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Review
. 2005 Jan;4(1):42-7.
doi: 10.1016/j.autrev.2004.06.003.

Complex genetics of Wegener granulomatosis

Peter Jagiello  1 , Wolfgang L Gross, Jörg T Epplen
Affiliations
Review

Complex genetics of Wegener granulomatosis

Peter Jagiello et al. Autoimmun Rev. 2005 Jan.

Abstract

Wegener granulomatosis (WG) belongs to a heterogeneous group of systemic anti-neutrophil cytoplasmatic antibody (ANCA) associated vasculitides (AASV). WG is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis and vasculitis. As a multifactorial model disease, WG is hallmarked by the presence of specific ANCA-subtypes directed against a defined antigen. WG is more predominant among Caucasians and the genetic predisposition appears quite complex. Here, we provide a brief overview concerning genetic factors in the pathogenesis of WG and discuss intricacies of molecular genetic approaches.

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