Idiopathic pulmonary fibrosis

Also known as: IPF, Lung Scarring Disease, Progressive Lung Scarring, Scarred Lung Tissue Disease, Thickened Lung Tissue Disease, Chronic Lung Stiffening Disease

Last Updated: October 31, 2026

Key Points

Lungs become scarred and stiff
Breathing gets progressively harder
Cause is unknown (idiopathic)
Mainly affects older adults, rare under 50
No cure, but treatments slow progression

Overview

Idiopathic pulmonary fibrosis (IPF) is a serious lung condition where the lung tissue becomes scarred and stiff over time, making it harder to breathe. The term 'idiopathic' means the exact cause of this scarring is not known. It typically affects older individuals, usually between 70 and 75 years of age, and is uncommon in people under 50. In IPF, the tiny air sacs in the lungs, called alveoli, are damaged and develop scar tissue. This scarring prevents oxygen from properly entering the bloodstream, leading to shortness of breath and other symptoms that worsen gradually. Many people initially mistake these symptoms for normal aging or being out of shape. Currently, there is no treatment that can reverse or stop the lung scarring caused by IPF. However, various treatments are available to help manage symptoms, slow down the rate at which the condition progresses, and improve quality of life.

Signs and Symptoms

Shortness of breath
Persistent dry cough
Tiredness
Loss of appetite and weight loss
Rounded and swollen fingertips (clubbed fingers)

Things That Increase the Risk

Exposure to certain dusts (metal, wood)
Past viral infections
Family history of IPF
Acid reflux
Smoking

Diagnosis

Breathing (lung function) tests
Blood tests
Chest X-ray and CT scan
Lung biopsy (small tissue sample)

Treatment & Management

Stopping smoking
Healthy eating and regular exercise
Medicines to slow scarring (e.g., pirfenidone, nintedanib)
Oxygen therapy (via mask)
Pulmonary rehabilitation (breathing exercises and advice)
Lung transplant (in select cases)

Prevention

Avoid smoking
Manage acid reflux
Limit exposure to industrial dusts
Seek medical advice for persistent cough or breathlessness
Regular monitoring if there is a family history

Available Treatments

Explore our range of treatment options for Idiopathic pulmonary fibrosis: