"Lupus Myelitis" Revisited: A Retrospective Single-Center Study of Myelitis Associated With Rheumatologic Disease.

IF 7.8 1区 医学 Q1 CLINICAL NEUROLOGY
Jonathan D Krett, Angeliki G Filippatou, Paula Barreras, Carlos A Pardo, Allan C Gelber, Elias S Sotirchos
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引用次数: 0

Abstract

Background and objectives: Previous reports of patients with myelitis associated with rheumatologic disease may have had unrecognized aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD). We clinicoradiologically and serologically characterized patients with myelitis associated with rheumatologic disease evaluated in the era of availability of MOG-IgG and more sensitive AQP4-IgG cell-based assays.

Methods: A retrospective cohort (2018-2023) at Johns Hopkins Medicine with diagnoses of myelopathy and rheumatologic comorbidity was identified by electronic medical record (EMR) query. All patients with myelitis unrelated to typical multiple sclerosis (MS) were included and analyzed by chart review.

Results: Of 238 patients identified by EMR query, 197 were excluded (148 not meeting prespecified inclusion criteria, 49 had typical MS), resulting in 41 patients for review. The mean age at myelitis onset was 44 ± 15 years; 39 (95%) were female. Rheumatologic diagnoses included 17 (41.5%) with systemic lupus erythematosus (SLE), 10 (24.3%) Sjögren syndrome (SS), 6 (15%) undifferentiated connective tissue disease (UCTD), 5 (12%) combinations of SLE/SS/UCTD with antiphospholipid antibody syndrome, 1 (2.4%) rheumatoid arthritis, 1 (2.4%) psoriatic arthritis, and 1 (2.4%) Behçet disease. 20 patients (49%) were diagnosed with AQP4-IgG seropositive NMOSD, 3 (7%) with MOGAD, and 18 (44%) had "double-seronegative" myelitis. Of these 18, 3 were diagnosed with AQP4-IgG seronegative NMOSD, 1 neuro-Behçet disease, and 14 other (unclassifiable) myelitis. Excluding 1 patient with neuro-Behçet disease, 18 (90%) of 20 AQP4-IgG seropositive patients had longitudinally extensive cord lesions compared with 5 (29%; p < 0.001) of 17 "double-seronegative" patients and 2 (67%) of 3 with MOGAD. "Double-seronegative" patients more commonly had CSF-restricted oligoclonal bands. Functional outcomes did not differ by diagnosis, and most patients received acute immunotherapy at the time of initial myelitis diagnosis with at least partial recovery over a median follow-up of 38 (interquartile range: 9-74) months.

Discussion: Approximately half of our rheumatologic disease cohort with myelitis unrelated to MS had AQP4-IgG seropositive NMOSD while MOGAD accounted for a small but clinically relevant proportion of patients. Further research is needed to characterize myelitis etiology in patients who are seronegative for both AQP4-IgG and MOG-IgG.

"狼疮性脊髓炎 "重新审视:风湿病相关骨髓炎的单中心回顾性研究
背景和目的:以前报道的与风湿病相关的脊髓炎患者中可能有未被发现的水通道蛋白-4(AQP4)-IgG血清阳性神经脊髓炎视谱系障碍(NMOSD)或髓鞘少突胶质细胞糖蛋白(MOG)-IgG相关疾病(MOGAD)。我们从临床放射学和血清学的角度对与风湿病相关的脊髓炎患者进行了评估,评估是在可以使用MOG-IgG和更灵敏的AQP4-IgG细胞检测的时代进行的:通过电子病历(EMR)查询,确定了约翰霍普金斯医学院诊断为脊髓病和风湿病合并症的回顾性队列(2018-2023 年)。所有与典型多发性硬化症(MS)无关的脊髓炎患者都被纳入其中,并通过病历审查进行分析:在通过电子病历查询确定的 238 名患者中,有 197 人被排除在外(148 人不符合预先确定的纳入标准,49 人患有典型多发性硬化症),最终有 41 名患者接受了复查。脊髓炎发病时的平均年龄为 44 ± 15 岁,其中 39 人(95%)为女性。风湿病诊断包括 17 例(41.5%)系统性红斑狼疮(SLE)、10 例(24.3%)患有斯约格伦综合征(SS),6 人(15%)患有未分化结缔组织病(UCTD),5 人(12%)合并有系统性红斑狼疮/SS/UCTD 和抗磷脂抗体综合征,1 人(2.4%)患有类风湿性关节炎,1 人(2.4%)患有银屑病关节炎,1 人(2.4%)患有白塞病。20名患者(49%)被诊断为AQP4-IgG血清反应阳性的NMOSD,3名患者(7%)被诊断为MOGAD,18名患者(44%)患有 "双酮体阴性 "脊髓炎。在这18人中,3人被诊断为AQP4-IgG血清反应阴性的NMOSD,1人被诊断为神经-贝赫切特病,14人被诊断为其他(无法分类的)脊髓炎。除去1名神经-贝赫切特病患者,20名AQP4-IgG血清反应阳性患者中有18名(90%)有纵向广泛的脊髓病变,而17名 "双克隆阴性 "患者中有5名(29%;p < 0.001)和3名MOGAD患者中有2名(67%)有纵向广泛的脊髓病变。"双克隆阴性 "患者更常出现脑脊液限制性寡克隆带。大多数患者在最初确诊脊髓炎时接受了急性免疫治疗,中位随访时间为38个月(四分位间范围:9-74个月),至少部分康复:讨论:在与多发性硬化症无关的脊髓炎风湿病队列中,约有半数患者的AQP4-IgG血清反应呈NMOSD阳性,而MOGAD患者所占比例虽小,但具有临床意义。对于AQP4-IgG和MOG-IgG血清均阴性的脊髓炎患者,还需要进一步研究其病因。
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来源期刊
CiteScore
15.60
自引率
2.30%
发文量
219
审稿时长
8 weeks
期刊介绍: Neurology Neuroimmunology & Neuroinflammation is an official journal of the American Academy of Neurology. Neurology: Neuroimmunology & Neuroinflammation will be the premier peer-reviewed journal in neuroimmunology and neuroinflammation. This journal publishes rigorously peer-reviewed open-access reports of original research and in-depth reviews of topics in neuroimmunology & neuroinflammation, affecting the full range of neurologic diseases including (but not limited to) Alzheimer's disease, Parkinson's disease, ALS, tauopathy, and stroke; multiple sclerosis and NMO; inflammatory peripheral nerve and muscle disease, Guillain-Barré and myasthenia gravis; nervous system infection; paraneoplastic syndromes, noninfectious encephalitides and other antibody-mediated disorders; and psychiatric and neurodevelopmental disorders. Clinical trials, instructive case reports, and small case series will also be featured.
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