Autoimmune pulmonary alveolar proteinosis
ORPHA:747· ICD-10 J84.0
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.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adolescent, Adult, Childhood