Importance Recent studies suggest that maintenance intravenous immunoglobulin (IVIG) may be an effective treatment to prevent relapses in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD); however, most of these studies had pediatric cohorts, and few studies have evaluated IVIG in adult patients. Objective To determine the association of maintenance IVIG with the prevention of disease relapse in a large adult cohort of patients with MOGAD. Design, Setting, and Participants This was a retrospective cohort study conducted from January 1, 2010, to October 31, 2021. Patients were recruited from 14 hospitals in 9 countries and were included in the analysis if they (1) had a history of 1 or more central nervous system demyelinating attacks consistent with MOGAD, (2) had MOG-IgG seropositivity tested by cell-based assay, and (3) were age 18 years or older when starting IVIG treatment. These patients were retrospectively evaluated for a history of maintenance IVIG treatment. Exposures Maintenance IVIG. Main Outcomes and Measures Relapse rates while receiving maintenance IVIG compared with before initiation of therapy. Results Of the 876 adult patients initially identified with MOGAD, 59 (median [range] age, 36 [18-69] years; 33 women [56%]) were treated with maintenance IVIG. IVIG was initiated as first-line immunotherapy in 15 patients (25%) and as second-line therapy in 37 patients (63%) owing to failure of prior immunotherapy and in 7 patients (12%) owing to intolerance to prior immunotherapy. The median (range) annualized relapse rate before IVIG treatment was 1.4 (0-6.1), compared with a median (range) annualized relapse rate while receiving IVIG of 0 (0-3) (t108 = 7.14; P < .001). Twenty patients (34%) had at least 1 relapse while receiving IVIG with a median (range) time to first relapse of 1 (0.03-4.8) years, and 17 patients (29%) were treated with concomitant maintenance immunotherapy. Only 5 of 29 patients (17%) who received 1 g/kg of IVIG every 4 weeks or more experienced disease relapse compared with 15 of 30 patients (50%) treated with lower or less frequent dosing (hazard ratio, 3.31; 95% CI, 1.19-9.09; P = .02). At final follow-up, 52 patients (88%) were still receiving maintenance IVIG with a median (range) duration of 1.7 (0.5-9.9) years of therapy. Seven of 59 patients (12%) discontinued IVIG therapy: 4 (57%) for inefficacy, 2 (29%) for adverse effects, and 1 (14%) for a trial not receiving therapy after a period of disease inactivity. Conclusions and Relevance Results of this retrospective, multicenter, cohort study of adult patients with MOGAD suggest that maintenance IVIG was associated with a reduction in disease relapse. Less frequent and lower dosing of IVIG may be associated with treatment failure. Future prospective randomized clinical trials are warranted to confirm these findings.

中文翻译：

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维持静脉注射免疫球蛋白与预防成人髓鞘少突胶质细胞糖蛋白抗体相关疾病复发的关联。

重要性 最近的研究表明，维持静脉注射免疫球蛋白 (IVIG) 可能是预防髓鞘少突胶质细胞糖蛋白抗体相关疾病 (MOGAD) 复发的有效治疗方法；然而，这些研究大多针对儿科队列，很少有研究在成年患者中评估 IVIG。目的 在 MOGAD 患者的大型成人队列中确定维持 IVIG 与预防疾病复发的关联。设计、地点和参与者 这是一项从 2010 年 1 月 1 日到 2021 年 10 月 31 日进行的回顾性队列研究。从 9 个国家/地区的 14 家医院招募了患者，如果他们 (1) 有 1 次或更多与 MOGAD 一致的中枢神经系统脱髓鞘发作，(2) 通过基于细胞的测定法测试了 MOG-IgG 血清阳性，(3) 开始 IVIG 治疗时年满 18 岁。回顾性评估了这些患者的 IVIG 维持治疗史。暴露维护 IVIG。与开始治疗前相比，接受 IVIG 维持治疗时的主要结果和测量指标的复发率。结果 在最初确定为 MOGAD 的 876 名成年患者中，59 名（中位 [范围] 年龄，36 [18-69] 岁；33 名女性 [56%]）接受了维持性 IVIG 治疗。15 名患者 (25%) 开始使用 IVIG 作为一线免疫治疗，37 名患者 (63%) 由于既往免疫治疗失败而作为二线治疗，7 名患者 (12%) 由于对既往免疫治疗不耐受而开始使用 IVIG。IVIG 治疗前的中位（范围）年化复发率为 1.4（0-6.1），与接受 IVIG 的中位（范围）年化复发率 0 (0-3) 相比（t108 = 7.14；P < .001）。20 名患者 (34%) 在接受 IVIG 时至少有 1 次复发，首次复发的中位（范围）时间为 1 (0.03-4.8) 年，17 名患者 (29%) 接受了伴随的维持免疫治疗。每 4 周或更长时间接受 1 g/kg IVIG 的 29 名患者中只有 5 名 (17%) 出现疾病复发，而接受较低或较低频率给药治疗的 30 名患者中有 15 名 (50%) 出现疾病复发（风险比，3.31；95%置信区间，1.19-9.09；P = .02）。在最后一次随访时，52 名患者 (88%) 仍在接受维持性 IVIG，中位（范围）持续时间为 1.7（0.5-9.9）年的治疗。59 名患者中有 7 名 (12%) 停止了 IVIG 治疗：4 名 (57%) 因无效，2 名 (29%) 因不良反应，1 例 (14%) 用于在一段时间的疾病不活动后未接受治疗的试验。结论和相关性 这项针对成年 MOGAD 患者的回顾性、多中心、队列研究的结果表明，维持 IVIG 与疾病复发的减少有关。IVIG 的频率较低和剂量较低可能与治疗失败有关。需要未来的前瞻性随机临床试验来证实这些发现。