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系统性红斑狼疮性横断性脊髓炎的复发率和不良神经预后的危险因素:系统评价和荟萃分析。

Relapse rates and risk factors for unfavorable neurological prognosis of transverse myelitis in systemic lupus erythematosus: A systematic review and meta-analysis.

机构信息

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

出版信息

Autoimmun Rev. 2022 Feb;21(2):102996. doi: 10.1016/j.autrev.2021.102996. Epub 2021 Nov 16.

Abstract

BACKGROUND

Transverse myelitis (TM) is a rare but severe systemic lupus erythematosus (SLE) manifestation. To date, the prognostic factors for SLE-associated TM have been far less well-studied. There are also controversial data on the association of antiphospholipid antibodies (aPLs), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score, longitudinal extensive transverse myelitis (LETM), and decreased complement levels with the outcome of TM. We aimed to review the potential prognostic factors and integrate relapse rates of observational studies for SLE-associated TM.

METHOD

To review the prognosis for SLE-associated TM, relevant articles published up to July 30, 2021, were comprehensively and systematically identified from PubMed, EMBASE and Web of Science databases. Five studies encompassing 283 patients with SLE-related TM were included in this meta-analysis; raw data were obtained from three studies.

RESULTS

The risk factors for unfavorable neurological outcome included demographic features, clinical characteristics, laboratory data, among which a grade of A, B or C on the American Spinal Injury Association Impairment Scale (AIS) at the onset of TM was associated with poor prognosis (OR: 56.05, 95% CI: 6.29-499.25, P < 0.001). The presence of hypoglycorrhachia was also correlated with a worse prognosis (OR: 10.78, 95% CI: 3.74-31.07, P < 0.001). No noticeable correlation was revealed between a poor outcome and positive aPLs and different aPLs profiles (anticardiolipin antibody [aCL], anti-β2-glycoprotein I (anti-β2GPI], lupus anticoagulant [LA]). The pooled 1-, 3- and 5-year relapse rates were 22% (95% CI: 0.13-0.31), 34% (95% CI: 0.22-0.47) and 36% (95% CI: 0.14-0.58), respectively. No significant publication bias was found.

CONCLUSION

A grade of A, B, or C on the AIS at initial TM and the presence of hypoglycorrhachia were found to be related to a worse prognosis in patients with SLE-associated TM. Notably, aPLs and different aPLs profiles may not suggest poor neurological outcome. The long-term relapse rate of patients with SLE-associated TM was relatively high. We recommend that treatment be stratified based on the initial severity of myelitis. For patients with severe myelitis, early intensive therapy may be initiated as soon as possible.

摘要

背景

横贯性脊髓炎(TM)是一种罕见但严重的系统性红斑狼疮(SLE)表现。迄今为止,SLE 相关性 TM 的预后因素研究得还远远不够充分。抗磷脂抗体(aPLs)、系统性红斑狼疮疾病活动指数(SLEDAI)评分、长节段横贯性脊髓炎(LETM)和补体水平降低与 TM 结局之间的相关性数据也存在争议。我们旨在综述与 TM 相关的 SLE 的潜在预后因素,并整合观察性研究的复发率。

方法

为了综述与 SLE 相关的 TM 的预后,我们全面、系统地检索了 PubMed、EMBASE 和 Web of Science 数据库中截至 2021 年 7 月 30 日发表的相关文章。这项荟萃分析纳入了 5 项共 283 例 SLE 相关性 TM 患者的研究;其中 3 项研究提供了原始数据。

结果

不良神经结局的危险因素包括人口统计学特征、临床特征、实验室数据,其中 TM 发病时美国脊髓损伤协会损伤量表(AIS)评分为 A、B 或 C 级与预后不良相关(OR:56.05,95%CI:6.29-499.25,P<0.001)。低颅压也与预后不良相关(OR:10.78,95%CI:3.74-31.07,P<0.001)。未发现不良预后与 aPLs 阳性和不同 aPLs 谱(抗心磷脂抗体[aCL]、抗-β2-糖蛋白 I [抗-β2GPI]、狼疮抗凝物 [LA])之间存在明显相关性。汇总的 1 年、3 年和 5 年复发率分别为 22%(95%CI:0.13-0.31)、34%(95%CI:0.22-0.47)和 36%(95%CI:0.14-0.58)。未发现明显的发表偏倚。

结论

SLE 相关性 TM 患者 AIS 初始 TM 评分为 A、B 或 C 级和低颅压与预后不良相关。值得注意的是,aPLs 和不同的 aPLs 谱可能并不预示不良的神经结局。SLE 相关性 TM 患者的长期复发率相对较高。我们建议根据初始脊髓炎的严重程度对治疗进行分层。对于严重脊髓炎患者,应尽早开始强化治疗。

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