Current landscape of antineutrophil cytoplasmic antibody-associated vasculitis: classification, diagnosis, and treatment.
This article provides an update on the diagnosis and management of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, granulomatosis with polyangiitis (formerly Wegener), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss). Focus is on new schemes of classification and the importance of ANCAs in the diagnosis and prognosis of these systemic vasculitides. Current therapeutic strategies consisting of glucocorticoids in conjunction with conventional or biologic agents for both induction of remission and remission maintenance are outlined. Future research directions include investigation of the optimal duration and frequency of maintenance therapy and development of targeted therapeutic agents.
Division of Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA. Electronic address: lallyl@hss.edu.
Lindsay
L
Lally
Division of Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA.
Robert
R
Spiera
D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D015267:Churg-Strauss Syndrome / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy; D004797:Enzyme-Linked Immunosorbent Assay; D005938:Glucocorticoids / Q000627:therapeutic use*; D014890:Granulomatosis with Polyangiitis / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D060046:Maintenance Chemotherapy / Q000379:methods; D055953:Microscopic Polyangiitis / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy; D053146:Myeloblastin / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D012074:Remission Induction / Q000379:methods
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D009195:Peroxidase; D053146:Myeloblastin
ANCA-associated vasculitis; Eosinophilic granulomatosis with polyangiitis (Churg-Strauss); Granulomatosis with polyangiitis (Wegener); Microscopic polyangiitis