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Diseases

Genetic and Rare Diseases Information Center (GARD)

Pulmonary alveolar proteinosis acquired


Other Names for this Disease
  • aPAP
  • Autoimmune PAP
  • Autoimmune pulmonary alveolar proteinosis
  • Idiopathic PAP
  • Idiopathic pulmonary alveolar proteinosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment

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How might acquired pulmonary alveolar proteinosis be treated?

The treatment of PAP varies from case to case depending upon the age of an affected individual and severity of the disease. Approximately one-third of individuals with idiopathic PAP (of unknown cause) will improve without treatment (spontaneous remission). The other two-thirds may be treated by a whole lung lavage, a procedure in which one lung is cleansed with a salt solution while the other is pumped with pure oxygen. In some cases, the procedure may need to be performed once; in others it may need to be repeated many times over several years.[1]

In secondary PAP (due to environmental exposure or an underlying disorder), removal and avoidance of the causative agent (e.g., silica exposure) or treatment of the underlying disorder may improve symptoms.[1] Inhaled GM-CSF (granulocyte-macrophage colony-stimulating factor), a blood-stimulating medication, has been shown to improve the condition in some individuals with PAP.[1][2] Lung transplantation has been used to treat adults with PAP as a last resort. According to the medical literature, in some cases, PAP has recurred in adults who have received lung transplantation.[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.
  2. Allen J. Blaivas. Pulmonary Alveolar Proteinosis. MedlinePlus. May 21, 2009; http://www.nlm.nih.gov/medlineplus/ency/article/000114.htm. Accessed 7/3/2011.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information

Clinical Trials & Research for this Disease

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
  • ClinicalTrials.gov lists trials that are studying or have studied Pulmonary alveolar proteinosis acquired. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • The Rare Lung Diseases Consortium: Molecular Pathway-Driven Diagnostics and Therapeutics for Rare Lung Diseases is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research involving pulmonary alveolar proteinosis, Hermansky-Pudlak Syndrome, and Lymphangioleiomyomatosis. A pilot project program supports research into other rare lung diseases that complement the main research projects.

Other Names for this Disease
  • aPAP
  • Autoimmune PAP
  • Autoimmune pulmonary alveolar proteinosis
  • Idiopathic PAP
  • Idiopathic pulmonary alveolar proteinosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.