Management of ANCA associated vasculitis

• Published in: BMJ (Online) Vol. 368; p. m421
• Main Authors: Wallace, Zachary S, Miloslavsky, Eli M
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 18-03-2020
• Subjects: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - drug therapy; Antineutrophil cytoplasmic antibodies; Azathioprine; Clinical trials; Cyclophosphamide; Cyclophosphamide - therapeutic use; Cytokines; Glucocorticoids; Glucocorticoids - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Immunotherapy; Kidney diseases; Methotrexate; Methotrexate - therapeutic use; Monoclonal antibodies; Morbidity; Mycophenolate mofetil; Mycophenolic acid; Mycophenolic Acid - therapeutic use; Neutrophils; Plasma; Remission; Remission Induction; Rituximab; Rituximab - therapeutic use; Toxicity; Vasculitis
• ISSN: 1756-1833; 1756-1833
• DOI: 10.1136/bmj.m421

ABSTRACTAnti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a small to medium vessel vasculitis associated with excess morbidity and mortality. This review explores how management of AAV has evolved over the past two decades with pivotal randomized controlled trials shaping the management of induction and maintenance of remission. Contemporary AAV care is characterized by approaches that minimize the cumulative exposure to cyclophosphamide and glucocorticoids, increasingly use rituximab for remission induction and maintenance, and consider therapies with less toxicity (for example, methotrexate, mycophenolate mofetil) for manifestations of AAV that do not threaten organ function or survival. Simultaneously, improvements in outcomes, such as renal and overall survival, have been observed. Additional trials and observational studies evaluating the comparative effectiveness of agents for AAV in various patient subgroups are needed. Prospective studies are necessary to assess the effect of psychosocial interventions on patient reported outcomes in AAV. Despite the expanding array of treatments for AAV, little guidance on how to personalize AAV care is available to physicians.