What is the prognosis of pulmonary alveolar proteinosis (PAP)?

Updated: Dec 18, 2019
  • Author: Roger B Olade, MD, MPH; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

The overall prognosis for primary pulmonary alveolar proteinosis (PAP) is very good, with complete remission achieved in many patients. Whole-lung lavage most often results in a dramatic response. Some patients require repeated lavages; these patients usually progress to pulmonary fibrosis and have a poor outcome. Congenital PAP responds favorably to lung transplantation.

The major complications are lung infections with Nocardia asteroidesPneumocystis carinii, and/or Mycobacterium avium-intracellulare. [25]  Pulmonary fibrosis and/or cor pulmonale also can complicate PAP.

There has been some association between anti-granulocyte-macrophage colony-stimulating factor (anti-GM-CSF) autoantibodies and some cases of cryptococcal meningitis in otherwise immunocompetent patients. [26]

Mortality as high as 30% within several years of disease onset has been reported, but the actual mortality rate may be less than 10%. A severe form of congenital PAP in children from la Réunion Island has an overall mortality rate of 59% and is characterized by an early onset, associated liver involvement, and frequent progression to lung fibrosis, despite treatment with whole-lung lavage treatment. [24]  

The natural history of secondary PAP depends on the underlying etiologic entity.


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