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    Idiopathic Pulmonary Fibrosis

    Idiopathic Pulmonary Fibrosis

    National Organization for Rare Disorders, Inc.

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    It is possible that the main title of the report Idiopathic Pulmonary Fibrosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.


    • cryptogenic fibrosing alveolitis
    • idiopathic diffuse interstitial pulmonary fibrosis
    • IPF

    Disorder Subdivisions

    • None

    General Discussion

    Idiopathic pulmonary fibrosis (IPF) is a chronic lung disorder characterized by thickening, stiffening and scarring (fibrosis) of tissue within the lungs. Affected individuals develop shortness of breath and progressive lung disease. Ultimately, IPF results in life-threatening complications such as respiratory failure. The rate of progression can vary greatly from one person to another. Over years, most individuals experience increasing respiratory symptoms, progressive scarring of the lungs and a gradual decline in lung function. Less often, affected individuals have mild scarring within the lungs and little to no change in the disease for many years. In some cases, the disorder can progress rapidly (acutely), causing life-threatening complications within several years of diagnosis. The term 'idiopathic' means that the underlying cause of the disorder is unknown or unproven. Although there is no cure for IPF, various different treatments are available to manage the disorder and several newer therapeutic options are being studied. Ultimately, some affected individuals will require a lung transplant.

    IPF is classified as a form of idiopathic interstitial pneumonia, which is a group of lung diseases that damage the lungs in a similar manner and occur due to unknown causes. This group of disorders is also known as diffuse parenchymal lung diseases. Collectively, these disorders are classified under the broader umbrella term, interstitial lung diseases (ILDs). ILDs a large group of disorders (more than 200) characterized by progressive scarring of the lungs. IPF is the most common form.


    American Autoimmune Related Diseases Association, Inc.
    22100 Gratiot Ave.
    Eastpointe, MI 48021
    Tel: (586)776-3900
    Fax: (586)776-3903
    Tel: (800)598-4668
    Email: aarda@aarda.org
    Internet: http://www.aarda.org/

    American Lung Association
    1301 Pennsylvania Ave NW
    Suite 800
    Washington, DC 20004
    Tel: (202)785-3355
    Fax: (202)452-1805
    Tel: (800)586-4872
    Email: info@lungusa.org
    Internet: http://www.lungusa.org

    NIH/National Heart, Lung and Blood Institute
    P.O. Box 30105
    Bethesda, MD 20892-0105
    Tel: (301)592-8573
    Fax: (301)251-1223
    Email: nhlbiinfo@rover.nhlbi.nih.gov
    Internet: http://www.nhlbi.nih.gov/

    Second Wind Lung Transplant Association, Inc.
    P.O. Box 1657
    Wimberley, TX 78676-1657
    Tel: (512)847-9303
    Tel: (888)855-9463
    Email: barlows9303@gmail.com
    Internet: http://www.2ndwind.org

    Coalition for Pulmonary Fibrosis
    10866 W Washington Blvd Ste 343
    Culver City, CA 90232
    Tel: (888)222-8541
    Fax: (408)266-3289
    Tel: (888)222-8541
    Email: info@coalitionforpf.org
    Internet: http://www.coalitionforpf.org

    Pulmonary Fibrosis Foundation
    230 East Ohio Street
    Suite 304
    Chicago, IL 60611-3201
    Fax: (866)587-9158
    Tel: (888)733-6741
    Email: info@pulmonaryfibrosis.org
    Internet: http://www.pulmonaryfibrosis.org

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    Autoimmune Information Network, Inc.
    PO Box 4121
    Brick, NJ 08723
    Fax: (732)543-7285
    Email: autoimmunehelp@aol.com

    AutoImmunity Community
    Email: moderator@autoimmunitycommunity.org
    Internet: http://www.autoimmunitycommunity.org

    Global Fibrosis Foundation
    250 Main Street
    Suite 425775
    Cambridge, MA 02142
    Tel: (727)433-8180
    Email: globalfibrosis@gmail.com
    Internet: http://www.globalfibrosis.com

    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated:  5/7/2014
    Copyright  1987, 1989, 1999, 2000, 2004, 2007, 2014 National Organization for Rare Disorders, Inc.

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