TESTING (2)
• Laboratory investigations
• OTHER INVESTIGATIONS
• Slide 28
• MANEGEMENT
• CORTICOSTERIODS
• IMMUNO SUPRISSIVE AGENTS
• N-acetyl cystein
• Pirfenidone
• Oxygen therapy
• Conclusion
• Slide 36