Idiopathic Pulmonary Fibrosis

National Organization for Rare Disorders, Inc.

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

American Lung Association
1301 Pennsylvania Ave NW
Suite 800
Washington, DC 20004
Tel: (202)785-3355
Fax: (202)452-1805
Tel: (800)586-4872

NIH/National Heart, Lung and Blood Institute
P.O. Box 30105
Bethesda, MD 20892-0105
Tel: (301)592-8573
Fax: (301)251-1223

Second Wind Lung Transplant Association, Inc.
P.O. Box 1657
Wimberley, TX 78676-1657
Tel: (512)847-9303
Tel: (888)855-9463

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

Pulmonary Fibrosis Foundation
230 East Ohio Street
Suite 304
Chicago, IL 60611-3201
Fax: (866)587-9158
Tel: (888)733-6741

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

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

AutoImmunity Community

Global Fibrosis Foundation
250 Main Street
Suite 425775
Cambridge, MA 02142
Tel: (727)433-8180

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 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 or email

Last Updated:  5/7/2014
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Idiopathic Pulmonary Fibrosis