title: Treatment of ANCA-associated vasculitis.
abstract: Antineutrophil cytoplasmic autoantibody (ANCA)-associated diseases are small-vessel vasculitides, encompassing granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Once considered life-threatening diseases, the introduction of stage-adapted immunosuppressive therapy and medications with decreased toxicity has improved patients' survival. Treatment is biphasic, consisting of induction of remission (3-6 months) for rapid control of disease activity and maintenance of remission (at least 18 months) to prevent disease relapse using therapeutic alternatives that have reduced toxicity. This Review summarizes current treatment strategies for these diseases, with a special focus on long-term follow-up data from key randomized controlled trials and new developments in remission induction and maintenance therapy. Current treatment strategies have substantial short-term and long-term adverse effects, and relapses are frequent; thus, less-toxic and more-effective approaches are needed. Moreover, the optimal intensity and duration of maintenance therapy remains under debate. Clinical trials have traditionally considered ANCA-associated vasculitides as a single disease entity. However, future studies must stratify participants according to their specific disease, clinical features (different types of organ manifestation, PR3-ANCA or MPO-ANCA positivity) and disease severity.
authors.affiliation: Medizinische Klinik und Poliklinik IV, Nephrologisches Zentrum, University Hospital Munich, Campus Grosshadern, Ludwig-Maximilians-University, Marchioninstrasse 15, Munich D-81377, Germany.
authors.forename: Ulf
authors.initials: U
authors.lastname: Schönermarck
authors.affiliation: University Hospital Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, Lübeck D-23538, Germany.
authors.forename: Wolfgang L
authors.initials: WL
authors.lastname: Gross
authors.affiliation: IIIrd Medical Department, Klinikum Offenbach GmbH, Starkenburgring 66, KfH Renal Center Starkenburgring 70, Offenbach/Main D-63069, Germany.
authors.forename: Kirsten
authors.initials: K
authors.lastname: de Groot
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D012074:Remission Induction / Q000379:methods
chemical_list: D007166:Immunosuppressive Agents