CB04.31 Pulmonary alveolar proteinosis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

postcoordination

synonyms

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

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