Previously termed cryptogenic fibrosing alveolitis

A chronic lung condition characterised by progressive fibrosis of the intersitium of the lungs

Whilst there are many causes of lung fibrosis (e.g. medications, connective tissue disease, asbestos) the term IPF is reserved when no underlying cause exists.

Epidemiology:

• Typically in patients aged 50-70 yrs old
• Twice as common in men

PC

• Progressive exertional dyspnoea
• dry cough

On examination:

• dry cough
• clubbing

Investigations

• Spirometry - A restrictive picture: decreased FEV1 and FVC, FEV1:FVC ratio normal
• Impaired gas exchange - Reduced transfer factor (TLCO)

Labs:

• ANA positive in 30%, rheumatoid factor positive in 10% but this does not necessarily mean that the fibrosis is secondary to a connective tissue disease. Titres are usually low