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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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Your Question

My brother-in-law has been diagnosed with this. What are the most common treatments or drugs for this condition? They plan to lavage the lungs. He has a history of chronic smoking.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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