Mueller A, Holl-Ulrich K, Feller A C, Gross W L, Lamprecht P
Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, D-23538 Luebeck, Germany.
Clin Exp Rheumatol. 2003 Nov-Dec;21(6 Suppl 32):S49-54.
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.
