Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive condition in which the lung tissue becomes scarred and stiff over time. “Idiopathic” means no clear cause has been identified. The scarring reduces the lungs’ ability to transfer oxygen into the bloodstream, so breathing becomes increasingly difficult. It affects adults, typically over 60, and is more common in men.
Key points
- A dry, persistent cough alongside breathlessness that slowly worsens with exertion is often the first sign, typically in men over 60.
- Nintedanib and pirfenidone cannot undo existing scarring but are shown in trials to slow the yearly decline in lung function.
- A sudden increase in breathlessness over days, new chest pain or a rapid drop in oxygen levels signals an acute exacerbation needing hospital care.
How IPF presents and progresses
The most common symptoms are a persistent dry cough and gradually worsening breathlessness on exertion. Many people notice they are slower on stairs or hills long before a diagnosis is made. As the disease progresses, even light activity can trigger shortness of breath. Some patients also develop clubbing of the fingertips. IPF typically follows a slow, steady decline, though some people experience acute episodes of rapid worsening.
Antifibrotic treatment
No treatment reverses existing scar tissue, but antifibrotic medicines can slow the rate of further scarring. Two agents are established for this purpose: nintedanib (a tyrosine kinase inhibitor) and pirfenidone (which has anti-inflammatory and antifibrotic properties). Both have been shown in clinical trials to reduce the annual decline in lung function. They are used long-term and are generally continued as tolerated. For patients who have also had Covid-related lung complications, post-Covid respiratory health follow-up is an important part of ongoing monitoring.
When to seek urgent review
Seek immediate medical attention if breathlessness worsens suddenly over days, chest pain develops, or there is a rapid fall in oxygen saturation. These signs can indicate an acute exacerbation, which requires prompt hospital assessment.