Association of Maintenance Intravenous Immunoglobulin With Prevention of Relapse in Adult Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease
Association of Maintenance Intravenous Immunoglobulin With Prevention of Relapse in Adult Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease
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DOI：
10.1001/jamaneurol.2022.0489
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发表时间：
2022-04-04
期刊：
影响因子：
29
通讯作者：
Marignier, Romain
中科院分区：
文献类型：
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作者：
Chen, John J.;Huda, Saif;Marignier, Romain
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，这些研究大多有儿科队列，很少有研究评估成人患者的IVIG。目的在一项大型成年加摩加迪沙患者队列研究中，确定维持IVIG与预防疾病复发的关系。背景和参与者：这是一项从2010年1月1日至2021年10月31日进行的回顾性队列研究。患者从9个国家的14家医院招募，如果他们(1)有1次或多次与MOGAD一致的中枢神经系统脱髓鞘发作史，(2)通过基于细胞的测定法检测MOG-IgG血清阳性，(3)在开始IVIG治疗时年龄在18岁或以上，则纳入分析。回顾性评估这些患者维持IVIG治疗的历史。暴露维护。与开始治疗前相比，接受维持性IVIG治疗时的复发率。结果：在876例最初确诊为MOGAD的成年患者中，59例（年龄中位数为36岁[18-69]岁；33例女性[56%]）接受了维持性IVIG治疗。IVIG作为一线免疫治疗在15例患者（25%）中开始，作为二线治疗在37例患者（63%）中开始，原因是先前的免疫治疗失败，7例患者（12%）是由于先前的免疫治疗不耐受。IVIG治疗前的年化复发率中位数（范围）为1.4(0-6.1)，而IVIG治疗后的年化复发率中位数（范围）为0 (0-3)(t(108) = 7.14；P < 0.001)。20例患者（34%）在接受IVIG治疗期间至少有一次复发，到首次复发的中位（范围）时间为1（0.03-4.8）年，17例患者（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治疗的29例患者中只有5例（17%）出现疾病复发，而较少或较少剂量治疗的30例患者中有15例（50%）出现疾病复发（风险比为3.31；95% CI为1.19-9.09;P = 0.02）。在最后随访时，52例患者（88%）仍在接受维持性IVIG治疗，治疗中位（范围）持续时间为1.7年（0.5-9.9年）。59例患者中有7例（12%）停止IVIG治疗：4例（57%）因无效，2例（29%）因不良反应，1例（14%）因疾病不活动一段时间后未接受治疗。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。注射频率较低和剂量较低可能与治疗失败有关。未来的前瞻性随机临床试验有必要证实这些发现。
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) (t(108) = 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.