Interstitial lung disease (ILD) is a group of more than 200 lung illnesses. Most of them are chronic or long-lasting. In ILD, inflammation scars and damages your lungs.

ILD has many risk factors. Just because you have a risk factor is no guarantee that you will get this disease.

What Is ILD?

ILD is also called pulmonary fibrosis. In ILD, inflammation attacks and damages your interstitium, the connective tissue in and around your lungs, air sacs, and breathing passages. You develop tough, fibrous scar tissue in your lungs that may make it difficult to breathe.

Most people with ILD have a cough or shortness of breath. You may also have symptoms like fatigue, unexplained weight loss, chest pain, loss of appetite, fast or shallow breathing, or bleeding in your lungs.

Some people with ILD have very mild symptoms, while others may have severe breathing problems. ILD can be mild for a long time, then suddenly get much worse.

Because these symptoms could result from many other lung conditions, it’s important to see your doctor as soon as possible. They can give you some tests to diagnose ILD:

  • Physical exam
  • Blood and lung fluid tests
  • Lung function tests to measure how well you can breathe
  • High-resolution computed tomography (CT scan) or chest X-ray
  • Bronchoscopy, where a device called a bronchoscope is inserted into your lungs to look at the tissue
  • Biopsy in some cases to rule out other causes, like cancer

Risk Factors for ILD

There are some risk factors that make you more likely to develop ILD, so talk with your doctor if you have questions about your risk.

Disease complication. You’re at higher risk for ILD if you have any of these autoimmune diseases where inflammation can damage your lungs:

  • Scleroderma
  • Systemic sclerosis (SSc)
  • Rheumatoid arthritis (RA)
  • Sarcoidosis
  • Dermatomyositis
  • Polymyositis
  • Mixed connective tissue disease (MCTD)

If you have systemic sclerosis, you’re at very high risk to develop ILD too. Most people who have SSc develop ILD or other serious lung disease. If you have the diffuse form of SSc or you were recently diagnosed with SSc, you’re at very high risk for ILD.

Because it’s common to develop ILD if you already have SSc, your doctor may screen you for this lung disease every few months during the first 3 years after your SSc diagnosis. ILD usually develops early in the course of SSc.

Harmful air at work. People who breathe in harsh chemicals or air particles from coal dust, tobacco smoke, hairstyling chemicals, asbestos, hot tubs, grain dust, or bird or animal droppings can develop ILD. If you work in mining, farming, construction, or other jobs where you’re exposed to these particles in the air, you may be at risk for ILD.

Treatments for other diseases. Chemotherapy, radiation therapy, and certain medications like anti-inflammatory drugs, drugs for irregular heartbeat, and some antibiotics could damage your lungs and cause ILD.

Genetic diseases. People with inherited diseases like neurofibromatosis or Gaucher disease can be at higher risk for a genetic form of ILD.

Smoking. Tobacco smokers are at higher risk for ILD, especially if you have emphysema. Vapers may be at risk too: New evidence shows that vaping, or using e-cigarettes that don’t contain tobacco, can cause ILD.

GERD. Gastroesophageal reflux disease (GERD), or long-term acid reflux, increases your risk for ILD.

Age. ILD is more common among adults, but children can develop it.

Can You Prevent ILD?

While you can’t prevent many types of ILD, you can lower your risk in some situations:

  • If you work in a job where you’re exposed to harmful chemicals, wear a respirator mask to filter out harsh air particles.
  • Don’t smoke. Avoid secondhand smoke if you can.
  • Get vaccines to help prevent lung infections like flu, pneumonia, or COVID-19. There is some concern that severe lung infections could cause scarring and ILD. Some people who have had COVID-19 may have long-term lung problems and develop ILD.

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