Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.
Biomedical Research Institute, Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea.
JAMA Neurol. 2024 Oct 1;81(10):1073-1084. doi: 10.1001/jamaneurol.2024.2811.
A proportion of people with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have a relapsing disease course and persistent anti-myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) seropositivity. Few studies have investigated whether treatment of the first MOGAD attack is associated with the long-term disease course and/or MOG-IgG seronegative conversion.
To investigate the association of time to treat the first acute MOGAD attack with relapse risk and MOG-IgG serostatus.
DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective, nationwide, multicenter cohort study involving 14 secondary or tertiary hospitals in South Korea between November 2009 and August 2023. People with adult-onset MOGAD, who either had a relapse or were followed up for more than 12 months after disease onset and had a detailed medical record of their first attack, were included. Individuals were excluded for adolescent-onset MOGAD or short disease duration.
Patients were categorized based on the time to treat the first acute MOGAD attack: early (<5 days), intermediate (5-14 days), and late (not treated within 14 days).
A multivariable analysis for clinical and treatment factors associated with relapsing disease course and/or MOG-IgG seronegative conversion. Further subgroup analyses were conducted among those without long-term nonsteroidal immunosuppressant (NSIS) maintenance treatment.
Among the 315 individuals screened, 75 were excluded. A total of 240 patients (median [IQR] age at onset, 40.4 [28.8-56.1] years; 125 female [52.1%]) with median (IQR) disease duration of 3.07 (1.95-6.15) years were included. A total of 110 of 240 patients (45.8%) relapsed after a median (IQR) of 0.45 (0.18-1.68) years, and 29 of 116 patients (25.0%) experienced a conversion to seronegative MOG-IgG. Both the time to treatment of the first MOGAD attack (late vs early: adjusted hazard ratio [aHR], 2.64; 95% CI, 1.43-4.84; P = .002; intermediate vs early: aHR, 2.02; 95% CI, 1.10-3.74; P = .02) and NSIS maintenance treatment (aHR, 0.24; 95% CI, 0.14-0.42; P < .001) were independently associated with the risk of relapse. In a subgroup without NSIS maintenance, the time to treat of the first MOGAD attack was still associated with higher risk of relapse (late vs early: aHR, 3.51; 95% CI, 1.64-7.50; P = .001; intermediate vs early: aHR, 2.68; 95% CI, 1.23-5.85; P = .01). Lastly, the time to treat of the first MOGAD attack was also associated with MOG-IgG seronegative conversion (early vs late: adjusted odds ratio, 7.04; 95% CI, 1.58-31.41; P = .01), whereas NSIS maintenance treatment was not.
Results of this cohort study suggest that early treatment of the first acute MOGAD attack was associated with a reduction in the proportion of relapsing disease course and an increase in the likelihood of MOG-IgG seronegative conversion. These data suggest that timing of acute phase treatment for the first MOGAD attack can be associated with the long-term prognosis and autoimmune status of patients.
一部分髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)患者具有复发病程和持续的抗髓鞘少突胶质细胞糖蛋白免疫球蛋白 G(MOG-IgG)血清阳性。很少有研究调查首次 MOGAD 发作的治疗是否与长期病程和/或 MOG-IgG 血清阴性转换有关。
调查首次急性 MOGAD 发作的治疗时间与复发风险和 MOG-IgG 血清状态的关系。
设计、地点和参与者:这是一项回顾性、全国性、多中心队列研究,涉及韩国的 14 家二级或三级医院,研究时间为 2009 年 11 月至 2023 年 8 月。纳入标准为成人发病的 MOGAD 患者,这些患者要么复发,要么在发病后随访超过 12 个月,且有详细的首次发作病史。排除标准为青少年发病的 MOGAD 或疾病持续时间短。
根据首次急性 MOGAD 发作的治疗时间将患者分为以下三组:早期(<5 天)、中期(5-14 天)和晚期(未在 14 天内治疗)。
多变量分析与复发病程和/或 MOG-IgG 血清阴性转换相关的临床和治疗因素。在没有长期非甾体免疫抑制剂(NSIS)维持治疗的患者中进行了进一步的亚组分析。
在筛选出的 315 名患者中,有 75 名被排除。最终纳入 240 名患者(中位[IQR]发病年龄为 40.4[28.8-56.1]岁,女性 125 名[52.1%]),中位(IQR)疾病持续时间为 3.07(1.95-6.15)年。240 名患者中有 110 名(45.8%)在中位(IQR)0.45(0.18-1.68)年后复发,116 名患者中有 29 名(25.0%)出现 MOG-IgG 血清阴性转换。首次 MOGAD 发作的治疗时间(晚期与早期:调整后的危险比[aHR],2.64;95%CI,1.43-4.84;P=0.002;中期与早期:aHR,2.02;95%CI,1.10-3.74;P=0.02)和 NSIS 维持治疗(aHR,0.24;95%CI,0.14-0.42;P<0.001)与复发风险独立相关。在没有 NSIS 维持治疗的亚组中,首次 MOGAD 发作的治疗时间仍然与更高的复发风险相关(晚期与早期:aHR,3.51;95%CI,1.64-7.50;P=0.001;中期与早期:aHR,2.68;95%CI,1.23-5.85;P=0.01)。最后,首次 MOGAD 发作的治疗时间也与 MOG-IgG 血清阴性转换相关(早期与晚期:调整后的优势比[aOR],7.04;95%CI,1.58-31.41;P=0.01),而 NSIS 维持治疗则没有。
本队列研究结果表明,首次急性 MOGAD 发作的早期治疗与降低复发病程比例和增加 MOG-IgG 血清阴性转换的可能性相关。这些数据表明,急性发作期治疗首次 MOGAD 发作的时间可能与患者的长期预后和自身免疫状态有关。