Bronchiolitis Obliterans Organizing Pneumonia

Interstitial Lung Disease

Ambulatory Care,Respiratory

Written by the doctors and editors at UpToDate
What is interstitial lung disease? — Interstitial lung disease is the medical term for a group of lung disorders that cause similar symptoms and changes in the lungs. Interstitial lung disease causes inflammation and sometimes scarring in the lungs. This makes it hard for oxygen to get from the air into the lungs, which can make it hard to breathe.
There are many causes of interstitial lung disease, including:
Autoimmune diseases – These are diseases in which the body's infection-fighting system attacks healthy tissue instead of infections.
Other medical conditions (such as cancer or sarcoidosis)
Certain medicines
Harmful substances that are breathed into the lungs over a long period of time – These include cigarette smoke, e-cigarette or "vaping" products, mold, chemicals, or dust from asbestos, silica, metals, or coal.
Radiation (high doses of X-rays)
Certain lung infections
In some cases, doctors don't know what is causing a person's interstitial lung disease. For example, the cause of "idiopathic pulmonary fibrosis" is not known.
What are the symptoms of interstitial lung disease? — The most common symptoms of interstitial lung disease are:
Trouble breathing or feeling short of breath, especially with activity or exercise
A dry cough that lasts for a long time
Early on, some people have no symptoms. Their doctor or nurse finds out they have interstitial lung disease when they have a chest X-ray or breathing tests done for another reason.
Some medical conditions that cause interstitial lung disease also cause symptoms in other parts of the body. For example, some autoimmune disorders also cause skin or joint symptoms.
Is there a test for interstitial lung disease? — Yes. If you have interstitial lung disease, your doctor or nurse will want to know what's causing it. They will ask about your medicines, past medical conditions, whether you have been around harmful substances you might have breathed in, and whether you have any other symptoms that might be related.
Your doctor or nurse will also order tests. The tests that are usually done to figure out the cause of interstitial lung disease include the following:
Blood tests
A chest X-ray
A CT scan of the chest – A CT scan is an imaging test that creates pictures of the inside of the body.
An electrocardiogram (also called an "ECG") – This test measures the electrical activity in the heart. It can show whether people also have a heart problem.
Breathing tests, also called "pulmonary function tests" – These tests measure how well the lungs are working.
In some cases, people might also have:
An echocardiogram – This test uses sound waves to create pictures of your heart. It can show if your heart is pumping normally.
Bronchoscopy – This is a procedure in which a doctor uses a thin tube (called a "bronchoscope") to look inside the lungs and get a sample of fluid.
A lung biopsy – For a lung biopsy, a doctor does surgery or a procedure to take a small sample of tissue from the lung. Then another doctor looks at the sample under a microscope. Not everyone will need a lung biopsy. It is done only if all of the other tests don't show what's causing the interstitial lung disease.
How is interstitial lung disease treated? — Treatment depends on the cause of your interstitial lung disease and how severe your symptoms are.
If your lung disease is caused by an infection, your doctor might prescribe medicines to kill the germs causing the infection.
If your lung disease is caused by a medicine you take, your doctor will recommend that you stop taking that medicine.
Many people with interstitial lung disease are treated with:
Extra oxygen (that you carry around with you in a tank)
Steroid medicines to reduce inflammation, such as prednisone – These are not the same as the steroids some athletes take illegally to build muscle. Some interstitial lung diseases are also treated with stronger medicines to reduce inflammation.
Medicines to slow lung damage, such as pirfenidone (brand name: Esbriet) and nintedanib (brand name: Ofev).
Pulmonary rehab – In pulmonary rehab, people learn exercises and ways to breathe that can help with symptoms.
In some cases, people with severe interstitial lung disease might be treated with a lung transplant. This is surgery to replace one or both diseased lungs with healthy lungs.
All topics are updated as new evidence becomes available and our peer review process is complete.
This topic retrieved from UpToDate on: Mar 30, 2020.
Topic 86972 Version 9.0
Release: 28.2.2 - C28.105
© 2020 UpToDate, Inc. and/or its affiliates. All rights reserved.

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