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References OMIM Gene GeneReviews HGMD HGNC
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