环孢素成功治疗难治性慢性炎症性脱髓鞘性多发性神经病
Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin.
作者信息
Odaka M, Tatsumoto M, Susuki K, Hirata K, Yuki N
机构信息
Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan.
出版信息
J Neurol Neurosurg Psychiatry. 2005 Aug;76(8):1115-20. doi: 10.1136/jnnp.2003.035428.
BACKGROUND
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder and both clinical course and response to treatment vary widely. Because of the propensity for relapse, CIDP requires maintenance therapy after the initial response to treatment. There is no consensus regarding this in the published literature.
PRESENT REPORT
A patient with CIDP was treated with oral prednisolone and cyclophosphamide pulse therapy but required repeated plasma exchange and intravenous immunoglobulin (IVIg). Treatment with ciclosporin freed the patient from repeated IVIg administration. Therapeutic responses in 14 subsequent cases including three patients who showed improvement with ciclosporin are also presented along with an algorithm of the authors' suggested protocol for treatment.
CONCLUSION
Ciclosporin should be considered for patients with intractable CIDP who require repeated IVIg.
背景
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种异质性疾病，临床病程和对治疗的反应差异很大。由于有复发倾向，CIDP在初始治疗反应后需要维持治疗。已发表的文献对此尚无共识。
当前报告
一名CIDP患者接受了口服泼尼松龙和环磷酰胺脉冲治疗，但需要反复进行血浆置换和静脉注射免疫球蛋白（IVIg）。环孢素治疗使该患者无需反复静脉注射免疫球蛋白。还介绍了随后14例患者的治疗反应，包括3例使用环孢素后病情改善的患者，以及作者建议的治疗方案算法。
结论
对于需要反复静脉注射免疫球蛋白的难治性CIDP患者，应考虑使用环孢素。
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