Clinical and laboratory features distinguishing MOG antibody disease from multiple sclerosis and AQP4 antibody-positive neuromyelitis optica
Clinical and laboratory features distinguishing MOG antibody disease from multiple sclerosis and AQP4 antibody-positive neuromyelitis optica
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DOI：
10.1016/j.msard.2020.102399
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发表时间：
2020-10-01
影响因子：
4
通讯作者：
Cross, Anne H.
中科院分区：
文献类型：
--
作者：
Ciotti, John R.;Eby, Noah S.;Cross, Anne H.
背景：髓鞘少突胶质细胞糖蛋白（MOG）抗体与中枢神经系统炎症性疾病有关，不同于多发性硬化症（MS）和水通道蛋白-4抗体阳性的视神经脊髓炎（NMO）。MOG抗体病（MOGAD）的临床谱知识仍然不完整，特别是与两种相关的炎症性脱髓鞘疾病MS和NMO相比。目的：比较年龄和性别匹配的MS和NMO患者的美国MOGAD队列的人口统计学、临床特征、估计残疾、实验室结果和治疗反应。设计、环境和参与者：这项观察性、病例对照、单中心研究通过ICD-10诊断代码搜索，通过John L. Trotter MS中心在2019年1月1日至2020年1月1日期间看到的成年患者的电子病历，确定了每组。MOGAD和NMO患者被确认至少有一项抗体检测阳性；多发性硬化症组由接受过多发性硬化症亚专科培训的医生确诊。数据在IRB批准后收集。结果：每组26例。MOGAD患者主要为白种人（88.5%），平均发病年龄为43.9岁。MOGAD患者没有其他自身免疫性疾病的合并症，家庭成员患有自身免疫性疾病的比例（20.0%）相对低于MS（40.0%）或NMO（34.6%）匹配的队列。91%的MOGAD发作为单灶性，70%以上表现为视神经炎。MOGAD发作的严重程度与血清NMO阳性相似，但恢复的稳健程度更接近ms。4例MOGAD患者转化为抗体阴性，转化后无发作。MOGAD患者血清ANA和ENA升高的频率（21.7%,5.0%）低于血清阳性NMO患者（66.7%,42.9%）。在我们的MOGAD队列中未见IgG合成率升高和csf限制性寡克隆带阳性，只有1例MOGAD患者IgG指数升高。尽管抗cd20治疗，28.6%的MOGAD患者继续遭受复发。结论：MOGAD的特征主要是单灶性表现（典型的视神经炎）和严重的发作，比血清NMO阳性发作的恢复更好。缺乏限制csf的寡克隆条带将MOGAD与MS区分开来。
Background: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with a CNS inflammatory disorder distinct from multiple sclerosis (MS) and aquaporin-4 antibody-positive neuromyelitis optica (NMO). Knowledge of the clinical spectrum of MOG antibody disease (MOGAD) remains incomplete, particularly in comparison to two related inflammatory demyelinating diseases, MS and NMO.Objective: Compare demographics, clinical characteristics, estimated disability, laboratory results, and treatment responses of a U.S. MOGAD cohort with age- and sex-matched MS and NMO patients.Design, setting, and participants: This observational, case-control, single-center study identified each group via ICD-10 diagnosis code searches through the electronic medical records of adult patients seen at the John L. Trotter MS Center between January 1, 2019 and January 1, 2020. MOGAD and NMO patients were confirmed to have at least one positive antibody test; those in the MS group had a confirmed diagnosis by a physician with MS subspecialty training. Data were collected after IRB approval.Results: Twenty-six patients were included in each group. MOGAD patients were predominantly Caucasian (88.5%) with mean onset age of 43.9 years. MOGAD patients had no comorbid other autoimmune diseases and comparatively lower rates of family members with autoimmune disease (20.0%) than either MS (40.0%) or NMO (34.6%) matched cohorts. 91% of MOGAD attacks were monofocal, and over 70% presented with optic neuritis. Severity of MOGAD attacks was similar to that of seropositive NMO, but the robust degree of recovery was more similar to MS. Four MOGAD patients converted to negative antibody status, with no attacks occurring after conversion. Serum ANA and ENA were less frequently elevated in MOGAD (21.7%, 5.0%) than in seropositive NMO patients (66.7%, 42.9%). Elevated IgG synthesis rate and positive CSF-restricted oligoclonal bands were not seen in our MOGAD cohort, and only one MOGAD patient had an elevated IgG index. Despite anti-CD20 therapy, 28.6% of MOGAD patients continued to suffer relapses.Conclusions: MOGAD was characterized by a predominantly monofocal presentation (typically optic neuritis) and severe attacks with better recovery than seen with seropositive NMO attacks. Lack of CSF-restricted oligoclonal bands distinguished MOGAD from MS.