Akash Virupakshaiah, Vinicius A Schoeps, Jonathan Race, Michael Waltz, Siefaddeen Sharayah, Zahra Nasr, Carson E Moseley, Scott S Zamvil, Cristina Gaudioso, Allison Schuette, Theron Charles Casper, John Rose, Eoin P Flanagan, Moses Rodriguez, Jan-Mendelt Tillema, Tanuja Chitnis, Mark P Gorman, Jennifer S Graves, Leslie A Benson, Mary Rensel, Aaron Abrams, Lauren Krupp, Timothy E Lotze, Gregory Aaen, Yolanda Wheeler, Teri Schreiner, Amy Waldman, Janet Chong, Soe Mar, Emmanuelle Waubant
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The study aimed to identify predictors at MOGAD onset that are associated with a relapsing course.</p><p><strong>Methods: </strong>Prospectively collected data from paediatric patients with MOGAD seen by the US Network of Paediatric MS Centres were leveraged. Univariable and adjusted multivariable models were used to predict recurrent disease.</p><p><strong>Results: </strong>We identified 326 MOGAD cases (mean age at first event 8.9 years [SD 4.3], 57% female, 77% white and 74% non-Hispanic) and 46% relapsed during a mean follow-up of 3.9 years (SD 4.1). In the adjusted multivariable model, female sex (HR 1.66, 95% CI 1.17 to 2.36, p=0.004) and Hispanic/Latino ethnicity (HR 1.77, 95% CI 1.19 to 2.64, p=0.005) were associated with a higher risk of relapsing MOGAD. Maintenance treatment initiated before a second event with rituximab (HR 0.25, 95% CI 0.07 to 0.92, p=0.037) or intravenous immunoglobulin (IVIG) (HR 0.35, 95% CI 0.14 to 0.88, p=0.026) was associated with lower risk of a second event in multivariable analyses. Conversely, maintenance steroids were associated with a higher estimated relapse risk (HR 1.76, 95% CI 0.90 to 3.45, p=0.097).</p><p><strong>Conclusion: </strong>Sex and ethnicity are associated with relapsing MOGAD. Use of rituximab or IVIG therapy shortly after onset is associated with a lower risk of the second event. 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Almost half of the patients experience relapses, but very few studies have evaluated predictors of relapse risk, challenging clinical management. The study aimed to identify predictors at MOGAD onset that are associated with a relapsing course.</p><p><strong>Methods: </strong>Prospectively collected data from paediatric patients with MOGAD seen by the US Network of Paediatric MS Centres were leveraged. Univariable and adjusted multivariable models were used to predict recurrent disease.</p><p><strong>Results: </strong>We identified 326 MOGAD cases (mean age at first event 8.9 years [SD 4.3], 57% female, 77% white and 74% non-Hispanic) and 46% relapsed during a mean follow-up of 3.9 years (SD 4.1). In the adjusted multivariable model, female sex (HR 1.66, 95% CI 1.17 to 2.36, p=0.004) and Hispanic/Latino ethnicity (HR 1.77, 95% CI 1.19 to 2.64, p=0.005) were associated with a higher risk of relapsing MOGAD. 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引用次数: 0
摘要
背景:髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)是最近才被描述的一种脱髓鞘疾病,儿童约占所有病例的 50%。近一半的患者会复发,但很少有研究对复发风险的预测因素进行评估,这给临床治疗带来了挑战。本研究旨在确定与复发病程相关的 MOGAD 发病预测因素:方法:研究人员利用从美国儿科多发性硬化症中心网络(US Network of Paediatric MS Centres)收治的MOGAD儿科患者中收集的前瞻性数据。结果:我们发现了 326 例 MOGAD 病例:我们发现了 326 例 MOGAD 病例(首次发病时的平均年龄为 8.9 岁 [SD 4.3],57% 为女性,77% 为白人,74% 为非西班牙裔),46% 在平均 3.9 年(SD 4.1)的随访期间复发。在调整后的多变量模型中,女性(HR 1.66,95% CI 1.17 至 2.36,P=0.004)和西班牙裔/拉丁裔(HR 1.77,95% CI 1.19 至 2.64,P=0.005)与复发 MOGAD 的风险较高有关。在多变量分析中,利妥昔单抗(HR 0.25,95% CI 0.07至0.92,P=0.037)或静脉注射免疫球蛋白(IVIG)(HR 0.35,95% CI 0.14至0.88,P=0.026)在第二次发病前开始的维持治疗与第二次发病的较低风险相关。相反,维持类固醇与较高的估计复发风险相关(HR 1.76,95% CI 0.90 至 3.45,P=0.097):结论:性别和种族与MOGAD复发有关。结论:性别和种族与 MOGAD 的复发有关,发病后不久使用利妥昔单抗或 IVIG 治疗与第二次复发风险较低有关。对于复发风险较高的患者,可考虑在首次发病后进行预防性治疗。
Predictors of a relapsing course in myelin oligodendrocyte glycoprotein antibody-associated disease.
Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described demyelinating disorder, and children represent about 50% of all cases. Almost half of the patients experience relapses, but very few studies have evaluated predictors of relapse risk, challenging clinical management. The study aimed to identify predictors at MOGAD onset that are associated with a relapsing course.
Methods: Prospectively collected data from paediatric patients with MOGAD seen by the US Network of Paediatric MS Centres were leveraged. Univariable and adjusted multivariable models were used to predict recurrent disease.
Results: We identified 326 MOGAD cases (mean age at first event 8.9 years [SD 4.3], 57% female, 77% white and 74% non-Hispanic) and 46% relapsed during a mean follow-up of 3.9 years (SD 4.1). In the adjusted multivariable model, female sex (HR 1.66, 95% CI 1.17 to 2.36, p=0.004) and Hispanic/Latino ethnicity (HR 1.77, 95% CI 1.19 to 2.64, p=0.005) were associated with a higher risk of relapsing MOGAD. Maintenance treatment initiated before a second event with rituximab (HR 0.25, 95% CI 0.07 to 0.92, p=0.037) or intravenous immunoglobulin (IVIG) (HR 0.35, 95% CI 0.14 to 0.88, p=0.026) was associated with lower risk of a second event in multivariable analyses. Conversely, maintenance steroids were associated with a higher estimated relapse risk (HR 1.76, 95% CI 0.90 to 3.45, p=0.097).
Conclusion: Sex and ethnicity are associated with relapsing MOGAD. Use of rituximab or IVIG therapy shortly after onset is associated with a lower risk of the second event. Preventive treatment after a first event could be considered for those with a higher relapse risk.
期刊介绍:
The Journal of Neurology, Neurosurgery & Psychiatry (JNNP) aspires to publish groundbreaking and cutting-edge research worldwide. Covering the entire spectrum of neurological sciences, the journal focuses on common disorders like stroke, multiple sclerosis, Parkinson’s disease, epilepsy, peripheral neuropathy, subarachnoid haemorrhage, and neuropsychiatry, while also addressing complex challenges such as ALS. With early online publication, regular podcasts, and an extensive archive collection boasting the longest half-life in clinical neuroscience journals, JNNP aims to be a trailblazer in the field.