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Diseases

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



What is acquired pulmonary alveolar proteinosis?

How might acquired pulmonary alveolar proteinosis be treated?


What is acquired pulmonary alveolar proteinosis?

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

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.