Autoimmune Interstitial Lung Disease

Interstitial lung disease (ILD) refers to a broad category of conditions that produce inflammation and scarring in the lungs. Autoimmune ILD is caused specifically by autoimmune disorders, which involve the body’s own immune system attacking the lungs.

Key Facts

  • Interstitial lung disease (ILD) is a category of conditions that cause inflammation and scarring in the lungs. This can lead to difficulty breathing and, eventually, heart failure.
  • Autoimmune ILD is a specific type caused by autoimmune disorders such as lupus, rheumatoid arthritis, and more. While autoimmune disorders tend to affect women more than men, the exact prevalence of autoimmune ILD among men vs women is unknown.
  • Available treatments cannot reverse existing damage, but they can help slow or stop additional damage from occurring.


While there are many different causes for ILD in general, autoimmune ILD is caused specifically by autoimmune diseases. These are conditions in which the body’s immune system attacks its own organs. They can include the following:

  • Dermatomyositis
  • Lupus
  • Mixed connective tissue disease
  • Polymyositis
  • Rheumatoid arthritis
  • Sarcoidosis
  • Scleroderma
  • Sjogren's syndrome


Autoimmune ILD is a progressive disease, which means it gradually develops over a long period of time. Because of this, symptoms may emerge very slowly as the lungs become more damaged. The first symptoms may be the following:

  • Difficulty breathing
  • Dry cough
  • Shortness of breath

Over time, it may become difficult to breathe even while resting. If left untreated, symptoms may expand to include high blood pressure and other heart trouble as well.


Diagnosing autoimmune ILD begins by confirming the presence of an autoimmune condition. This can be done by taking a sample of blood and testing for certain proteins, antibodies, or other markers of an autoimmune disease.

Because ILD can resemble so many other respiratory infections, doctors may also use any of the following diagnostic methods:

  • Imaging Tests: These produce a detailed image of the lungs so that doctors can look for evidence of ILD, such as inflammation or scarring. The most common imaging test used for this purpose is a computerized tomography (CT) scan.
  • Pulmonary Function Tests: These measure the flow of oxygen from the lungs to the bloodstream. One example is spirometry, which tests the airflow of the lungs.
  • Tissue Biopsy: These tests take a small sample of tissue from the lungs and examine them for evidence of ILD. Biopsies can be done non-surgically using a device called a bronchoscope, which passes through the nose and into the lungs.


There are no treatments that can cure autoimmune ILD or reverse lung damage. But treatments are available that can help slow or even stop damage, as well as manage symptoms. Any of the following, as well as a combination, may be recommended:

  • Corticosteroids: These medications can help reduce any harmful swelling in the lungs. They are often given at the outset of symptoms, sometimes with immunosuppressants. 
  • Immunosuppressants: These medications help keep the body’s immune system from attacking the lungs and other organs. This can reduce or stop additional damage.
  • Oxygen Therapy: This just involves giving the patient additional oxygen through a mask or breathing tube. While it cannot reverse or slow damage, it can help make it easier to breathe. Portable oxygen is an option for those who need therapy at home. 
  • Pulmonary Rehabilitation: These programs involve a mixture of diet, exercise, and breathing techniques so that patients can better manage their condition.
  • Lung Transplantation: As a last resort, a lung transplantation will replace the diseased lungs with healthy lungs from a deceased donor. This option is not available for everyone and will not necessarily cure the condition. Learn more about this treatment.


Because there is no cure, autoimmune ILD is a lifelong condition. Although scarring or damage to the lungs cannot be reversed, additional damage can be reduced with proper treatment. This means that the prognosis for those with autoimmune ILD depends on the severity of the existing lung damage, when treatment begins, and how closely the patient follows their treatment plan.

Most people with little to moderate lung damage who begin proper treatment will be able to lead full and normal lives. It will likely be necessary to do regular breathing exercises, make lifestyle changes, and regularly take medication, but these patients can expect to live for a long time.

Patients who have more severe lung damage may have complications that give them a more limited outlook — sometimes only several years. In these cases, a lung transplantation may be the best option. 

Next Steps

Autoimmune ILD is a complex and serious condition that requires consistent care and expert management. Our multidisciplinary team is ready to create a personalized treatment plan for you that helps slow and stop any additional lung damage and gives you the techniques you need to better manage your health. In the most severe cases, our surgeons lead one of the country’s most experienced and successful lung transplantation teams.

If you need help for a lung or chest issue, we’re here for you. Call (212) 305-3408 for existing patients, (212) 304-7535 for new patients, or request an appointment online to get started today.

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