Orphanet: Autoimmune pulmonary alveolar proteinosis

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Autoimmune pulmonary alveolar proteinosis

Disease definition

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of a lipoproteinaceous substance in the distal air spaces which positively stains with periodic acid-Schiff (PAS).


Classification level: Disorder
  • Synonym(s):
    • Autoimmune PAP
    • Idiopathic PAP
    • Idiopathic pulmonary alveolar proteinosis
    • aPAP
    • iPAP
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: J84.0
  • OMIM: 610910
  • UMLS: C1970472
  • MeSH: -
  • GARD: 7499
  • MedDRA: -

Detailed information


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