Pulmonary Fibrosis and Gradual Onset Breathlessness

Dr. Tim SutherlandConsultant with a specialist interest in ILD

• How do patients with pulmonary fibrosis present?• When do I refer?• What are the common causes of pulmonary fibrosis?• How do we manage patients with pulmonary fibrosis?

Overview – through the three concerns of my friend

• Majority have progressive breathlessness over a period of time

• Other differentials include cardiac failure, COPD, obesity, deconditioning

• Associated medical conditions• Drugs (www.pneumotox.com)• Occupation• Environment• Smoking• Family history

Breathlessness and an approach to pulmonary fibrosis

• 81 year old gentleman• History of breathlessness and weight loss • Past medical history of vascular disease• Previous smoker• Crackles on auscultation and was clubbed• Spirometry shows a FVC of 86%

Recent cases

• How to interpret spirometry

First concern

• How to interpret spirometry

• Confirm the quality of the test• As a general rule 80-120% predicated is normal• Caution - treat generated reports with caution,

especially if they say the unexpected

First concern

• Much more common than restriction• Caused by a process that obstructs flow – generally in

larger airways• Typically causes reductions in FEV1 and FEV1/FVC

ratio• Causes of obstructive defects:

COPDAsthmaBronchiectasis/Cystic Fibrosis

Obstruction

• Restrictive defects are generally rare, esp in fibrosis• Caused by a process that reduces all lung volumes

- needs to be confirmed by a PFT lab• Causes:

(Intrinsic) - Interstitial lung diseases

(Extrinsic) - Kyphoscoliosis

- Ankylosing spondylitis

- Muscular dystrophies

Restriction

• Interpretation can only be:- Normal - Obstructive - Restrictive (rare)- Undetermined

Interpretation

• 47 year old gentleman• History of breathlessness over a year• No past medical history• Worked with aluminium, iron and steel• Previous smoker• Crackles on auscultation and was clubbed• Spirometry shows a FVC of 98%

Alternative presentation

• What do I do with this?

Second concern

• If in doubt, refer

Second concern

What causes pulmonary fibrosis?

Classification of Interstitial Lung Disease

Interstitial Lung Disease

OthersGranulomatous Disease

Idiopathic Interstitial Pneumonias

ILD ofknown cause

Chronic fibrosing

Smokingrelated

Acute/subacute

DrugsCTD

RA etc

IPFNSIP

RBILDDIP

COPAIP

LAMLCH etc

Classification of Interstitial Lung Disease

Interstitial Lung Disease

OthersGranulomatous Disease

Idiopathic Interstitial Pneumonias

ILD ofknown cause

Chronic fibrosing

Smokingrelated

Acute/subacute

DrugsCTD

RA etc

IPFNSIP

RBILDDIP

COPAIP

LAMLCH etc

• One of the most frequent diagnoses – approximately 30 cases per 100,000

• Used to be called CFA• No known cause• Tends to be older people, men more than women• No cure but there is treatment

Idiopathic Pulmonary Fibrosis

• Lung cancer survival • IPF survival

Thank god it’s not lung cancer Doctor!

• Lung cancer survival • IPF survival

Thank god it’s not lung cancer Doctor!

• Lung cancer survival • IPF survival

Thank god it’s not lung cancer Doctor!

• Lung cancer survival • IPF survival

• Main management is: - symptom control- oxygen assessment- pulmonary rehabilitation- planning for the future/managing

expectations- transplant?- newer medication

Thank god it’s not lung cancer Doctor!

• Antifibrotic agent – currently a red drug• Up to 9 capsules a day• Significant side effects – liver, sun-burn, GI• Requires LFT monitoring• We currently have about 110 patients on this drug

Pirfenidone

• Tyrosine kinase inhibitor – currently named patient only• 2 capsules a day• Fewer side effects• Requires LFT monitoring

Nintedanib

• If in doubt, call us or the specialist nurses• Control symptoms, check oxygen and discuss patient

expectations• Initiate future planning when able to

Third concern – how do I co-manage these patients?

• www.blf.org.uk• www.actionpulmonaryfibrosis.org

Any questions?