The end-stage of many different pulmonary diseases. The lungs become fibrosed (thickened, stiff and scarred) and lack their usual elasticity and compliance. It can be localised (e.g. one lobe) or affect the whole lung.
Epidemiology of pulmonary fibrosis
Idiopathic disease approximately 10 per 100,000 per year
Increases with age: peak incidence 50-70 year-olds
Exclusion of other causes e.g. drug toxicities, connective tissue disease
Abnormal pulmonary function studies (evidence of restriction)
Bibasilar reticular abnormalities with minimal ground glass opacities on HRCT
No evidence of alternative diagnosis on transbronchial biopsy or BAL
Minor Criteria
Age > 50
Insidious onset of otherwise unexplained dyspnoea on exertion
Duration > 3 months
Bibasilar inspiratory crackles
Usual interstitial pneumonia (UIP) is defined pathologically by a heterogenous appearance with alternating areas of normal lung, interstitial inflammation, fibrosis and honeycomb change affecting the peripheral supleural parenchyma most severely.
Management of pulmonary fibrosis
Medical
Prednisolone
Azathioprine and cyclophosphamide
Pirfenidone
Licensed for use in patient with UIP with FEV 50-80% predicted (NICE guidelines)
Home oxygen
Pulmonary rehabilitation
Lung transplant
Indications
Age under 60 with expectancy under 18 months
Match for ABO, not Rh
Double transplant preferred
Donor
Good cardiac and lung function, age under 40
Chest diameter slightly less than recipient
Complications of pulmonary fibrosis
Respiratory failure
Pulmonary hypertension
Pneumothorax
Increased risk of bronchogenic cancer
Prognosis of pulmonary fibrosis
Variable and depends on type
Highly cellular with ground glass infiltrate: good response to immunosuppression
80% 5 year survival
Honey-combing on CT and no response to immunosuppression
