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.
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
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American Lung Association
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Genetic and Rare Diseases (GARD) Information Center
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Global Fibrosis Foundation
- Yale Dermatopathology Service
- 15 York Street, LMP 5031
- New Haven, CT 06520-8059
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- Website: http://www.globalfibrosis.com
NIH/National Heart, Lung and Blood Institute
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Pulmonary Fibrosis Foundation
- 230 East Ohio Street
- Suite 304
- Chicago, IL 60611-3201
- Fax: (866)587-9158
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- Email: firstname.lastname@example.org
- Website: http://www.pulmonaryfibrosis.org
Second Wind Lung Transplant Association, Inc.
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- Wimberley, TX 78676-1657
- Tel: (512)847-9303
- Tel: (888)855-9463
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- Website: http://www.2ndwind.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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Last Updated: 7/14/2015
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