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Genetic and Rare Diseases Information Center (GARD)

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Pulmonary alveolar proteinosis acquired


Other Names for this Disease

  • PAP
  • PAP acquired
  • Pulmonary alveolar lipoproteinosis acquired
  • Pulmonary alveolar proteinosis autoimmune
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Overview

Acquired pulmonary alveolar proteinosis (PAP) is a rare, acquired lung disorder characterized by the accumulation of grainy material consisting mostly of protein and fat (lipoproteinaceous material) in the air sacs of the lungs (alveoli). Most cases affect adults between the ages of 20-50. The symptoms can vary greatly; some individuals may not show symptoms, while others may experience progressive difficulty breathing and shortness of breath upon exertion. Other signs and symptoms may include a dry, chronic cough; fatigue; weight loss; chest pain; and a general feeling of ill health. In rare cases, the coughing up of blood, rounding and swelling of the tips of the fingers, and cyanosis may be present. Most cases occur for no known reason, but some cases may occur secondary to environmental exposures or underlying diseases; some researchers believe it may be an autoimmune disorder. The treatment varies from case to case depending upon the age of the affected individual and severity of the disease. Acquired PAP differs from congenital PAP, an extremely rare form of PAP that occurs in some newborns.[1]
Last updated: 7/3/2011

References

  1. Pulmonary Alveolar Proteinosis. NORD. May 6, 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/594/viewAbstract. Accessed 7/3/2011.
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Basic Information

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. 
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

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  • The Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
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  • PubMed is a searchable database of medical literature and lists journal articles that discuss Pulmonary alveolar proteinosis acquired. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • PAP
  • PAP acquired
  • Pulmonary alveolar lipoproteinosis acquired
  • Pulmonary alveolar proteinosis autoimmune
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.