Rituximab For Remission In Relapsing Anca Negative Retro Orbital Wegener's Granulomatosis

翻译者：梁娜娜 校对者：郭乾育 审阅者：张莉芸

We present a follow-up on a previously published case of a 59 year-old female diagnosed with ANCA negative limited Wegener’s Granulomatosis(WG ) of the right orbit with history of Graves’disease who presented with exophthalmos. Despite treatment with steroids and cyclophosphamide, patient had a relapse while steroids were being tapered. She was then treated with IV Rituximab with remission and resolution of her symptoms. She is currently doing well on maintenance therapy with methotrexate. Rituximab is a monoclonal anti-CD20 antibody effective in treatment of ANCA associated vasculitis including systemic WG. Our case shows Rituximab may be a therapeutic option in the treatment of ANCA negative retro orbital WG in patients who failed treatment with cyclophosphamide.

一名59岁中老年女性患者，被诊断为ANCA阴性的，以右眼眶为主要表现的韦格纳肉芽肿病，既往患有突眼表现的Graves病。虽然联合使用类固醇激素和环磷酰胺治疗，但当类固醇激素逐渐减量的过程中，患者病情复发。之后患者使用利妥昔单抗治疗，症状缓解。患者目前使用维持量甲氨喋呤，病情控制尚可。利妥昔单抗是一种抗CD20的单克隆抗体，治疗ANCA相关性血管炎包括系统性WG有效。我们的病例表明，利妥昔单抗可能是ANCA阴性的眼窝WG患者在环磷酰胺治疗失败后的良好选择。