Wegener's granulomatosis

Wegener's granulomatosis (WG) is;currently categorized as one of the antine utrophil cytoplasmic antibody (ANCA)associated small-vessel vasculitides di stinguished by its predilection to affect the upper and lower respiratory t racts and kidneys clinically and histologically by the presence of necrosis , granulomatous inflammation, and vasculitis. However, small biopsies, espe cially from the head and neck region, often lack all these diagnostic histo logic findings. Other common histologic features of WG include microabscess es and scattered multinucleated giant cells in a highly inflammatory backgr ound. Support from distinctive clinical setting or positive cytoplasmic (C) -ANCA testing may help establish the diagnosis of WG in cases lacking all t he typical pathologic findings. The histopathologic differential diagnosis of WG includes nonspecific inflammatory conditions, infections, angiocentri c lymphomas, collagen vascular diseases, and other forms of angiitis and gr anulomatosis. The prognosis of WG has dramatically improved from the 18% 5- month survival rate before the era of immunosuppressive therapy to the curr ent remission rate of over 75% with a regimen of cyclophosphamide and gluco corticoids. A significant rate of relapse and profound disease- and/or trea tment-related morbidity still occur. The cause of WG remains unknown, but c ircumstantial evidences;suggest the potential roles of ANCA and infection i n the pathogenesis. Copyright (C) 2001 by W.B. Saunders Company.