From the Department of Neuroscience (P.S., A.V.D.W., P.G.S., Y.C.F., W.Z.Y., C.Z., V.G.J., H.B., M.M.), Central Clinical School, Monash University, Melbourne, Victoria; Department of Neurology (P.S., A.V.D.W., P.G.S., Y.C.F., W.Z.Y., V.G.J., H.B., M.M.), Alfred Health, Melbourne, Victoria, Australia; Department of Neurology (P.S., S.H.), Walton Centre NHS Foundation Trust, Liverpool, United Kingdom; Neuroimmunology Centre (S.S., I.R., T.K.), Department of Neurology, The Royal Melbourne Hospital, Parkville; CORe (S.S., I.R., T.K.), Department of Medicine, University of Melbourne, Victoria; Royal Hobart Hospital (Y.C.F.), Hobart, Tasmania, Australia; Nehme and Therese Tohme Multiple Sclerosis Center (S.J.K.), American University of Beirut Medical Center, Beirut, Lebanon; Department of Neurology (T.C.), Faculty of Medicine, University of Debrecen, Hungary; Department of Neurology (B.W.), Antwerp University Hospital, Edegem; Translational Neurosciences Research Group (B.W.), Faculty of Medicine and Health Sciences, University of Antwerp, Wilrijk, Belgium; Faculty of Medicine (M.E.), Isfahan University of Medical Sciences; Neurology (M.E.), Dr. Etemadifar MS Institute, Isfahan, Iran; Izmir University of Economics (S.O.), Medical Point Hospital; Multiple Sclerosis Research Association (S.O.), Izmir, Turkey; Department of Neurology and Center of Clinical Neuroscience (P.N., D.H.), First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic; Department of Neurology (A.A.), School of Medicine and Koc University Research Center for Translational Medicine (KUTTAM), Koc University, Istanbul, Turkey; College of Medicine & Health Sciences and Sultan Qaboos University Hospital (A.A.-A.), Sultan Qaboos University, Al-Khodh, Oman; Department of Neuroscience (C.M.R.-T.), Hospital Germans Trias I Pujol, Badalona, Spain; Department of Neurology (G.L.), University Hospital Ghent, Belgium; Department of Medical and Surgical Sciences and Advanced Technologies (F.P.), GF Ingrassia, Catania, Italy; Multiple Sclerosis Unit (F.P.), AOU Policlinico G Rodolico-San Marco, University of Catania; Department of Neuroscience (M.F.), MS Center, Neurology Unit, S. Maria delle Croci Hospital, AUSL Romagna, Ravenna, Italy; Department of Biotechnological and Applied Clinical Sciences (DISCAB) (M.F.), University of L'Aquila, Italy; Department of Neurology (C.B.), Karadeniz Technical University, Medical Faculty, Trabzon, Turkey; Department of Neurology (P.A.M.), Royal Brisbane Hospital; University of Queensland (P.A.M.), Australia; Department of Neurology (R.T.), Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey; Hunter Medical Research Institute (J.L.-S.), Neurology, University of Newcastle; and Hunter New England Health (J.L.-S.), John Hunter Hospital, New South Wales, Australia.
Neurology. 2024 Dec 24;103(12):e209940. doi: 10.1212/WNL.0000000000209940. Epub 2024 Nov 19.
In aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), disability accrual is mostly attributed to relapses. This study aimed to assess the prevalence of progression independent of relapse activity (PIRA) and relapse-associated worsening (RAW) in AQP4-IgG NMOSD.
This was a retrospective cohort study of patients with AQP4-IgG NMOSD enrolled in the MSBase international data registry. Patients required a minimum of 3 recorded Expanded Disability Status Scale (EDSS) scores: baseline, event, and a 6-month confirmation score. Presence and absence of relapses between the baseline and event EDSS scores determined RAW and PIRA, respectively. Descriptive statistics were used to present the results.
A total of 181 patients followed for a median of 4.5 years (Q1 1.7, Q3 7.8) were included. Most patients were female (88.4%), and the median age at disease onset was 38.1 years. Overall, 4 patients (2.2%) developed 5 incidences of PIRA and 13 patients developed RAW (7.2%).
This multicenter study highlights that PIRA is very rare in AQP4-IgG NMOSD. Limitations of this study include the sole focus of overall EDSS to measure disability, lack of requirement for a second EDSS score to confirm baseline EDSS, and the absence of magnetic resonance imaging information for all patients.
在水通道蛋白-4 抗体阳性视神经脊髓炎谱系疾病（AQP4-IgG NMOSD）中，残疾的累积主要归因于复发。本研究旨在评估与复发活动无关的进展（PIRA）和与复发相关的恶化（RAW）在 AQP4-IgG NMOSD 中的发生率。
这是一项回顾性队列研究，纳入了 AQP4-IgG NMOSD 患者，这些患者均来自 MSBase 国际数据注册中心。患者需要至少有 3 次记录的扩展残疾状况量表（EDSS）评分：基线、事件和 6 个月的确认评分。基线和事件 EDSS 评分之间的复发存在与否分别确定了 RAW 和 PIRA。使用描述性统计来呈现结果。
共纳入了 181 名患者，中位随访时间为 4.5 年（Q1 1.7，Q3 7.8）。大多数患者为女性（88.4%），疾病发病的中位年龄为 38.1 岁。总体而言，有 4 名患者（2.2%）发生了 5 次 PIRA，13 名患者发生了 RAW（7.2%）。
这项多中心研究表明，AQP4-IgG NMOSD 中 PIRA 非常罕见。本研究的局限性包括仅使用整体 EDSS 来衡量残疾、缺乏对基线 EDSS 进行第二次 EDSS 评分以确认以及并非所有患者都有磁共振成像信息。
