Translational Neuroimmunology Group, Kids Neuroscience Centre and Brain and Mind Centre, Sydney Medical School, Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia.
Clinical Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Sydney, New South Wales, Australia.
J Neurol Neurosurg Psychiatry. 2024 Oct 16;95(11):1054-1063. doi: 10.1136/jnnp-2024-333463.
We sought to identify an optimal oral corticosteroid regimen at the onset of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), which would delay time to first relapse while minimising cumulative corticosteroid exposure.
In a retrospective multicentre cohort study, Cox proportional hazards models examined the relationship between corticosteroid course as a time-varying covariate and time to first relapse. Simon-Makuch and Kaplan-Meier plots identified an optimal dosing strategy.
We evaluated 109 patients (62 female, 57%; 41 paediatric, 38%; median age at onset 26 years, (IQR 8-38); median follow-up 6.2 years (IQR 2.6-9.6)). 76/109 (70%) experienced a relapse (median time to first relapse 13.7 months; 95% CI 8.2 to 37.9). In a multivariable model, higher doses of oral prednisone delayed time to first relapse with an effect estimate of 3.7% (95% CI 0.8% to 6.6%; p0.014) reduced hazard of relapse for every 1 mg/day dose increment. There was evidence of reduced hazard of relapse for patients dosed ≥12.5 mg/day (HR 0.21, 95% CI 0.07 to 0.6; p0.0036), corresponding to a 79% reduction in relapse risk. There was evidence of reduced hazard of relapse for those dosed ≥12.5 mg/day for at least 3 months (HR 0.12, 95% CI 0.03 to 0.44; p0.0012), corresponding to an 88% reduction in relapse risk compared with those never treated in this range. No patient with this recommended dosing at onset experienced a Common Terminology Criteria for Adverse Events grade >3 adverse effect.
The optimal dose of 12.5 mg of prednisone daily in adults (0.16 mg/kg/day for children) for a minimum of 3 months at the onset of MOGAD delays time to first relapse.
我们试图确定髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)发病时的最佳口服皮质类固醇治疗方案,该方案既要延迟首次复发时间,又要将皮质类固醇累积暴露量最小化。
在一项回顾性多中心队列研究中,Cox 比例风险模型分析了皮质类固醇疗程作为时变协变量与首次复发时间之间的关系。Simon-Makuch 和 Kaplan-Meier 图确定了最佳给药策略。
我们评估了 109 例患者(62 例女性,占 57%;41 例儿科患者,占 38%;发病时的中位年龄为 26 岁,IQR 为 8-38 岁;中位随访时间为 6.2 年,IQR 为 2.6-9.6 年)。109 例患者中有 76 例(70%)发生复发(首次复发的中位时间为 13.7 个月,95%CI 为 8.2-37.9 个月)。多变量模型显示,较高剂量的口服泼尼松可延迟首次复发时间,其效应估计值为 3.7%(95%CI 为 0.8%-6.6%;p=0.014),每增加 1mg/天剂量可降低复发风险。对于每天剂量≥12.5mg 的患者,有证据表明复发风险降低(HR 0.21,95%CI 为 0.07-0.6;p=0.0036),复发风险降低 79%。对于每天至少 3 个月剂量≥12.5mg 的患者,有证据表明复发风险降低(HR 0.12,95%CI 为 0.03-0.44;p=0.0012),与从未在此范围内治疗的患者相比,复发风险降低 88%。在发病时接受推荐剂量治疗的患者中,没有出现不良事件通用术语标准>3 级的不良事件。
MOGAD 发病时,成人每日 12.5mg 泼尼松(儿童为 0.16mg/kg/天)的最佳剂量至少持续 3 个月,可延迟首次复发时间。