Is interstitial lung disease the same as pulmonary fibrosis?

Interstitial lung disease (ILD) and pulmonary fibrosis are not the same thing. Pulmonary fibrosis is one type of ILD.

What is interstitial lung disease or ILD?

The interstitium is the network of tissue around the air sacs in your lungs, where oxygen and other gases enter and leave your bloodstream.

ILDs affect this network of tissue. There are many types of interstitial lung diseases. Some ILDs cause lung scarring (fibrosis), such as idiopathic pulmonary fibrosis and asbestosis. Other ILDs cause inflammation (the body’s reaction to injury) but not lung scarring.

Some ILDs can cause both inflammation and lung scarring, including sarcoidosis and hypersensitivity pneumonitis.

The distinction is important because scarring (fibrosis) is usually permanent and may get worse, whereas inflammation can improve with treatment.

What is pulmonary fibrosis?

Pulmonary fibrosis describes a group of interstitial lung diseases (ILDs) where there is lung scarred. Because of the scarring, the lungs become progressively smaller and stiffer. This makes it hard to breathe, and eventually reduces the oxygen levels in the blood.

Pulmonary fibrosis symptoms include:

·       Shortness of breath

·       Cough

·       Finger clubbing (fingertips that are rounded and swollen)

·       Tiredness and fatigue

The treatment for pulmonary fibrosis depends on the condition you have. If you have Idiopathic Pulmonary Fibrosis, there are specific drug treatments. There are also treatments for other kinds of pulmonary fibrosis.

The outlook for pulmonary fibrosis depends on your condition, your general health and when you were diagnosed. In some people, lung scarring progresses quickly, while in others, it progresses slowly. There is research being conducted to try and understand why scarring occurs more quickly in some individuals and not in others.

ILD conditions

The most common ILDs cause pulmonary fibrosis. Pulmonary fibrosis conditions include:

Going to the ILD service

Many people with pulmonary fibrosis are treated by a specialist ILD service at a hospital or health centre or at their local District General Hospital. There are 23 specialist ILD services in the United Kingdom. Find a specialist ILD centre near you.

ILD services and Action for Pulmonary Fibrosis often run support groups for patients, offering information, emotional support and signposting advice.

There are Pulmonary Fibrosis support groups and networks available across the UK. Support groups provide emotional support, information, practical guidance and signposting advice for anyone living with and affected by pulmonary fibrosis.

Information provided by Action for Pulmonary Fibrosis (APF) is not a substitute for professional medical advice. It’s intended as general information only. APF is not responsible for any errors or omissions or for any loss or damage suffered by users resulting from the information published on

Further information for carers:

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