clinical features

Last reviewed 07/2019

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 in more than 50% of cases (1)
  • 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:

  • Prescribers' Journal (2000), 40 (2), 113-120.