Immune phenomena in localized and generalized Wegener's granulomatosis.
Wegener's granulomatosis (WG) is characterized by granulomatous inflammation and systemic vasculitis with a predilection for the lungs and kidneys. In most patients WG begins with a localized organ involvement of the upper respiratory tract that progresses to systemic disease (generalized WG) (1). Because of the life-threatening nature of systemic vasculitis, much effort has concentrated on elucidating the pathogenesis of the vasculitis. However, based upon a renewed interest in (innate) immune defenses against microbes, a better understanding of the chronic granulomatous inflammation may contribute to a more precise insight into the early genesis of WG. Thus, this review focuses on summarizing and discussing data for a potential pattern of disease, i.e. from localized to generalized WG with a special emphasis on granulomatous lesions of the upper respiratory tract and their alterations during the disease course.
Mueller
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A
Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, D-23538 Luebeck, Germany. mueller@medinf.mu-luebeck.de
Holl-Ulrich
K
K
Feller
A C
AC
Gross
W L
WL
Lamprecht
P
P
D014890:Granulomatosis with Polyangiitis / Q000276:immunology* / Q000473:pathology*; D006801:Humans; D008168:Lung / Q000276:immunology / Q000473:pathology