Idiopathic pulmonary fibrosis is often diagnosed late, after months of being treated as another cause of breathlessness, and delay costs lung function that antifibrotic therapy might have preserved.
Suspect it in an older adult, more often a man and usually with a smoking history, who has progressive exertional breathlessness and a persistent dry cough, with fine bibasal inspiratory crackles often described as Velcro-like, and finger clubbing in a proportion. The key reflex is to get high-resolution CT rather than settling for a chest radiograph, and to refer to an interstitial lung disease service, where a multidisciplinary diagnosis and early antifibrotic treatment can be considered.
The habit worth keeping is to think of IPF when bibasal Velcro crackles accompany unexplained progressive breathlessness, and to image and refer before attributing symptoms to age, heart failure or COPD.
- Suspect IPF with progressive exertional breathlessness and a persistent dry cough in an older adult.
- Listen for fine, Velcro-like bibasal inspiratory crackles, and look for finger clubbing.
- Order high-resolution CT rather than relying on a chest radiograph.
- Refer to an interstitial lung disease service for multidisciplinary diagnosis.
- Early diagnosis allows antifibrotic therapy before more lung function is lost.
Why it matters
IPF is frequently diagnosed late, and the delay costs lung function that early antifibrotic treatment could protect.
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