CORe, Department of Medicine, University of Melbourne, Melbourne, Australia; Department of Neurology, Royal Melbourne Hospital, Melbourne, Australia; Department of Neurology, Royal Melbourne Hospital, Melbourne, Australia.
CORe, Department of Medicine, University of Melbourne, Melbourne, Australia; Department of Neurology, Royal Melbourne Hospital, Melbourne, Australia; Department of Neurology, Royal Melbourne Hospital, Melbourne, Australia.
Mult Scler Relat Disord. 2020 Feb;38:101868. doi: 10.1016/j.msard.2019.101868. Epub 2019 Nov 25.
Aquaporin-4-IgG positive (AQP4-IgG+) Neuromyelitis Optica Spectrum Disorder (NMOSD) is an uncommon central nervous system autoimmune disorder. Disease outcomes in AQP4-IgG+NMOSD are typically measured by relapse rate and disability. Using the MSBase, a multi-centre international registry, we aimed to examine the impact immunosuppressive therapies and patient characteristics as predictors of disease outcome measures in AQP4-IgG+NMOSD.
This MSBase cohort study of AQP4-IgG+NMOSD patients examined modifiers of relapse in a multivariable proportional hazards model and expanded disability status score (EDSS) using a mixed effects model.
206 AQP4-IgG+ patients were included (median follow-up 3.7 years). Age (hazard ratio [HR] = 0.82 per decade, p = 0.001), brainstem onset (HR = 0.45, p = 0.009), azathioprine (HR = 0.46, p<0.001) and mycophenolate mofetil (HR = 0.09, p = 0.012) were associated with a reduced risk of relapse. A greater EDSS was associated with age (β = 0.45 (per decade), p<0.001) and disease duration (β = 0.07 per year, p<0.001). A slower increase in EDSS was associated with azathioprine (β = -0.48, p<0.001), mycophenolate mofetil (β = -0.69, p = 0.04) and rituximab (β = -0.35, p = 0.024).
This study has demonstrated that azathioprine and mycophenolate mofetil reduce the risk of relapses and disability progression is modified by azathioprine, mycophenolate mofetil and rituximab. Age and disease duration were the only patient characteristics that modified the risk of relapse and disability in our cohort.
水通道蛋白 4 免疫球蛋白 G 阳性（AQP4-IgG+）视神经脊髓炎谱系疾病（NMOSD）是一种罕见的中枢神经系统自身免疫性疾病。AQP4-IgG+NMOSD 的疾病结果通常通过复发率和残疾来衡量。本研究利用 MSBase（一个多中心国际登记处），旨在检查免疫抑制治疗和患者特征作为 AQP4-IgG+NMOSD 疾病结局预测因素的影响。
这项针对 AQP4-IgG+NMOSD 患者的 MSBase 队列研究，在多变量比例风险模型中检查了复发的修饰因子，并使用混合效应模型检查了扩展残疾状况评分（EDSS）。
共纳入 206 例 AQP4-IgG+患者（中位随访 3.7 年）。年龄（每十年的风险比[HR]为 0.82，p=0.001）、脑干起病（HR 为 0.45，p=0.009）、硫唑嘌呤（HR 为 0.46，p<0.001）和霉酚酸酯（HR 为 0.09，p=0.012）与复发风险降低相关。更大的 EDSS 与年龄（β=0.45（每十年），p<0.001）和疾病持续时间（β=0.07 每年，p<0.001）相关。EDSS 增加速度较慢与硫唑嘌呤（β=-0.48，p<0.001）、霉酚酸酯（β=-0.69，p=0.04）和利妥昔单抗（β=-0.35，p=0.024）相关。
本研究表明，硫唑嘌呤和霉酚酸酯降低了复发的风险，而残疾的进展则通过硫唑嘌呤、霉酚酸酯和利妥昔单抗来改变。年龄和疾病持续时间是唯一改变我们队列中复发和残疾风险的患者特征。
