From the Department of Neurology (R.D.); Clinical Research Department (J.G.), Rothschild Foundation Hospital, Paris; Department of Neurology (J.C.), University Hospital of Toulouse; Department of Neurology (B.A.), Pôle de Neurosciences Cliniques, APHM, Aix Marseille University, Hôpital de la Timone; Department of Neurology (A.R.), University Hospital of Bordeaux; Centre de référence des maladies inflammatoires rares du cerveau et de la moelle (MIRCEM) (E.M., C.G.), Department of Neurology, AP-HP, Pitié-Salpêtrière Hospital, Paris; Centre de référence des maladies inflammatoires rares du cerveau et de la moelle (MIRCEM) (J.P., L.B., R.M.), Department of Neurology, sclérose en plaques, pathologies de la myéline et neuro-inflammation, Hôpital Neurologique Pierre Wertheimer; Department of Neurology (D.A.L.), CR2TI-Inserm U1064, CIC1314, Nantes Université, University Hospital of Nantes; Department of Neurology (L. Michel), INSERM UMR 1236, Université de Rennes 1, University Hospital of Rennes, Rennes, France; Center for Clinical Investigation (N.C.), INSERM U1434; Biopathology of Myelin, Neuroprotection and Therapeutic Strategy, INSERM U1119; Department of Neurology, University Hospital of Strasbourg; CRC SEP Neurologie Pasteur 2 (M.C.), University Hospital of Nice, Université Cote d'Azur, UMR2CA (URRIS), Nice; CRC SEP (X.A.), CRMR Leukofrance, Department of Neurology, INM / U1298, Gui de Chauliac Hospital, University Hospital of Montpellier; Department of Neurology (E.T.), University Hospital of Nimes; CRC SEP (H.Z.), Department of Neurology, U 1172, University Hospital of Lille; Department of Neurology (B.B.), University Hospital of Rouen; Neuro Ophthalmology Unit (C.F.T.), Hospices Civils de Lyon, Claude Bernard Lyon 1 University, Lyon Neuroscience Research Center CRNL U1028 UMR 5292, IMPACT F-69500, Neurological Hospital of Lyon; Department of Neurology (T.M.), University Hospital of Dijon; Department of Neurology (P.C.), University Hospital of la Rochelle; Department of Neurology (P.K.), Hospital of Luxembourg, Luxembourg-Ville, Luxembourg; Department of Child Neurology and Child Intensive Care Unit (S.C.), Hospital of Pau; Department of Neurology (N. Maubeuge), University Hospital of Poitiers; Department of Neurology (K.H.), Delafontaine Hospital, Saint-Denis; Department of Neurology (C.N.), Andre Mignot hospital, Hospital of Versailles, Le Chesnay; Department of Neurology (E.B.), University Hospital of Besancon; Department of Neurology (H.M.), Sud Francilien Hospital, Corbeil-Essonnes; CRC SEP Limoges/Poitiers (L. Magy), CRMR Maladies Neuromusculaires AOC (FILNEMUS), UR 2018 NeurIT, University Hospital of Limoges; Department of Neurology (F.K.), Meaux Hospital; Department of Neurology (M.S.R.), University Hospital of Bicêtre, Le Kremlin-Bicêtre; Department of Neurology (L.H.), Felix Guyon University Hospital, Saint Denis, Reunion; Department of Neurology (M.G.), Hospital of Valenciennes; Department of Neurology (P.B.), University Hospital of Caen Normandie; CRC SEP (A.M.), Department of Neurology, University Hospital of Tours, Bretonneau Hospital; Department of Neurology (G.M.), Nancy University Hospital; Université de Lorraine, EA 4360 APEMAC, Vandoeuvre-Lès-Nancy; Department of Neurology (M.M.), University Hospital of Reims; Department of Neurology (N. Mélé), Sainte-Anne Hospital, GHU Paris Psychiatrie et Neurosciences, Paris Cité University, INSERM 1266; and Department of Neurology (C.P.), Rothschild Foundation Hospital, Paris, France.
Neurology. 2024 Aug 13;103(3):e209624. doi: 10.1212/WNL.0000000000209624. Epub 2024 Jul 11.
Because myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently identified autoimmune disorder, the natural history of MOGAD is still not well described. The objective of this study was to describe the long-term outcomes of adult patients with MOGAD. In addition, we aimed to identify factors affecting relapse risk and neurologic outcomes.
Clinical and biological data were obtained from patients with a first event of MOGAD and included in the French nationwide incident cohort between February 2014 and March 2017. Only patients aged 18 years or older at disease onset and with observation period of at least 3 months were included. Data were collected prospectively until July 2023 and registered in the dedicated French nationwide database. This form includes every relapse with phenotype description during follow-up, date of last assessment, final clinical outcome with Expanded Disability Status Scale score and visual acuity, and maintenance therapy. The probability of recurrence-free survival was assessed using the Kaplan-Meier method.
We included 128 patients. The onset phenotype was isolated optic neuritis in 81 patients (63.3%) and isolated myelitis in 25 patients (19.5%). The median follow-up duration was 77.8 months (range 3.2-111.2), with 49 patients (38.3%) experienced at least one relapse. Median times from onset to second and third attacks were 3.2 (1.0-86.2) and 13.0 (2.6-64.4) months, respectively. At the last assessment, Expanded Disability Status Scale Score was ≥3 and ≥6 in 22 (17.2%) and 6 (4.7%) patients, respectively. Eighty patients received at least one maintenance treatment. This treatment was initiated after the first attack in 47 patients (36.7% of the whole cohort) and at the time of a second attack in 25 (19.5%). Multivariate analysis revealed that initiating maintenance treatment after the first attack was associated with a lower relapse risk (OR = 0.26 [95% CI 0.11-0.62], = 0.002). In patients receiving maintenance therapy after first attack, the 2-year, 4-year, 6-year, and 8-year relapse risks were 11%, 15%, 20%, and 20%, respectively. In other patients, the risks were 41%, 46%, 51%, and 56%.
The highest risk of a relapse in MOGAD occurs early, and initiating maintenance therapy from the first attack substantially reduced the relapse risk.
This study provides Class III evidence that initiating maintenance therapy from the first attack in patients with MOGAD reduces the relapse risk.
由于髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）是一种新近确定的自身免疫性疾病，因此其自然病程仍未得到充分描述。本研究的目的是描述成年 MOGAD 患者的长期结局。此外，我们旨在确定影响复发风险和神经结局的因素。
从 2014 年 2 月至 2017 年 3 月期间参与法国全国性发病队列的首次 MOGAD 发作的患者中获取临床和生物学数据并将其纳入研究。仅纳入发病时年龄在 18 岁及以上且观察期至少 3 个月的患者。数据前瞻性收集，直至 2023 年 7 月，并在专门的法国全国性数据库中登记。该表格包括随访期间每次复发的表型描述、末次评估日期、最后临床结局（扩展残疾状况量表评分和视力）和维持治疗。使用 Kaplan-Meier 法评估无复发生存率。
我们纳入了 128 例患者。首发表现为孤立性视神经炎的患者 81 例（63.3%），孤立性脊髓炎的患者 25 例（19.5%）。中位随访时间为 77.8 个月（范围 3.2-111.2），49 例（38.3%）至少经历过一次复发。从发病到第二次和第三次发作的中位时间分别为 3.2（1.0-86.2）和 13.0（2.6-64.4）个月。末次评估时，扩展残疾状况量表评分≥3 和≥6 的患者分别为 22 例（17.2%）和 6 例（4.7%）。80 例患者接受了至少一种维持治疗。47 例（整个队列的 36.7%）在首次发作后开始维持治疗，25 例（19.5%）在第二次发作时开始维持治疗。多变量分析显示，首次发作后开始维持治疗与较低的复发风险相关（OR = 0.26 [95%CI 0.11-0.62]， = 0.002）。在首次发作后接受维持治疗的患者中，2 年、4 年、6 年和 8 年的复发风险分别为 11%、15%、20%和 20%。在其他患者中，风险分别为 41%、46%、51%和 56%。
MOGAD 复发的最高风险发生在早期，从首次发作开始即进行维持治疗可显著降低复发风险。
本研究提供了 III 级证据，表明在 MOGAD 患者中从首次发作开始进行维持治疗可降低复发风险。
