Idiopathic Pulmonary Fibrosis

2 medicines

Idiopathic pulmonary fibrosis is a progressive lung disease where scar tissue builds up in the lungs, gradually reducing breathing capacity, slowed with antifibrotic medicines.

Esbriet

Pirfenidone

200/400mg

Esbriet is a covid 19 medication containing Pirfenidone, available as 200/400mg tablets.

from $2.18 / tablet View

Ofev

Nintedanib

100mg

Ofev is a covid 19 medication containing Nintedanib, available as 100mg capsules.

from $3.90 / capsule View

Key facts

  • Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease in which lung tissue becomes scarred and stiff, with no clear identified cause.
  • It mainly affects adults over 60 and is more common in men, presenting with a persistent dry cough and gradually worsening breathlessness on exertion.
  • No treatment reverses existing scarring, but antifibrotic medicines, nintedanib and pirfenidone, can slow further decline in lung function.
  • Sudden worsening of breathlessness, chest pain or a rapid fall in oxygen levels needs immediate hospital assessment.

How IPF presents and progresses

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.

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. Ongoing respiratory health follow-up is an important part of monitoring, particularly for patients who have also had significant lung infections.

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.

This page is educational and does not replace advice from a doctor or pharmacist who knows your health history.