Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is progressive scarring of lung tissue with no identified cause after appropriate investigation. It causes increasing exertional breathlessness and dry cough, but other interstitial lung diseases can look similar.
Key points
- Diagnosis uses specialist review of high-resolution imaging, exposures, medicines and autoimmune features.
- Antifibrotic medicines slow decline for some people but do not reverse established scar tissue.
- Sudden worsening breathlessness can reflect an acute exacerbation, infection, clot or collapsed lung and needs urgent care.
An antifibrotic listing cannot confirm IPF; diagnosis, lung function, liver health, interactions and treatment goals guide specialist prescribing.
How is IPF diagnosed and monitored?
High-resolution CT may show a characteristic pattern; uncertain cases can require multidisciplinary review or tissue sampling. Lung-function trends, walking oxygen levels and symptoms help track progression and oxygen needs.
What can treatment change?
Nintedanib and pirfenidone can slow loss of lung function but have different adverse effects and monitoring needs. Pulmonary rehabilitation, vaccination, oxygen assessment and transplant referral may also matter. See respiratory health for related context.
When to seek urgent care
Seek emergency care for sudden or rapidly worsening breathlessness, new chest pain, coughing blood, confusion, blue lips or a substantial fall in oxygen saturation from the usual level.