Complex genetics of Wegener granulomatosis.
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.
Jagiello
Peter
P
Department of Human Genetics, Ruhr-University Bochum, Universitaetsstrasse 150, 44801 Bochum, Germany. peter.jagiello@rub.de
Gross
Wolfgang L
WL
Epplen
Jörg T
JT
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D020022:Genetic Predisposition to Disease; D014890:Granulomatosis with Polyangiitis / Q000235:genetics* / Q000276:immunology; D006680:HLA Antigens / Q000235:genetics / Q000276:immunology; D006801:Humans; D009504:Neutrophils / Q000276:immunology; D016176:T-Lymphocyte Subsets / Q000276:immunology; D018417:Th1 Cells / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D006680:HLA Antigens