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GENATLAS PHENOTYPE

last update : 28-11-2017

Symbol	WG
Location	6p21.3, 19p13.3
Name	Wegener's granulomatosis
Corresponding gene	PRTN3 , HLA-DPB1
Main clinical features	small vessels vasculitis leading to necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis and vasculitis the main autoantigen of ANCA in WG is proteinase 3 (PR3)
Genetic determination
Prevalence	. WG is more predominant among Caucasians, prevalence of WG ranging from 24 to 157 per million and annual incidence rates from 3
Function/system disorder	defense and immunity
Type	susceptibility factor

Remark(s)

Wegener granulomatosis (WG) belongs to a heterogeneous group of systemic anti-neutrophil cytoplasmatic antibody (ANCA) associated vasculitides (AASV), the genetic predisposition appears quite complex