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.

Medicines used to treat Idiopathic Pulmonary Fibrosis

Ofev

Nintedanib

100mg

Developed to target idiopathic pulmonary fibrosis to alleviate breathing difficulties.

From $3.90 / capsule View

Esbriet

Pirfenidone

200 · 400mg

Utilized to manage idiopathic pulmonary fibrosis to alleviate lung fibrosis progression.

From $2.18 / tablet View

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.