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Long-term immunosuppressive therapy is required in most cases. High-dose prednisone combined with intravenous pulse or oral daily cyclophosphamide is standard initial therapy. In those with NSVN, cyclophosphamide also should be used if prednisone monotherapy is ineffective or the patient relapses with tapering. Other agents, such as azathioprine, methotrexate, intravenous immunoglobulin, mycophenolate mofetil, plasma exchange, and rituximab can be offered to patients who are intolerant or have a contraindication to cyclophosphamide. However, evidence for the benefit of these agents is limited to case reports and small case series. |
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