抗水通道蛋白4阳性视神经脊髓炎频谱障碍和原发性Sjögren综合征的重叠综合征:对个体患者数据的系统回顾。

IF 3.2 3区 医学 Q2 RHEUMATOLOGY
Rheumatology International Pub Date : 2024-12-01 Epub Date: 2023-07-27 DOI:10.1007/s00296-023-05397-0
Chandra Bhushan Prasad, Chirag Rajkumar Kopp, Gsrsnk Naidu, Vishal Sharma, Durga Prasanna Misra, Vikas Agarwal, Aman Sharma
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引用次数: 0

摘要

由于同时存在视神经脊髓炎谱系障碍(NMOSD),中枢神经系统(CNS)受累可发生在原发性Sjögren综合征(pSS)中,这种疾病具有高度复发的过程,需要无限期的免疫抑制,如果不及早诊断,随着时间的推移,损伤会逐渐累积,导致永久性残疾和发病率。在这篇综述中,我们描述和概述了抗水通道蛋白4 (AQP4)抗体血清阳性NMOSD与pSS重叠病例的临床过程和结果。为了研究AQP4 + NMOSD与pSS的共存性,我们对主要数据库中病例报告和病例系列的个体患者数据进行了回顾。该研究提取了这些患者的临床人口学特征、影像学和实验室资料、治疗方法和结果。纳入标准要求患者血液和/或脑脊液(CSF)中抗aqp4或NMO-IgG自身抗体呈阳性,并至少表现出pSS和NMOSD的一种表现。在AQP4 + NMOSD与pSS重叠的44例患者中,女性41例(93.2%)。pSS发病的平均年龄为44.8±18.4岁,NMOSD发病的平均年龄为43.2±19.8岁。在20例(45.5%)患者中,NMOSD发生在pSS发病之前,13例(29.5%)NMOSD发生在pSS发病之后,11例(25%)患者同时出现。急性横脊髓炎31例(70.5%),视神经炎21例(47.7%),脑综合征14例(31.8%),急性脑干综合征10例(22.7%),后脑区综合征5例(11.4%),间脑临床综合征2例(4.5%)。急性期静脉注射甲基强的松龙40例(90.9%),血浆置换15例(34.1%),静脉注射免疫球蛋白10例(22.7%);诱导/维持治疗中,16例(36.4%)患者使用环磷酰胺,6例(13.6%)患者使用利妥昔单抗,16例(36.4%)患者使用硫唑嘌呤,10例(22.7%)患者使用霉酚酸酯。病程为单相2例(4.5%),复发27例(61.4%)。中位(IQR)随访时间为2.4(6)年,39例(88.6%)患者表现出改善,3例(6.8%)患者表现出稳定,2例(4.5%)患者表现出NMOSD症状恶化。在AQP4 + NMOSD和pSS的重叠综合征中,患者有一种神经致残障碍,可以模仿pSS的神经学表现,经常发生在pSS发病之前,有一个复发过程,对免疫抑制剂反应良好,需要无限期治疗。需要多中心合作研究来阐明这种罕见重叠综合征的自然历史和结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Overlap syndrome of anti-aquaporin 4 positive neuromyelitis optica spectrum disorder and primary Sjögren's syndrome: a systematic review of individual patient data.

Central nervous system (CNS) involvement can occur in primary Sjögren's syndrome (pSS) due to co-existing neuromyelitis optica spectrum disorder (NMOSD) which has a highly relapsing course requiring indefinite immunosuppression, and if not diagnosed early, damage accrual occurs over time leading to permanent disability and morbidity. In this review, we describe and outline the clinical course and outcomes of anti-aquaporin 4 (AQP4) antibody seropositive NMOSD with pSS overlap cases. To investigate the co-existence of AQP4 + NMOSD with pSS, we conducted a review of individual patient data from case reports and case series found in major databases. The study extracted clinico-demographic features, imaging and laboratory profiles, treatment approaches, and outcomes of these patients. Inclusion criteria for the review required patients to have positivity for anti-AQP4 or NMO-IgG autoantibodies in the blood and/or cerebrospinal fluid (CSF) and exhibit at least one manifestation of both pSS and NMOSD. In this overlap between AQP4 + NMOSD and pSS, 44 patients were included of whom 41 (93.2%) were females. The mean age of pSS onset was 44.8 ± 18.4 years and NMOSD onset was 43.2 ± 19.8 years. In 20 (45.5%) patients, NMOSD preceded pSS onset, 13 (29.5%) NMOSD occurred after pSS onset, and 11 (25%) patients had a simultaneous presentation. 31 (70.5%) patients experienced acute transverse myelitis, 21 (47.7%) optic neuritis, 14 (31.8%) cerebral syndrome, 10 (22.7%) acute brainstem syndrome, 5 (11.4%) area postrema syndrome, and 2 (4.5%) diencephalic clinical syndromes. For the treatment of acute phase, 40 (90.9%) patients received intravenous methylprednisolone, 15 (34.1%) received plasma exchange, and 10 (22.7%) received intravenous immunoglobulin; and for the induction/maintenance therapy, 16 (36.4%) patients received cyclophosphamide, 6 (13.6%) received rituximab, 16 (36.4%) received azathioprine, and 10 (22.7%) received mycophenolate mofetil. Disease course was monophasic in 2 (4.5%) and relapsing in 27 (61.4%) patients. At median (IQR) follow-up duration of 2.4 (6) years, 39 (88.6%) patients showed improvement, 3 (6.8%) showed stabilization and 2 (4.5%) showed worsening of their NMOSD manifestations. In this overlap syndrome of AQP4 + NMOSD and pSS, patients have a neurologically disabling disorder that can mimic neurological manifestations of pSS, frequently occurs prior to the onset of pSS, has a relapsing course, responds well to immunosuppressants, and necessitates indefinite treatment. Collaborative multicentre studies are needed to clarify the natural history and outcomes of this rare overlap syndrome.

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来源期刊
Rheumatology International
Rheumatology International 医学-风湿病学
CiteScore
7.30
自引率
5.00%
发文量
191
审稿时长
16. months
期刊介绍: RHEUMATOLOGY INTERNATIONAL is an independent journal reflecting world-wide progress in the research, diagnosis and treatment of the various rheumatic diseases. It is designed to serve researchers and clinicians in the field of rheumatology. RHEUMATOLOGY INTERNATIONAL will cover all modern trends in clinical research as well as in the management of rheumatic diseases. Special emphasis will be given to public health issues related to rheumatic diseases, applying rheumatology research to clinical practice, epidemiology of rheumatic diseases, diagnostic tests for rheumatic diseases, patient reported outcomes (PROs) in rheumatology and evidence on education of rheumatology. Contributions to these topics will appear in the form of original publications, short communications, editorials, and reviews. "Letters to the editor" will be welcome as an enhancement to discussion. Basic science research, including in vitro or animal studies, is discouraged to submit, as we will only review studies on humans with an epidemological or clinical perspective. Case reports without a proper review of the literatura (Case-based Reviews) will not be published. Every effort will be made to ensure speed of publication while maintaining a high standard of contents and production. Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.
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