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