Nice | Idiopathic pulmonary fibrosis

This guideline is mainly useful to us in terms of when to consider Idiopathic Pulmonary Fibrosis , but there was some information in here that I was not aware of that is important.

When to consider Idiopathic Pulmonary Fibrosis:
• Patients over age 45
• Persistent breathlessness on exertion
• Persistent cough, with or without sputum
• Bilateral inspiratory crackles
• Clubbing
• Normal spirometry (though there may be a restrictive, or less commonly obstructive) pattern

How to investigate
The above symptoms will prompt you to do a CXR, but fibrosis does not show up on CXR, so if you have a suspicion, refer the patient to respiratory as they are likely to need a CT.

What does diagnosis mean for the patient?
It has a poor prognosis, with a median survival of 3 years, though 20% live longer than 5 years.
• There is no conclusive evidence that any medication improves survival. Most treatment is therefore supportive.
• From our point of view, consider the psychological impact and palliative care aspects.

Louise Hudman

I'm a freelance GP locum in Winchester & Southampton.

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