pulmonary alveolar proteinosis

MeSH: D011649ORPHA: 747

Overview

lung disease that is characterized by abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with 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

MeSH Code

D011649

Orphanet Code

ORPHA:747
pulmonary alveolar proteinosis
MeSHD011649
OrphanetORPHA:747
Treatments0 drug(s)
Symptoms on record20 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO