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Pathology
Inflammatory infiltrate and fibrosis of unknown cause
Aetiology
Commonest cause of interstitial lung disease (ILD)
Also known as cryptogenic fibrosing alveolitis (CFA)
Signs
Bilateral fine end-inspiratory crackles, clubbing
Symptoms
Dry cough and exertional dyspnoea
Investigations
Clinical, exclude other diagnoses and causes
Treatment
Steroids, azathioprine, cyclophosphamide, transplant
Prognosis
50% at 5 years
10% develop lung tumour
Key Facts
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Key References
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