CB04.31

ICD-11 MMS 2026-01

Pulmonary alveolar proteinosis

Description

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.

Index terms

  • Pulmonary alveolar proteinosis
  • Autoimmune pulmonary alveolar proteinosis
  • Idiopathic alveolar lipoproteinosis
Show allHide
  • Idiopathic pulmonary alveolar proteinosis
  • Congenital pulmonary alveolar proteinosis
  • Secondary pulmonary alveolar proteinosis

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
Show allHide
  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations and chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • CB04.30 – Idiopathic pulmonary haemosiderosis
  • CB04.3Y – Other specified alveolar or peri-alveolar conditions
  • CB04.3Z – Alveolar or peri-alveolar conditions, unspecified

Foundation entity

Pulmonary alveolar proteinosis – http://id.who.int/icd/entity/1869739196

Allowed postcoordination axes

Part of: CB04.3: Alveolar or peri-alveolar conditions › CB04: Primary interstitial lung diseases specific to infancy or childhood › 12: Diseases of the respiratory system