Autoimmune pulmonary alveolar proteinosis

Orpha code: 747OMIM code: 610910

Definition

A rare primary interstitial lung disease characterized by the accumulation of lipids and proteins related to surfactant in the alveoli in association with the presence of antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). The disease leads to a progressive impairment of gas exchange and respiratory insufficiency.

Disease data
Classification

Disease

Synonyms
Autoimmune PAP
aPAP
Autoimmunologiczna PAP
Idiopatyczna PAP
Idiopatyczna proteinoza pęcherzyków płucnych
iPAP
aPAP
ORPHA code
747
OMIM code
610910
ICD10 code
J84.0
ICD11 code
CB04.31

No additional description.

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