interstitial lung diseases (ild)
TRANSCRIPT
8/12/2019 Interstitial Lung Diseases (ILD)
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Interstitial Lung Diseases
(ILD)
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Introduction
• Also known asDiffuse Parenchymal Lung Disease(DPLD).
• Heterogenous group of conditions causedby diffuse thickening of the alveolar walls
with inflammatory cells & exudate (ARDS),granulomas (Sarcoidosis), haemorrhage(Goodpasture’s syndrome) and / orfibrosis.
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• Restrictive ventilatory defect.
• Slowly progressive loss of alveolarcapillary exchange units which ultimately
will lead to impairment of pulmonary gastransfer & arterial hypoxemia.
• In many cases eventually progresses torespiratory failure , pulmonary HTN &cor pulmonale.
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Aetiology
1. Sarcoidosis.
2. Cryptogenic fibrosing alveolitis (CFA).
3. Exposure to organic dusts :Farmer’s lung.
4. Exposure to inorganic dusts : Asbestosis.
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5. Pulmonary eosinophilia .
6. Collagen vascular diseases :SS, SLE, RA, Vasculitis.
7. Exposure to irradiation & drugs .
8. Miscellaneous : Alveolar proteinosis, Histiocytosis .
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Conditions mimicking ILD
• Malignancies :Leukemia, lymphoma,Carcinomatosis lymphangitis,alveolar cell carcinoma, metastasis.
• Infections :Viral, miliary tuberculosis,histoplasmosis, P. carinii
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Diagnosis• History :
Occupational / pets / smoking / drugs /family history.Symptoms- general, respiratory,
extrapulmonary.• Physical signs :
General, pulmonary, extrapulmonary.• Investigations :
Blood, Radiology (CXR, HRCT), PFT,
Histology.
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CFA
• Also known as Idiopathic PulmonaryFibrosis (IPF).
• A common ILD of unknown cause.
• May be associated with systemicdiseases.
• About twice as common among cigarettesmokers than in non-smokers.
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• Progressively downhill course withdevelopment of respiratory failure & deathwithin 5 years of diagnosis.
• Rapidly progressive form :Hamman – Rich syndrome .
• Disease of late middle age;M:F= 2:1.
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Pathology
• Alveolitis :inflammatory cells in alveoli & interstitium.
• Fibrosis : variable involvement.- Initially patchy involvement,
- Late stage, lung is converted into cysticspaces of varying size.
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Clinical features• Symptoms :
Progressive exertional dyspnea, dry coughGeneral - malaise, fever, wt loss, myalgia,
arthralgia.• Signs :
Majority - finger clubbing, cyanosis,
tachycardia, tachycardia. Almost all- superficial fine end inspiratory
crepitations at lung bases.
Diaphragm may be raised.
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Investigations• Blood:
ANA & RF may be positive in low titers.ESR raised.
Hypergammaglobulinemia.• Arterial desaturation (hypoxemia withouthypercapnea) after exercise- may be anearly indication.
• PFT : Restrictive pattern- decreased TLC, VC, TV
- normal / increased FEV1/ VC.
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Pulse oximeter
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SPIROMETER
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• CXR : Loss of lung volumeEarly – ground glass appearance;
Late – reticulonodular pattern ; Advanced – multiple cystic spaces
(honeycomb)
• HRCT- more sensitive method todemonstrate early disease, but lacksspecificity.
• BAL : increased cells; PMNL predominant.
• Open lung biopsy : Definitive diagnosis.
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CFA- CXR
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CFA- CT scan
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Diagnosis
• Clinical• Investigations.
• Exclude- Pneumoconiosis.
- Collagen vascular disease.
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Treatment
A. Drug therapy :1. Corticosteroids
Prednisolone:1 mg/kg body wt. for 6-8 wksgradually tapered to maintenancedose (10-12.5 mg daily).
Advanced disease/ elderly- poor response .Subjective improvement in majorityObjective improvement in minority.
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• Second line drugs :1. Azathioprine- monitor blood count & LFT.2. Cyclophosphamide - monitor blood count.
Regimens :1. Azathioprine 100 mg daily +
Prednisolone 20mg OD on alternate days
2. Cyclophosphamide 2-3mg/ kg daily +Prednisolone
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• B. Supportive therapy : Oxygenof cor pulmonale.
• C. Single lung transplantation :For young pts with advanced disease.
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Prognosis
• Median survival time of untreated cases is4 years.
• In majority of pts the disease isprogressive, even in those who haveresponded to treatment.
• Approximately 50% survive for 5 years &
25% for 10 years.• Tenfold increase in the incidence ofbronchogenic carcinoma.
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SARCOIDOSIS
• It is a multisystem granulomatous diseaseof unknown etiology, which proceed eitherto resolution or to fibrosis.
• Lungs are usually involved.
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Prevalence & incidence
• Prevalent in West & Japan.
• Worldwide prevalence: 20 / 100000.
• Inversely related to TB.
• Blacks > Whites.
• Genetic- twins/ siblings.
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Aetiology
• Unknown.
• Immunological response to someunidentified antigen in geneticallysusceptible individual.
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Pathology
• Alveolitis – predominantly lymphocytic.• Non caseating granuloma.
• In 70% cases inflammatory changes stopearly & resolution occurs spontaneouslywithout sequelae;fibrosis occurs in 5-10% cases.
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Sarcoidosis- Histology
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Clinical features
• Asymptomatic: 20-30% cases.• Constitutional disturbances- fever,
malaise, fatigue, wt. loss, cough.
• Can affect virtually all organs & tissues ofthe body , except adrenal glands.• Lungs, eyes, skin & liver are most
frequently affected.• Alarming CXR findings, scanty physical
signs on auscultation of respiratorysystem.
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Sarcoidosis- skin lesions
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Sarcoid- nose
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LUPUS PERNIO
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LUPUS PERNIO
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Dactylitis
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Investigations
• Blood :WBC count – N/ Increased (lymphopenia)ESR- moderate raise.
S. calcium – increased in 20-40% cases.Hypergammaglobulinemia with reversal of A:G.S. ACE- elevated in pts with active disease;
useful in f/up.
• Tuberculin test : 60% show negative result.
Strongly positive reaction excludes sarcoidosis.
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• Biopsy- transbronchial lung / skin / LN.confirms the diagnosis.
• Kveim test : in cases where biopsy isdifficult / negative.
Intradermal injection of 0.1-0.2 ml of human
sarcoid tissue; positive test after 4-6 wks in theform of purplish red nodule at the site ofinjection.
• BAL : Increased lymphocytes (normal <10%)
• PFT : Restrictive pattern
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• CXR : Thoracic sarcoidosis.Stage I : Bilateral Hilar Lymphadenopathy
with or without right paratrachealadenopathy; may be associatedwith erythema nodosum,
arthralgia , low grade fever;
Stage II : Pulmonary infiltrates coexist(often diffuse) with adenopathy .Gradual onset of exertional
dyspnea, cough.
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STAGE III : Diffuse pulmonary infiltrates
without adenopathy;development of pulmonaryfibrosis (mid & upper zone).
Pts become breathless, hypoxic,ultimately develop cor pulmonale
• CT scan :early LN , parenchymal infiltration.
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Stage I
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SARCOIDOSIS- FIBROSIS
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Treatment
• Symptomatic ; spontaneous resolution inmost cases.• Corticosteroids
Prednisolone: 0.5-1 mg / kg single dailydose for 4 wks; graduallytapered to maintenance
dose of 7.5-10 mg daily.Indications : Acute/ severe form, CNS,
eye, vital organ involvement.
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Prognosis• Stage I : Spontaneous recovery within 1 yr
in majority.• Stage II : 50% resolve spontaneously within
2 yrs; 30-40% require steroid therapy;
small number develop progressivepulmonary fibrosis.
• Stage III: 30% show improvement with steroid
therapy; may eventually developpulmonary fibrosis & respiratory failure.
Terminally – Respiratory/ Renal/ Cardiac failure.
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