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Аутоімунний альвеолярний протеїноз легень

ORPHA:747· ICD-10 J84.0· Autoimmune pulmonary alveolar proteinosis

Визначення(English summary)

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.

Поширеність
1-9 / 1 000 000
Успадкування
Multigenic/multifactorial, Not applicable
Вік початку
Adolescent, Adult, Childhood