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Idiopathic pulmonary fibrosis (IPF)

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Last updated: 16th Sep 2020
Published:14th Sep 2020
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease. IPF is more common in elderly patients and over time as healthy lung tissue is replaced by altered extracellular matrix (fibrosis) the alveolar architecture is destroyed, leading to decreased lung function and finally respiratory failure and death.

Although the cause of IPF is not clear, smoking, viral infections and previous family history of the condition are contributing factors. Patients with IPF often have chronic exertional dyspnoea, bibasilar lung crackles (‘Velcro’ crackles) and finger clubbing. Physicians should consider IPF in patients with these signs and a definitive diagnosis can be made based on characteristic imaging and histological patterns.

Treating IPF includes pharmacological and pulmonary rehabilitation approaches. Effective disease-modifying treatments are available which is why it is important to diagnose and treat IPF as early as possible. In extreme cases of IPF a lung transplant may be needed.

To find out how to recognise and treat idiopathic pulmonary fibrosis, visit our dedicated Learning Zone which contains further information and expert opinion on diagnosis and current IPF treatment guidelines.

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