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Granulomatosis with polyangiitis

ORPHA:900· ICD-10 M31.3

Definition

A rare anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis characterized by necrotizing inflammation of small and medium vessels (capillaries, venules and arterioles), resulting in tissue ischemia.

Prevalence
1-9 / 1 000 000
Inheritance
Not applicable
Age of onset
Adolescent, Adult, Childhood, Elderly