Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica
Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica
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DOI：
10.1177/1352458506071174
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发表时间：
2007-01-01
影响因子：
5.8
通讯作者：
Itoyama, Y.
中科院分区：
文献类型：
--
作者：
Watanabe, S.;Nakashima, I.;Itoyama, Y.
视神经肌萎缩症（NMO）是一种中枢神经系统（CNS）炎症性脱髓鞘疾病，预后不良。最近，血清自身抗体（NMO-IgG）结合血脑屏障区域被检测到专门在NMO患者及其高危人群。我们治疗了6例NMO-IgG阳性患者（均为女性;年龄21-67岁，中位数41岁; 3例患有视神经炎，3例患有肌病），这些患者对大剂量静脉内甲泼尼龙（HIMP）无反应，采用血浆置换（PE）（3 - 5次置换，每次2-3 L）。其中3例患者（1例视神经炎和2例肌萎缩）在PE后显示出明确的功能改善。在一次或两次交换后，临床改善开始出现，而其他三名患者的改善很少或没有改善。对PE的这种快速临床反应表明体液免疫因子在NMO中的致病作用，尽管对皮质类固醇治疗的延迟反应可能部分地促成了治疗效果。需要进一步的临床和体外研究来确定NMO-IgG的去除是否与治疗效果直接相关。PE可能加速某些NMO-IgG阳性NMO患者皮质类固醇耐药复发后的功能恢复。
Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system (CNS) with a poor prognosis in terms of the optic-spinal function. Recently, a serum autoantibody (NMO-IgG) binding to the blood-brain barrier region was detected exclusively in patients with NMO and its high risk group. We treated six NMO-IgG-positive patients (all female; age 21-67 years old, median 41; three with optic neuritis and three with myelitis) who were unresponsive to high-dose intravenous methylprednisolone (HIMP), with plasma exchange (PE) (three to five exchanges, 2-3 L each). Three of the patients (one with optic neuritis and two with myelitis) showed definite functional improvement following PE. The clinical improvement started to appear after one or two exchanges, while there was little or no improvement in the other three patients. Such quick clinical responses to PE suggest a pathogenetic role of humoral immune factors in NMO, although delayed responses to the corticosteroid therapy might have contributed to the therapeutic efficacy, in part. Further clinical and in vitro studies are needed to determine whether the removal of NMO-IgG is directly relevant to the therapeutic efficacy. PE may hasten the functional recovery from corticosteroid-resistant relapses in some NMO-IgG-positive patients with NMO.