Chronic immunoglobulin maintenance therapy in myasthenia gravis
Chronic immunoglobulin maintenance therapy in myasthenia gravis
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DOI：
10.1111/ene.14547
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发表时间：
2020-10-15
影响因子：
5.1
通讯作者：
Bril, V.
中科院分区：
文献类型：
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作者：
Alcantara, M.;Sarpong, E.;Bril, V.
背景和目的重症肌无力（MG）的长期治疗包括针对免疫系统的对症治疗和病程调整治疗。最近，静脉注射免疫球蛋白（IVIG）和皮下注射免疫球蛋白（SCIG）已成为慢性治疗的可行选择，考虑到有利的安全性-有效性和可能的免疫抑制剂节省属性。目的是探讨免疫球蛋白（IG）治疗泛发性MG的长期护理结果。方法采用回顾性、重复测量的研究设计。分析了2015年1月至2020年1月接受IVIG/SCIG治疗至少6个月的全身性MG患者的图表。主要结果是IG治疗后重症肌无力损害指数（MGII）的平均变化，比较基线与IVIG和SCIG治疗期。次要结局包括吡啶斯的明、免疫抑制药物的变化和患者报告的结局“正常百分比”（0%-100%）。结果34例患者接受了长期IG治疗，其中IVIG/SCIG 30例，SCIG 3例，IVIG 1例。IVIG和SCIG的平均持续时间分别为21.8 ± 19.4（范围3-64）个月和19.5 ± 11.3（范围5-45）个月。MGII评分显著降低（基线27.7 +/- 15.7; IVIG期22.0 +/- 17.4; SCIG期19.5 +/- 18.1;F = 17.9; d.f. = 1.7;P < 0.01）、吡啶斯的明和免疫抑制剂使用（P = 0.00）。结果“正常百分比”与两种治疗均呈显著正相关（P = 0.00）。结论IG治疗可使MG患者顺利过渡到IVIG或从IVIG过渡到SCIG，并减少其他药物的使用和损害，改善患者的整体状况。需要前瞻性随机研究来阐明成本和比较有效性。
Background and purpose Long-term treatment of myasthenia gravis (MG) includes symptomatic and course-modifying therapies that target the immune system. Recently, both intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) have emerged as viable options for chronic therapy, considering the favourable safety-efficacy profile and possible immunosuppressant sparing properties. The aim was to investigate the outcomes of the long-term care of generalized MG with immunoglobulin (Ig). Methods This is a retrospective, repeated-measures design study. Charts of generalized MG patients, treated with IVIG/SCIG for at least 6 months, from January 2015 to January 2020, were analysed. The primary outcome was the mean change in Myasthenia Gravis Impairment Index (MGII) after treatment with Ig, comparing baseline to IVIG and SCIG treatment periods. Secondary outcomes included the changes in pyridostigmine, immunosuppressive medications and patient-reported outcome 'percentage of normal' (0%-100%). Results Thirty-four patients were treated with chronic Ig therapy (30 IVIG/SCIG, three SCIG, one IVIG). The mean durations of IVIG and SCIG periods were 21.8 +/- 19.4 (range 3-64) months and 19.5 +/- 11.3 (range 5-45) months respectively. There was a significant reduction in MGII scores (27.7 +/- 15.7 baseline; 22.0 +/- 17.4 IVIG period; 19.5 +/- 18.1 SCIG period;F = 17.9; d.f. = 1.7;P < 0.01), pyridostigmine and immunosuppressant use (P = 0.00). The outcome 'percentage of normal' had a significant positive association with both treatments (P = 0.00). Conclusion Our study results suggest that patients can be successfully transitioned to IVIG and from IVIG to SCIG in the chronic treatment of generalized MG with reductions in impairments and use of other medications and improvement in overall status with Ig therapy. Prospective, randomized studies are needed to clarify costs and comparative effectiveness.