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慢性炎症性脱髓鞘性多发性神经根神经病患者皮下免疫球蛋白的长期治疗:长达 7 年的随访期。

Long-term treatment with subcutaneous immunoglobulin in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a follow-up period up to 7 years.

机构信息

Unit of Neurology and Neuromuscular Diseases, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

出版信息

Sci Rep. 2020 May 13;10(1):7910. doi: 10.1038/s41598-020-64699-6.

Abstract

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare and heterogeneous acquired sensory-motor polyneuropathy with autoimmune pathogenesis. Intravenous immunoglobulins (IVIG) are a well-established therapy for CIDP: it is well known that at least two-thirds of these patients need these infusions for several years. More recently, Subcutaneous Immunoglobulins (SCIg) have been proved to be effective: this finding has been confirmed either in isolated cases or in few randomized trials. However, it appeared that the longest SCIg treatment follow up lasted no longer than 48 months. We report herein the results of a long-term SCIg treatment with a follow up period up to 7 years (84 months), considering safety, tolerability and patients' perception of SCIg treatment in a CIDP population. We studied 17 patients (10 M; 7 F) with a diagnosis of CIDP, defined according to the EFNS/PNS criteria, successfully treated with IVIG every 4/6 weeks before being switched to SCIg treatment. Clinical follow-up included, apart from a routinely clinical assessment, the administration of Medical Research Council (MRC) sum-score, the Overall Neuropathy Limitation Scale (ONLS) and the Life Quality Index questionnaire (LQI). The results showed that, in the majority of this pre-selected group of CIDP patients (16/17), SCIg were well tolerated and were preferred over IVIG. Strength and motor functions remained stable or even improved during the long term follow-up (up to 84 months) with benefits on walking capability and resistance, manual activity performances and fatigue reduction.

摘要

慢性炎症性脱髓鞘性多发性神经病(CIDP)是一种罕见且异质性的获得性感觉运动多发性神经病,具有自身免疫发病机制。静脉注射免疫球蛋白(IVIG)是 CIDP 的一种成熟治疗方法:众所周知,这些患者中至少有三分之二需要这些输注多年。最近,皮下免疫球蛋白(SCIg)已被证明是有效的:这一发现无论是在单独的病例还是在少数随机试验中都得到了证实。然而,似乎最长的 SCIg 治疗随访时间不超过 48 个月。我们在此报告了一项长达 7 年(84 个月)的 SCIg 治疗的长期结果,考虑了安全性、耐受性以及 CIDP 患者对 SCIg 治疗的看法。我们研究了 17 名(10 名男性;7 名女性)根据 EFNS/PNS 标准诊断为 CIDP 的患者,他们在接受 SCIg 治疗之前成功接受了每 4/6 周一次的 IVIG 治疗。临床随访除了常规临床评估外,还包括使用肌电图(MRC)总和评分、总体神经功能障碍量表(ONLS)和生活质量指数问卷(LQI)进行评估。结果表明,在大多数预先选择的 CIDP 患者(16/17)中,SCIg 具有良好的耐受性,并且优于 IVIG。在长达 84 个月的长期随访中,力量和运动功能保持稳定甚至改善,行走能力和抵抗力、手部活动性能和疲劳减轻都有获益。

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