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静脉注射免疫球蛋白预防重症肌无力急性加重。

Intravenous immunoglobulin for prophylaxis of acute exacerbation in Myasthenia Gravis.

机构信息

Department of Neurology, Ankara University School of Medicine, İbni Sina Hospital, Samanpazarı, Ankara, Turkey,

出版信息

Neurol Sci. 2014 Jun;35(6):891-6. doi: 10.1007/s10072-013-1621-4. Epub 2014 Jan 8.

DOI:10.1007/s10072-013-1621-4
PMID:24399309
Abstract

Intravenous immunoglobulin (IVIg) treatment for acute exacerbations of Myasthenia Gravis (MG) was shown in several open-label studies. There are only two studies demonstrating the efficiency of regular intermittent IVIg therapy on MG patients who are not in their acute attack periods. Thirteen patients who had displayed an inadequate clinical response to immunosuppressive treatments, or who were not appropriate for immunosuppressive treatment due to the age factor and thus were given regular IVIg therapy, were retrospectively investigated. Moreover, the pre- and post-treatment attack frequencies were also evaluated. The mean number of attacks was 0.0960 attacks/year before IVIg therapy, and 0.0056 attacks/year after IVIg therapy (p = 0.002). The number and severity of the attacks were decreased in all patients. Eight patients (62 %) had used steroids; among them, steroid was completely stopped in two patients following the regular IVIg therapy, and the dose was decreased by 50 % in the other six patients. The requirement for pyridostigmine did not decrease in four patients, whereas this need decreased by 20-50 % in nine patients. IVIg can produce repeated beneficial effects in patients with MG and may be useful as an adjunct in the management of MG. IVIg has minimal adverse effects and ability to reduce corticosteroid dose. These results suggest that intravenous immunoglobulin maintenance therapy is a valid modality in patients with resistant treatment MG.

摘要

静脉注射免疫球蛋白(IVIg)治疗重症肌无力(MG)急性加重在几项开放标签研究中得到了证实。只有两项研究证明了定期间歇性 IVIg 治疗对不在急性发作期的 MG 患者的疗效。对 13 名因年龄因素不适合免疫抑制治疗或因免疫抑制治疗反应不佳而接受常规 IVIg 治疗的患者进行了回顾性调查。此外,还评估了治疗前后的发作频率。IVIg 治疗前的平均发作次数为 0.0960 次/年,治疗后的平均发作次数为 0.0056 次/年(p = 0.002)。所有患者的发作次数和严重程度均有所减少。8 名患者(62%)使用过类固醇;其中,2 名患者在接受常规 IVIg 治疗后完全停用类固醇,6 名患者的剂量减少了 50%。4 名患者的吡啶斯的明需求没有减少,而 9 名患者的需求减少了 20-50%。IVIg 可在 MG 患者中产生重复的有益效果,可能有助于 MG 的治疗。IVIg 具有最小的不良反应和减少皮质类固醇剂量的能力。这些结果表明,静脉注射免疫球蛋白维持治疗是治疗耐药性 MG 的有效方法。

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