Michigan Lung and Critical Care MLCC sleep lab Grand Blanc Sleep Apnea Gregory Streff Mark Rittenger Pradeep Ramachandran

Interstitial Lung Disease

Interstitial lung disease (ILD) is an umbrella term for a group of lung disorders characterized by inflammation and eventual scarring of the deep lung tissues. Another term for these disorders is Pulmonary Fibrosis.

Causes, incidence & risk factors
ILDs are a group of diseases that are caused by inflammation and scarring of the air sacs (alveoli) in the lungs and their supporting structures (the interstitium). The scarring leads to decreased oxygen transfer from the lungs to the blood and an eventual decreased blood oxygen level. There are numerous causes of ILD and there is no uniformly accepted classification system; however, ILD can be broken down into two large groups:

  • Those that have no known cause (known as idiopathic ILD)
  • Those with identifiable causes – examples include:
    • Occupational exposure to things such as asbestos, silica, certain fungal spores, certain dusts or chemicals, etc.
    • Connective tissue disorders that can affect the lungs such as rheumatoid arthritis, scleroderma, lupus and sarcoidosis
    • Some medications can cause ILD such as bleomycin, amiodarone and methotrexate

The risk factors, genetic predispositions, and rate of ILD depend on the specific disease being considered. For example, sarcoidosis is relatively common in African-Americans, but idiopathic ILD is relatively rare in African-Americans.

Cigarette smoking has been shown to increase the risk of developing some forms of ILD and to increase the severity of all forms of ILD.


  • Shortness of breath at rest or with exertion
  • Decreased activity tolerance
  • Cough
  • Fatigue


  • Chest X-ray
  • High-resolution CT scan
  • Pulmonary function tests
  • Arterial blood gases
  • Lab tests to look for connective tissue diseases
  • Bronchoscopy
  • Lung biopsy

Treatment for ILD depends on the underlying cause. Most often, anti-inflammatory drugs such as corticosteroids or immune-suppressing drugs are used. If the underlying cause is not able to be determined (idiopathic) or does not have a specific treatment option available, then supportive treatment is indicated. Oxygen therapy is prescribed for those with low blood oxygen levels. For some people, pulmonary rehabilitation may be helpful. For those who smoke, smoking cessation programs should be incorporated into their treatment plan.

For those with severe ILD that is not responding to treatment, lung transplantation may be an option.



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