Fibrotic lung disease

Question 1

Fibrosis affecting upper zones

Answer 1

Hypersensitivity pneumonitis

Coal worker’s pneumoconiosis

Silicosis

Sarcoidosis

Ankylosing spondylitis

Histiocytosis

Tuberculosis

Radiation induced pulmonary fibrosis

Question 2

Fibrosis affecting the lower zones

Answer 2

Idiopathic pulmonary fibrosis

Most connective tissue disorders

Drug induced: amiodarone, bleomycine, methotrexate

Asbestosis

Question 3

Features of idiopathic lung fibrosis

Answer 3

Progressive exertional dyspnoea

Bibasal fine end-inspiratory crepitations

Dry cough

Clubbing

Question 4

Diagnosis

Answer 4

Restrictive spirometry (FEV1 normal/ decreased, FVC decreased, FEV1:FVC increased)

Reduced transfer factor (TLCO)

ANA positive in 30%, rheumatoid factor positive in 10%

Question 5

Imaging

Answer 5

Bilateral interstitial shadowing ‘ground glass’ on chest xray

CT investigation of choice and required to make diagnosis of IPF

Question 6

Management of idiopathic pulmonary fibrosis

Answer 6

Pulmonary rehabilitation

Perfenidone may be useful in selected patients

Supplementary oxygen and lung transplant

Question 7

Drugs causing lung fibrosis

Answer 7

Amiodarone

Cytotoxic agents: busulphan, bleomycin

Anti-rheumatoid drugs: methotrexate, sulfasalazine

Nitrofurantoin

Ergot-derived dopamine receptor agonists (bromocriptine, cabergoline, pergolide)