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Pneumonia, Interstitial

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Pneumonia, Interstitial 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.

Synonyms

  • Chronic Fibrous Pneumonia
  • Usual Interstitial Pneumonia (UIP)
  • Fibrous Interstitial Pneumonia
  • Bronchiolitis Obliterans Organizing Pneumonia (Boop)
  • Diffuse Alveolar Damage
  • Giant Cell Interstitial Pneumonia
  • Idiopathic Interstitial Pneumonia

Disorder Subdivisions

  • Idiopathic Pulmonary Fibrosis (IPF)
  • Desquamative Interstitial Pneumonia (DIP)
  • Lymphoid Interstitial Pneumonia (LIP)
  • Nonspecific Interstitial Pneumonia (NSIP)
  • Cryptogenic Organizing Pneumonia (COP)
  • Acute Interstitial Pneumonia (AIP)
  • Respiratory Bronchiolitis-Associated Interstitial Lung Disease (RB-ILD)

General Discussion

The abnormal accumulation of inflammatory cells in lung tissue may lead to any one of several disorders with similar signs and symptoms. As white blood cells and protein-rich plasma build up in the air sacs of the lungs (alveoli), inflammation is generated. The inflammatory process, if it lasts long enough, may harden the fluid and the resultant firm, fibrous substance (scarring) may replace the lung tissue. If the scarring is extensive, the air sacs may be destroyed over time and the resultant space replaced by cysts.

The American Thoracic Society and the European Respiratory Society jointly studied the interstitial pneumonias during 2001 and issued a Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This consensus statement was intended to replace several older classification schemes that had led to a confusion of names and syndromes.

Participants agreed that the Idiopathic Interstitial Pneumonias (IIPs) comprise a number of clinical entities that are each rare and sufficiently different from one another to be considered as distinct disorders. This report follows the joint ARS/ERS classification.

Resources

American Lung Association
1301 Pennsylvania Ave NW
Suite 800
Washington, DC 20004
USA
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/

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/

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:  3/22/2008
Copyright  1989, 2002 National Organization for Rare Disorders, Inc.

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