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Clinical features

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

The clinical features of cryptogenic fibrosing alveolitis include:

  • highest incidence in late middle age
  • preponderance of males (M:F = 2:1)
  • in the early stages there is panting dyspnoea and exhaustion on effort
  • as the disease progresses the dyspnoea may develop on trivial exercise
  • there may be a dry cough
  • in addition there may be symptoms due to hypoxia, cardiac failure, and bronchopulmonary infection
  • in the terminal stages of the disease there may be respiratory failure and pulmonary embolism.

Signs include:

  • dyspnoea and tachypnoea
  • in severe disease there may be cyanosis
  • clubbing of fingers
  • on auscultation there are fine crepitations which are marked at the end of inspiration ('velcro-like' crackles)
  • wheezes are rare.

A rapidly progressive course, resulting in death within a few months is termed the Hamman-Rich syndrome.


  1. NICE (May 2017). Idiopathic pulmonary fibrosis: the diagnosis and management of suspected idiopathic pulmonary fibrosis

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