Pathogenesis: immunogenetic factors.
The occurrence, albeit infrequent, of systemic vasculitis in closely related family members suggests that both environmental and genetic factors may play a role in the pathogenesis of these diseases. Malfunction of immune regulation in the systemic vasculitides may indicate a role for genes that encode molecules critical to the immune responses. The extremely polymorphic sequences of MHC molecules may provide a structural basis for associations of MHC genes and systemic vasculitis. This review summarizes recent reports of MHC associations, mechanisms by which MHC may play a role in certain vasculitides, and also examines the role for genes encoding non-MHC molecules, such as Fcgamma receptors, cytokines and T cell co-stimulators. Data suggest that the pathogenesis of systemic vasculitides such as giant-cell arteritis, Takayasu's arteritis and Wegener's granulomatosis might be governed by multiple genes encoding host defence molecules, in conjunction with environmental factors.
Huang
D
D
Lerner Research Institute, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.
Zhou
Y
Y
Hoffman
G S
GS
D016000:Cluster Analysis; D016207:Cytokines / Q000235:genetics; D005192:Family Health; D020022:Genetic Predisposition to Disease; D014890:Granulomatosis with Polyangiitis / Q000276:immunology; D008213:Lymphocyte Activation; D008285:Major Histocompatibility Complex / Q000235:genetics; D017452:Receptors, IgG / Q000235:genetics; D013601:T-Lymphocytes / Q000276:immunology; D014657:Vasculitis / Q000235:genetics* / Q000276:immunology*
D016207:Cytokines; D017452:Receptors, IgG