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Clinical features

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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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.

Reference:

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

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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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