title: Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab.
abstract: Granulomatosis with polyangiitis and microscopic polyangiitis are anti-neutrophil cytoplasmic antibody-associated vasculitides (AAVs) that are prone to cycles of remission and relapse. The introduction of cytotoxic therapy has changed the prognosis for these diseases from typically fatal to manageable chronic illnesses with a relapsing course. Despite improvements in outcomes, recurrence of disease and drug-related toxicity continue to produce significant morbidity and mortality. Better understanding of the pathogenesis of AAV and the mechanism of action of cyclophosphamide has led to investigation of therapies that target B cells. Two randomized controlled trials have shown that rituximab is not inferior to cyclophosphamide for induction of remission in severe AAV, with no significant difference in the incidence of overall adverse events in rituximab- versus cyclophosphamide-treated patients. Data from ongoing clinical trials will determine the role of rituximab in the maintenance of remission.
authors.affiliation: Division of Nephrology, Johns Hopkins University School of Medicine, Johns Hopkins Bayview Medical Center, 301 Mason Lord Drive, Suite 2500, Baltimore, MD, USA, gduvura@jhmi.edu.
authors.forename: Duvuru
authors.initials: D
authors.lastname: Geetha
authors.forename: Cees
authors.initials: C
authors.lastname: Kallenberg
authors.forename: John H
authors.initials: JH
authors.lastname: Stone
authors.forename: Alan D
authors.initials: AD
authors.lastname: Salama
authors.forename: Gerald B
authors.initials: GB
authors.lastname: Appel
authors.forename: George
authors.initials: G
authors.lastname: Duna
authors.forename: Paul
authors.initials: P
authors.lastname: Brunetta
authors.forename: David
authors.initials: D
authors.lastname: Jayne
mesh_terms: D003520:Cyclophosphamide / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000009:adverse effects / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000150:complications; D060046:Maintenance Chemotherapy; D055953:Microscopic Polyangiitis / Q000150:complications / Q000188:drug therapy*; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab / Q000009:adverse effects / Q000627:therapeutic use*
chemical_list: D005938:Glucocorticoids; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide