Demyelinating syndrome in SLE encompasses different subtypes: Do we need new classification criteria? Pooled results from systematic literature review and monocentric cohort analysis
Demyelinating syndrome in SLE encompasses different subtypes: Do we need new classification criteria? Pooled results from systematic literature review and monocentric cohort analysis
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DOI：
10.1016/j.autrev.2017.01.011
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发表时间：
2017-03-01
影响因子：
13.6
通讯作者：
Cauli, Alberto
中科院分区：
文献类型：
--
作者：
Piga, Matteo;Chessa, Elisabetta;Cauli, Alberto
目的：目的：探讨系统性红斑狼疮（SLE）脱髓鞘综合征（DS）的临床特征。方法：采用PubMed中的Mesh术语对343例成人SLE患者进行系统回顾性分析。根据1999年ACR命名法，将检索到的病例归类为DS受累，并应用2015年算法归因于SLE。结果：SLE队列中DS（包括CIS）的估计患病率为1.3%，发病率为1.5例/1000患者年。总体而言，从文献综述中确定了100例病例，从SLE队列中确定了4例病例，并将其作为一个整体列出：49例（47.1%）被归类为视神经肌病谱系疾病（NMOSD），29例（27.9%）被归类为CIS，14例（13.5%）被归类为NMO，7例（6.7%）被归类为主要累及脑干的DS，5例（4.8%）被归类为主要累及大脑的DS。41例（39.4%）SLE患者以DS为首发表现。73例（70.2%）患者（NMOSD 37例，CIS 21例，NMO 14例，DSB 1例）中最常见的表现是纵向广泛的横肌萎缩。甲基强的松龙（79.8%）和环磷酰胺（55.8%）脉冲，但也血浆置换（16.3%）和利妥昔单抗（7.6%）在复发难治性病例中，大多数处方。完全恢复率范围从CIS的62%到NMO的7%。结论：DS在SLE中是罕见的（1%），包括不同的亚型，包括CIS。建议及时诊断和早期治疗，以减少并发症。(C)2017爱思唯尔B. V.保留所有权利。
Objective: To describe features of demyelinating syndrome (DS) in systemic lupus erythematosus (SLE).Methods: A systematic review using a combination of Mesh terms in PubMed and a retrospective analysis of 343 adult patients with SLE were carried out to identify patients with DS. Retrieved cases were classified as affected with DS according to 1999 ACR nomenclature and attributed to SLE by applying the 2015 algorithm. DS defined according to the clinical but not temporal 1999 ACR criteria was classified as clinically isolated syndrome (CIS).Results: Estimated prevalence of DS (including CIS) in the SLE cohort was 1.3% and incidence rate was 1.5 cases per 1000 patient-years. Overall, 100 cases from literature review and 4 from SLE cohort were identified and are presented as a whole: 49 (47.1%) were classified as neuromyelitis optica spectrum disorders (NMOSD), 29 (27.9%) as CIS, 14 (13.5%) as NMO, 7 (6.7%) as DS prominently involving the brainstem and 5 (4.8%) as DS prominently involving the brain. DS was the SLE onset manifestation in 41 (39.4%) patients. Longitudinally extensive transverse myelitis was the most frequent manifestations being present in 73 (70.2%) patients (37 NMOSD, 21 CIS, 14 NMO, 1 DSB). Methylprednisolone (79.8%) and cyclophosphamide (55.8%) pulses, but also plasma-exchange (16.3%) and rituximab (7.6%) in relapsing-refractory cases, were mostly prescribed. Complete recovery rate ranged between 62% in CIS to 7% in NMO.Conclusion: DS in SLE is rare (1%) and encompasses different subtypes including CIS. Timely diagnosis and early treatment are recommended to minimize complications. (C) 2017 Elsevier B.V. All rights reserved.