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 Treatment of ANCA-associated systemic vasculitis Belmont HMBull NYU Hosp Jt Dis  2006[]; 64 (1-2): 60-6The antineutrophil cytoplasmic antibodies (ANCA)-associated small vessel  vasculitides include Wegener's granulomatosis, Churg-Strauss syndrome,  microscopic polyangiitis (MPA), and the renal limited form of MPA, also known as  pauci-immune or idiopathic crescentic glomerulonephritis. ANCA are probably  necessary but not sufficient for disease pathogenicity. Classical induction and  maintenance therapy of these conditions with corticosteroids and long-term  cyclophosphamide is associated with occasional relapse and major toxicities.  Therefore, treatment regimens being investigated include induction with  methotrexate or, especially for patients with more aggressive disease accompanied  by renal insufficiency, therapies that include either pulses of  methylprednisolone or plasma exchanges. Nontraditional options for maintenance  therapy may include step-down treatment with azathioprine or mycophenolate  mofetil. For patients with Wegener's granulomatosis, studies have shown a reduced  occurrence of flares with the use of co-trimoxazole. Finally, although a  carefully randomized controlled trial with etanercept demonstrated that this  tumor necrosis factor (TNF)-blocking agent was not superior to conventional  maintenance therapy, a biologic agent with a different mechanism of action,  rituximab, may prove a satisfactory alternative.|Antibodies, Antineutrophil Cytoplasmic/*immunology[MESH]|Glucocorticoids/*therapeutic use[MESH]|Humans[MESH]|Immunosuppressive Agents/*therapeutic use[MESH]|Plasma Exchange/*methods[MESH]|Remission Induction/methods[MESH]|Treatment Outcome[MESH]|Vasculitis/*immunology/*therapy[MESH]
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