Pulmonary Alveolar Proteinosis
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Pulmonary Alveolar Proteinosis is not the name you expected.
The lung is composed of millions of tiny air sacs (alveoli) with very thin walls that allow oxygen in the air we breathe to pass through into the blood. Surfactant is a natural substance consisting of fat (mostly phospholipids) and a small amount of protein made in alveoli. Normally, a thin layer of surfactant present on the surface of alveoli helps them stay open. This allows air to come in and out as we breathe. Once used, surfactant is removed (cleared) from alveoli by cells called alveolar macrophages. This helps prevent surfactant from building up too much. Alveolar macrophages require a protein called granulocyte/macrophage-colony stimulating factor (GM-CSF) to maintain a normal surfactant level in alveoli (a process called surfactant homeostasis).
Pulmonary alveolar proteinosis (PAP) is a syndrome, a set of symptoms and signs and not just a single disease, in which surfactant slowly builds up in alveoli. This blocks air from entering alveoli and oxygen from passing through into the blood, which results in a feeling of breathlessness (dyspnea). Research has greatly improved our understanding of the diseases that cause PAP and how to identify (diagnose) and treat them. Diseases that cause PAP can occur in men, women, and children of all ages, ethnic backgrounds, and geographic locations. Disease severity varies from mild to severe and depends on which disease is present. Thus, it is important to know which disease is causing PAP in order to determine the best therapy and expected treatment response. Diseases that cause PAP can be grouped into three categories: primary PAP, secondary PAP, and congenital PAP (more accurately called disorders of surfactant production).
American Lung Association
- 55 W. Wacker Drive
- Suite 1150
- Chicago, IL 60601
- Tel: 1-800-548-8252
- Email: firstname.lastname@example.org
- Website: http://www.lungusa.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
- Website: http://rarediseases.info.nih.gov/GARD/
NIH/National Heart, Lung and Blood Institute
- P.O. Box 30105
- Bethesda, MD 20892-0105
- Tel: (301)592-8573
- Fax: (301)251-1223
- Email: email@example.com
- Website: http://www.nhlbi.nih.gov/
Rare Diseases Clinical Research Network
- University of South Florida, Pediatrics Epidemiology Center
- 3650 Spectrum Boulevard, Suite 100
- Tampa, FL 33612
- Tel: (813)396-9501
- Fax: (813)910-5997
- Tel: (866)533-9104
- Email: RareDiseasesNetwork.org, RDCRN.org
- Website: http://rarediseasesnetwork.epi.usf.edu/registry/
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".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
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.
Last Updated: 9/10/2014
Copyright 2014 National Organization for Rare Disorders, Inc.
PeaceHealth endeavors to provide comprehensive health care information, however some topics in this database describe services and procedures not offered by our providers or within our facilities because they do not comply with, nor are they condoned by, the ethics policies of our organization.
Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.