From the Departments of Ophthalmology (J.J.C., M.T.B.), Neurology (J.J.C., E.P.F., M.T.B., J.J., D.D., A.S.L.C., B.G.W., A.M., J.-M.T., V.A.L., C.F.L., A.K., S.J.P.), Laboratory Medicine and Pathology (E.P.F., J.J., D.D, J.P.F., A.M., V.A.L., S.J.P.), and Immunology (V.A.L.) and Center for MS and Autoimmune Neurology (E.P.F., D.D., B.G.W., A.M., V.A.L., C.F.L., A.K., S.J.P.), Mayo Clinic, Rochester, MN; Department of Ophthalmology and Visual Neurosciences (C.M.M., M.S.L.), University of Minnesota, Minneapolis; Departments of Neurology and Ophthalmology (J.L.B., V.S.P.), University of Colorado Denver School of Medicine, Aurora; Departments of Ophthalmology and Visual Sciences and Neurology (G.V.S.), Washington University, St. Louis School of Medicine, MO; Departments of Ophthalmology and Visual Science and Neurology (O.-O.O.A.), McGovern Medical School, Houston, TX; Departments of Neurology, Neurosurgery, and Neuro-Ophthalmology (E.R.E.), Mayo Clinic, Jacksonville, FL; Departments of Ophthalmology (M.D.A.) and Neurology (D.M.W.), Mayo Clinic, Scottsdale, AZ; Bascom Palmer Eye Institute (B.L.L.), University of Miami, FL; Department of Neurology and Ophthalmology (H.M., S.B.), Stanford University, Palo Alto, CA; Neuro-Ophthalmology (A.L.G.), Kaiser Permanente, Northern California, Vallejo; Department of Ophthalmology (V.S.), Baylor College of Medicine/Texas Children's Hospital, Houston; Department of Ophthalmology (G.A., D.M.C.), Massachusetts Eye and Ear Infirmary/Harvard Medical School, Boston; Department of Ophthalmology (G.H.), Boston Children's Hospital, Harvard Medical School, MA; and Neuro-Ophthalmology Unit (H.S.-K.), Department of Ophthalmology, Rabin Medical Center, Sackler School of Medicine, Tel Aviv University, Israel.
Neurology. 2020 Jul 14;95(2):e111-e120. doi: 10.1212/WNL.0000000000009758. Epub 2020 Jun 17.
Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments.
We determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy.
Seventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3-61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5).
This large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.
髓鞘少突胶质细胞糖蛋白免疫球蛋白 G（MOG-IgG）相关疾病（MOGAD）常表现为复发性中枢神经系统脱髓鞘发作。减少复发的最佳治疗方法尚不清楚。为了帮助确定长期免疫疗法在预防 MOGAD 患者复发中的疗效，我们进行了一项多中心回顾性研究，以确定各种治疗方法的复发率。
我们确定了接受各种形式的 MOGAD 长期免疫治疗的患者的复发频率。纳入标准为有≥1 次中枢神经系统脱髓鞘发作史、MOG-IgG 阳性和免疫治疗≥6 个月。对患者进行回顾性分析，以了解长期免疫治疗前后中枢神经系统脱髓鞘发作的情况。
共纳入 70 例患者。首发中枢神经系统脱髓鞘发作的中位年龄为 29 岁（范围 3-61 岁；33%＜18 岁），59%为女性。治疗前的中位年化复发率（ARR）为 1.6。在维持免疫治疗中，复发患者的比例如下：霉酚酸酯 74%（19 例中的 14 例；ARR 0.67）、利妥昔单抗 61%（36 例中的 22 例；ARR 0.59）、硫唑嘌呤 59%（22 例中的 13 例；ARR 0.2）和静脉注射免疫球蛋白（IVIG）20%（10 例中的 2 例；ARR 0）。这 4 种治疗方法的总体中位 ARR 为 0.3。接受多发性硬化症（MS）疾病修正治疗药物治疗的 9 例患者在治疗中均发生突破性复发（ARR 1.5）。
这项针对 MOGAD 患者的大型回顾性多中心研究表明，维持免疫治疗可减少复发性中枢神经系统脱髓鞘发作，ARR 最低的是维持 IVIG 治疗。传统的 MS 疾病修正治疗药物似乎无效。需要前瞻性随机对照研究来验证这些结论。
