Preferred Label : Autoimmune pulmonary alveolar proteinosis;
ICD-11 definition : Pulmonary alveolar proteinosis (PAP) is a rare restrictive lung disease characterized
by the accumulation of a lipoproteinaceous substance in the distal air spaces which
positively stains with periodic acid-Schiff (PAS). T;
ICD-11 synonym : Idiopathic alveolar lipoproteinosis; Idiopathic pulmonary alveolar proteinosis;
Origin ID : 676409940;
Automatic exact mappings (from CISMeF team)
Pulmonary alveolar proteinosis (PAP) is a rare restrictive lung disease characterized
by the accumulation of a lipoproteinaceous substance in the distal air spaces which
positively stains with periodic acid-Schiff (PAS). T