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Idiopathic pulmonary fibrosis 

Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis

A.U. Wells

, A.G. Nicholson

, and N. Hirani

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date: 15 October 2021

The synonymous terms idiopathic pulmonary fibrosis (IPF) and cryptogenic fibrosing alveolitis (CFA) refer to a relentlessly progressive fibrotic lung disorder that is the underlying diagnosis in over one-half of patients presenting with typical clinical features of the ‘CFA clinical syndrome’ (see Chapter 18.11.1). Incidence is about 10 to 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....

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