12Diseases of the respiratory system
Respiratory diseases principally affecting the lung interstitium
CB04Primary interstitial lung diseases specific to infancy or childhood
CB04.3Alveolar or peri-alveolar conditions
CB04.31
category
Pulmonary alveolar proteinosis
Definition This is a rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange. PAP can occur in a primary form or secondarily in the settings of malignancy (especially in myeloid leukemia), pulmonary infection, or environmental exposure to dusts or chemicals.
Also indexed as
Autoimmune pulmonary alveolar proteinosis Congenital pulmonary alveolar proteinosis Idiopathic alveolar lipoproteinosis Idiopathic pulmonary alveolar proteinosis Pulmonary alveolar proteinosis Secondary pulmonary alveolar proteinosis
Nearby in Alveolar or peri-alveolar conditions
International Classification of Diseases, Eleventh Revision (ICD-11), World Health Organization (WHO) 2019/2021, https://icd.who.int/browse11. Licensed under Creative Commons Attribution-NoDerivatives 3.0 IGO (CC BY-ND 3.0 IGO).
Release 2026-01.
View this entity in the official WHO ICD-11 browser .
This page reproduces WHO classification content for reference. It is not
medical advice and must not be used to diagnose or treat. For coding
decisions, consult the official release and your national coding guidelines.
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