Clinical features
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:
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