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:

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