Idiopathic Pulmonary Fibrosis


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



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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".

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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

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Last Updated:  5/7/2014

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