Wegener's Granulomatosis (WG) is an autoimmune disease with manifestations in different organ systems. The hallmark of WG is a necrotizing granulomatous inflammation of the upper and/or lower respiratory tract and systemic small vessel vasculitis which can involve multiple organ systems. The treatment of WG has evolved over the last decades. Steroid, cytotoxic and biologic therapies have been used leading to great improvements in outcome. However, still mortality is high and relapses are a major cause of mortality and morbidity. Despite intensified maintenance regimens and new possibilities of biologic therapies in WG the relapse rate is high. Even patients treated with high dose cytotoxic therapies in autologous stem cell treatment protocols have shown relapses in the course of disease. Increasing knowledge of the pathophysiology of granuloma in WG and new biologic therapies might be of great importance for future treatment of WG.
2010 Elsevier B.V. All rights reserved.