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Disease Profile

Interstitial lung disease

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

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US Estimated

Europe Estimated

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

ILD

Summary

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

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • Mayo Clinic has an information page on Interstitial lung disease.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • The Merck Manual provides information on this condition for patients and caregivers.

        In-Depth Information

        • The Merck Manual for health care professionals provides information on Interstitial lung disease.
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

          References

          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.