Interstitial lung disease is a group of conditions that cause scarring in your lungs. The scarring often leads to breathing problems, which can range from mild to severe.

Interstitial lung disease includes over 200 different conditions that cause inflammation and scarring around the balloon-like air sacs in your lungs, called the alveoli. Oxygen travels through the alveoli into your bloodstream. When they are scarred, these sacs do not expand well. As a result, less oxygen enters your blood.

Other parts of your lungs can be affected too, such as the airways, lung lining, and blood vessels.

There are over 200 different types of interstitial lung disease. Some of these include:

  • Asbestosis: inflammation and scarring in the lungs caused by breathing in asbestos fibers
  • Bronchiolitis obliterans: a condition that causes blockages in the smallest airways of the lungs, called bronchioles
  • Coal worker’s pneumoconiosis: a lung condition caused by exposure to coal dust (also called black lung disease)
  • Chronic silicosis: a lung disease caused by breathing in the mineral silica
  • Connective tissue-related pulmonary fibrosis: a lung disease that affects some people with connective tissue diseases, such as scleroderma or Sjögren syndrome
  • Desquamative interstitial pneumonitis: a condition that causes lung inflammation and is more common in people who smoke
  • Familial pulmonary fibrosis: a buildup of scar tissue in the lungs that affects two or more members of the same family
  • Hypersensitivity pneumonitis: inflammation of the alveoli caused by breathing in allergens or other irritants
  • Idiopathic pulmonary fibrosis: a disease of unknown cause in which scar tissue develops throughout the lung tissue
  • Sarcoidosis: a disease that causes small clumps of inflammatory cells to form in organs such as the lungs and lymph nodes

When you have interstitial lung disease, lung damage, irritation, and lack of oxygen can cause a variety of symptoms. These include:

Symptoms can range in severity and may be similar to those associated with other respiratory conditions or health problems.

See your doctor if you notice shortness of breath or other unusual symptoms. If your symptoms are caused by interstitial lung disease, you can start treatments to manage the inflammation and scarring.

Many times, doctors can’t find the cause of interstitial lung disease. In these cases, the condition is called idiopathic interstitial lung disease.

Other causes of interstitial lung disease include medical conditions, the use of some medications, or exposure to toxic substances that damage your lungs. These causes of interstitial lung disease are grouped into three main categories:

Autoimmune conditions

Your body’s immune system attacks and damages the lungs and other organs in these conditions:

  • Dermatomyositis: an inflammatory disease that causes muscle weakness and skin rash
  • Lupus: a condition in which the immune system attacks many types of tissue, including the skin, joints, and other organs
  • Mixed connective tissue disease: a condition that has symptoms of several connective tissue diseases, including polymyositis, lupus, and scleroderma
  • Polymyositis: a condition that causes inflammation of the muscles
  • Vasculitis: inflammation and damage to blood vessels in the body
  • Rheumatoid arthritis: a disease in which the immune system attacks the joints, lungs, and other organs
  • Sarcoidosis: a disease involving clumps of inflammatory cells that form in various organs
  • Scleroderma: a group of diseases that cause the skin and connective tissue to thicken and tighten
  • Sjögren syndrome: a condition that causes joint pain, dry eyes, and dry mouth

Exposure to toxic substances or irritants

Exposure to the following substances at work or in the environment can cause lung scarring as well:

  • animal proteins, such as from farming, or raising birds
  • asbestos fibers
  • coal dust
  • grain dust
  • mold
  • silica dust
  • tobacco smoke

Medications

Some medications can damage the lungs, such as:

Treatments can’t reverse lung scarring if it has already occurred, but they may help to prevent further scarring or slow down disease progression.

If exposure to a toxic material or drug caused your interstitial lung disease, it’s important to avoid that substance.

Your doctor can prescribe a few different types of treatments to manage interstitial lung disease:

  • Supplemental oxygen can help you increase your oxygen levels, if they’re low.
  • Pulmonary rehabilitation may help improve your activity levels and ability to exercise.
  • Anti-inflammatory drugs, such as the steroid prednisone, can reduce inflammation in the lungs.
  • Immune-suppressing drugs may help stop immune system attacks that can damage the lungs.
  • Antifibrotic drugs such as pirfenidone (Esbriet) and nintedanib (Ovef) may prevent further scarring in the lungs. These drugs are both approved by the Food and Drug Administration (FDA) for the treatment of idiopathic pulmonary fibrosis.

If your condition is severe and other treatments don’t help, your doctor may recommend a lung transplant. Typically, this surgery is not recommended if you’re over the age of 75 years or you have certain serious health conditions.

Tips

While you’re undergoing treatment, here are a few things you can do to stay healthy:

  • If you smoke, consider quitting: Smoking can damage your lungs even more.
  • Follow your doctor’s advice on exercise: Talk with your doctor about ways to stay active, which may include supports like oxygen therapy and pulmonary rehabilitation.
  • Discuss vaccines for pneumonia, whooping cough, flu, and COVID-19 with your doctor: Certain infections can worsen your lung symptoms. If you have interstitial lung disease, talk with your doctor about whether these vaccines are appropriate for you.
  • Keep up to date with medical care: Do your best to keep your medical appointments and follow your treatment plan.
  • Take care of your mental health, too: If you notice changes to your mental health, talk with your doctor, family, and friends.

Certain factors may increase your risk for interstitial lung disease. These include:

  • Genetics: A gene called MUC5B is associated with increased risk for idiopathic pulmonary fibrosis (IPF), the most common type of interstitial lung disease.
  • Smoking: Smoking raises your risk for interstitial lung disease.
  • Family history: If a close relative has interstitial lung disease, you’re more likely to develop it.
  • Sex: Females are more likely to develop a type of interstitial lung disease called lymphangioleiomyomatosis (LAM). A different type, IPF, is more common in males.
  • COVID-19 infection: Viral infections that affect the lungs, such as COVID-19, may increase your risk for interstitial lung disease.
  • Workplace and living conditions: Exposure to molds, dusts, and other irritants can damage your lungs over time. Jobs like construction and mining are associated with an increased risk for interstitial lung disease.

Complications

Interstitial lung disease can lead to complications such as:

Diagnosing interstitial lung disease early and following your treatment plan may help reduce your risk for complications.

Interstitial lung disease can vary from person to person. Sometimes it progresses slowly, and in other cases, it worsens quickly. Your symptoms can range from mild to severe.

Some interstitial lung diseases have a better prognosis than others. One of the most common types, called idiopathic pulmonary fibrosis, can have a limited outlook. The average survival for people with this type is currently 3 to 5 years. It can be longer with certain medications and depending on its course.

People with other types of interstitial lung disease, like sarcoidosis, can live much longer.

For some people with severe disease, a lung transplant can improve the chance of survival.

What is the life expectancy of a person with interstitial lung disease?

Life expectancy with interstitial lung disease can vary greatly. The most common form of interstitial lung disease, idiopathic pulmonary fibrosis (IPF), has a life expectancy of approximately 3 to 5 years. But other, less severe types can have much longer survival times.

With any type of interstitial lung disease, treatments can help maintain quality of life. In some cases, it’s possible to slow the progression of lung damage.

How serious is interstitial lung disease?

There are many different types of interstitial lung disease. Depending on the underlying cause, the condition can range from mild to life threatening.

Damage to your lungs can get worse over time. It’s important to talk with your doctor if you notice symptoms like shortness of breath or a dry cough that does not go away.


What are the first signs of interstitial lung disease?

Usually, the first sign of interstitial lung disease is shortness of breath that comes on gradually over time. In other cases, the first sign is a cough.


What is the most common cause of interstitial lung disease?

Idiopathic pulmonary fibrosis (IPF) is the most common type of interstitial lung disease. “Idiopathic” means the underlying cause of the condition is not known.

But genetics appear to play a role in the development of IPF. About 30% of the risk of developing IPF is related to a gene called MUC5B.

Other causes of interstitial lung disease include certain autoimmune conditions and dust, mold, or irritants in your environment.

Scarring in your lungs can’t be reversed. Still, treatments may be able to slow lung damage and help you breathe more easily. Lung transplant remains an option for those who do not respond to other medical treatments.