Immune phenomena in localized and generalized Wegener's granulomatosis 

A. Mueller¹, K. Holl-Ulrich², A.C. Feller², W.L. Gross¹, P. Lamprecht¹

¹Department of Rheumatology and ²Institute of Pathology, University Hospital of Schleswig-Holstein, Campus Luebeck, Germany

ABSTRACT
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. 

Key words
Wegener's granulomatosis, granuloma, necrosis, vasculitis, ANCA.

Please address correspondence and reprint requests to: Antje Mueller, PhD, Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, D-23538 Luebeck, Germany. 
E-mail: mueller@medinf.mu-luebeck.de

Clin Exp Rheumatol 2003; 21: (Suppl. 32): S49-S54.
© Copyright Clinical and Experimental Rheumatology 2003.