How is pulmonary alveolar proteinosis (PAP) classified?

Updated: Dec 18, 2019
  • Author: Rodolfo Laucirica, MD; Chief Editor: Philip T Cagle, MD  more...
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Pulmonary alveolar proteinosis (PAP) is classified into two main categories: congenital (neonatal) and acquired. Acquired PAP is subdivided into an autoimmune form (previously termed idiopathic/primary) and secondary form (ie, due to an underlying disease). [14] Most cases of PAP are the autoimmune form (90%), [1, 14, 15] followed by secondary (4%), and congenital (1%), with the remaining 5% consisting of undetermined PAP-like disease. [1] Adults between aged 39 and 51 years are most commonly affected, with a 2:1 male-to-female predominence. [1] Infants and children can also be affect. [1]

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