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Diseases

Genetic and Rare Diseases Information Center (GARD)

Acquired pulmonary alveolar proteinosis


Other Names for this Disease
  • PAP
  • Pulmonary alveolar lipoproteinosis acquired
  • PAP acquired
  • Pulmonary alveolar proteinosis autoimmune
  • Pulmonary alveolar proteinosis acquired
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Acquired pulmonary alveolar proteinosis (PAP) is a rare, acquired lung disorder that causes the build up of surfactant 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. The standard treatment is a procedure called lung lavage. Acquired PAP differs from congenital PAP, an extremely rare form of PAP that occurs in some newborns.[1]
Last updated: 4/22/2016

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) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
  • A page For Individuals with PAP is available from the PAP Foundation. This page covers frequently asked questions about the condition. Please note: this organization appears to be inactive, so the page may not be kept up-to-date. 

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • 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. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Acquired pulmonary alveolar proteinosis. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • PAP
  • Pulmonary alveolar lipoproteinosis acquired
  • PAP acquired
  • Pulmonary alveolar proteinosis autoimmune
  • Pulmonary alveolar proteinosis acquired
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.