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Idiopathic pulmonary fibrosis 

Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis

P.L. Molyneaux

, A.G. Nicholson

, N. Hirani

, and A.U. Wells

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date: 25 February 2021

The synonymous terms idiopathic pulmonary fibrosis and cryptogenic fibrosing alveolitis refer to a relentlessly progressive fibrotic lung disorder. Incidence is about 5–15 per 100,000, men are more often affected than women, and it most commonly presents in the seventh and eighth decades. Aetiology remains uncertain. Typical presentation is with progressive exertional dyspnoea without wheeze, a non-productive cough, digital clubbing, and very fine end-inspiratory crackles. Central cyanosis and clinical evidence of pulmonary hypertension are late features. Diagnosis depends on careful exclusion of known causes of interstitial lung disease, followed by demonstration by radiological imaging or biopsy of the pathognomonic lesion of usual interstitial pneumonia. Two antifibrotic compounds, pirfenidone and nintedanib, have been proven to slow functional decline in idiopathic pulmonary fibrosis. Lung transplantation is appropriate in selected cases. Supportive therapy is central to the management of advanced disease. Five-year survival is 10–15%.

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