Autoimmune Pulmonary Alveolar Proteinosis
ICD-10: CA61.0MeSH: D011648ORPHA: 7471 Treatment Available
Overview
A rare lung disorder characterized by accumulation of surfactant proteins in alveoli due to autoantibodies against GM-CSF. Patients experience progressive dyspnea and are at increased risk for opportunistic infections. Global prevalence is estimated at 1 in 100,000.
Available Treatments (1)
| Drug | Form | Status | Countries | Lead Time |
|---|---|---|---|---|
| GM-CSF Orphan Cold Chain | Powder for solution | FDA Approved | 1 | — |
Clinical Presentation
Signs and symptoms associated with Autoimmune Pulmonary Alveolar Proteinosis, sourced from HPO and Orphanet clinical annotations.
Intraalveolar phospholipid accumulationCyanosisClubbingAbnormality of the upper respiratory tractRestrictive ventilatory defectDyspneaFoam cellsAbnormal circulating protein concentrationHypoxemiaIncreased circulating lactate dehydrogenase concentrationAutoimmune antibody positivityDecreased DLCOCoughCrazy paving patternCracklesWeight lossFeverHemoptysisFatigueChest pain
Classification & Codes
ICD-10 Code
CA61.0MeSH Code
D011648Orphanet Code
ORPHA:747Autoimmune Pulmonary Alveolar Proteinosis
| ICD-10 | CA61.0 |
| MeSH | D011648 |
| Orphanet | ORPHA:747 |
| Treatments | 1 drug(s) |
| Symptoms on record | 20 signs |
| Status | published |
Treatment Summary
Factual Authority
Last Updated3/21/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO