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Disease Profile

Interstitial lung disease



Age of onset

















Not applicable


Other names (AKA)



Interstitial lung diseases (ILDs) are a large group of disorders characterized by thickening of the walls between the alveoli, increased production and buildup of collagen, and pulmonary fibrosis (progressive scarring of lung tissue).[1] The scarring eventually affects the ability to breathe and get enough oxygen into the bloodstream. Symptoms typically include shortness of breath and a dry cough.[2]

There are numerous possible causes of ILD, including connective tissue disorders, autoimmune diseases (e.g. rheumatoid arthritis), occupational exposures (such as asbestos), infection, and many drugs.[1][2] Specific diseases leading to ILD may include sarcoidosis, pulmonary Langerhans cell histiocytosis, and eosinophilic pulmonary diseases.[1] The list of substances and conditions that can lead to ILD is long.[2] However, in many cases, the cause is not known.[2] Some cases of unknown cause that share similar characteristics are called idiopathic interstitial pneumonias.[1]

Lung scarring that occurs in ILD is permanent. However, some treatments may temporarily improve symptoms or slow the disease's progress. Treatment options may include various medications, oxygen therapy, pulmonary rehabilitation, and as a last resort, lung transplantation.[2]



Organizations Supporting this Disease

    Organizations Providing General Support

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      Where to Start

      • Mayo Clinic has an information page on Interstitial lung disease.

        In-Depth Information

        • The Merck Manual for health care professionals provides information on Interstitial lung disease.


          1. Lee J. Overview of Interstitial Lung Disease (Diffuse Parenchymal Lung Diseases). Merck Manual. April, 2016; https://www.merckmanuals.com/professional/pulmonary-disorders/interstitial-lung-diseases/overview-of-interstitial-lung-disease.
          2. Interstitial lung disease. Mayo Clinic. July 21, 2017; https://www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/home/ovc-20341378.