难治性重症肌无力患者先静脉注射环磷酰胺诱导治疗，随后口服免疫抑制剂维持治疗。
Induction intravenous cyclophosphamide followed by maintenance oral immunosuppression in refractory myasthenia gravis.
作者信息
Buzzard Katherine A, Meyer Nicholas J, Hardy Todd A, Riminton D Sean, Reddel Stephen W
机构信息
Department of Neurology, Concord Repatriation General Hospital, Concord West, New South Wales, 2139, Australia.
Department of Immunology, Concord Repatriation General Hospital, Concord West, New South Wales, Australia.
出版信息
Muscle Nerve. 2015 Aug;52(2):204-10. doi: 10.1002/mus.24536. Epub 2015 Jun 18.
INTRODUCTION
Myasthenia gravis (MG) can be refractory to conventional immunotherapy. We report on the efficacy and durability of intravenous (IV) remission-induction cyclophosphamide (CYC) followed by oral immunosuppression in refractory MG.
METHODS
We identified 8 patients from our medical records with moderate or severe refractory MG who were treated with 6 cycles of IV CYC (0.75 g/m(2) ) every 4 weeks followed by oral immunosuppression.
RESULTS
Six patients improved within 3 months of treatment. Four patients remained in clinical remission (mean follow-up 31 months). Two patients responded partially, and 1 patient relapsed after 11 months. Two patients were non-responders. CYC was well tolerated. Acetylcholine receptor antibody levels remained below pretreatment levels in patients in clinical remission. The leukocyte nadir was lower in CYC responders.
CONCLUSIONS
Remission-induction IV CYC followed by oral immunosuppression is a rapid, effective, and durable treatment for refractory MG. Adding a post-CYC immunosuppressant may account for low relapse rates compared with other published series.
引言
重症肌无力（MG）可能对传统免疫疗法无效。我们报告了静脉注射（IV）诱导缓解环磷酰胺（CYC）随后口服免疫抑制剂治疗难治性MG的疗效和持久性。
方法
我们从病历中识别出8例中度或重度难治性MG患者，每4周接受6个周期的静脉注射CYC（0.75 g/m²）治疗，随后进行口服免疫抑制治疗。
结果
6例患者在治疗3个月内病情改善。4例患者保持临床缓解（平均随访31个月）。2例患者部分缓解，1例患者在11个月后复发。2例患者无反应。CYC耐受性良好。临床缓解患者的乙酰胆碱受体抗体水平仍低于治疗前水平。CYC反应者的白细胞最低点较低。
结论
静脉注射诱导缓解CYC随后口服免疫抑制剂是治疗难治性MG的一种快速、有效且持久的治疗方法。与其他已发表的系列研究相比，CYC后添加免疫抑制剂可能是复发率低的原因。
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