Idiopathic pulmonary fibrosis (IPF)
What is IPF?
IPF is a type of interstitial lung disease in which lung tissue becomes progressively scarred and stiff. “Idiopathic” means that no specific cause has been identified. It is not cancer or an infection. Progression varies between individuals.
Symptoms
Typical symptoms are increasing breathlessness during activity and a persistent dry cough. Fatigue and changes in the shape of the fingertips may occur. These symptoms can have other causes, so specialist assessment is important.
How is it diagnosed?
Your clinician reviews your medicines, exposures and medical history to look for other explanations for lung scarring. Investigation includes lung function and gas transfer, blood tests and a high-resolution CT scan. Diagnosis is agreed by a specialist multidisciplinary team. A biopsy is considered only when needed after discussing its likely benefit and risks.
How is it treated?
Antifibrotic medicines such as nintedanib or pirfenidone may slow progression in eligible patients, but do not remove existing scarring. The specialist team considers current NICE guidance, your lung function, other conditions, possible side effects and your preferences.
Care also includes pulmonary rehabilitation, stopping smoking, vaccinations, symptom relief and oxygen when assessment shows it is needed. Some people may be suitable for lung-transplant assessment. Support for breathlessness, cough and wellbeing can be offered alongside disease-directed treatment.
Follow-up and urgent advice
Regular reviews monitor symptoms, lung function and treatment effects. Seek prompt medical help for a sudden deterioration rather than assuming it is simply progression of fibrosis. Call 999 for severe breathing difficulty or sudden confusion.
Routine appointment contacts | About other interstitial lung diseases
Sources and further information
NICE CG163: IPF diagnosis and management
NICE TA864: nintedanib in IPF with preserved lung function
NHS: idiopathic pulmonary fibrosis
General information for adults; investigation and treatment are tailored to you.