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系统性红斑狼疮中的横断性脊髓炎:拉丁美洲大样本患者的临床特征和预后因素。

Transverse Myelitis in Systemic Lupus Erythematosus: Clinical Features and Prognostic Factors in a Large Cohort of Latin American Patients.

机构信息

From the Departments of Immunology and Histocompatibility.

Neurology, Hospital Carlos G. Durand.

出版信息

J Clin Rheumatol. 2021 Sep 1;27(6S):S204-S211. doi: 10.1097/RHU.0000000000001322.

Abstract

BACKGROUND

Acute transverse myelitis (ATM) is an infrequent but severe complication of systemic lupus erythematosus (SLE). The purpose of study was to describe clinical features and prognostic factors of patients with SLE-related ATM.

METHODS

In this medical records review study, data were collected from 60 patients from 16 centers seen between 1996 and 2017 who met diagnostic criteria for SLE and myelitis as defined by the American College of Rheumatology/Systemic International Collaborating Clinics and the Working Group of the Transverse Myelitis Consortium, respectively. Objective neurological impairment was measured with American Spinal Injury Association Impairment Scale (AIS) and European Database for Multiple Sclerosis Grade Scale (EGS).

RESULTS

Among patients included, 95% (n = 57) were female, and the average age was 31.6 ± 9.6 years. Myelitis developed after diagnosis of SLE in 60% (n = 36). Symmetrical paraparesis with hypoesthesia, flaccidity, sphincter dysfunction, AIS = A/B, and EGS ≥ 8 was the most common presentation. Intravenous methylprednisolone was used in 95% (n = 57), and 78.3% (n = 47) received intravenous cyclophosphamide. Sensory/motor recovery at 6 months was observed in 75% (42 of 56), but only in 16.1% (9 of 56) was complete. Hypoglycorrhachia and EGS ≥ 7 in the nadir were associated with an unfavorable neurological outcome at 6 months (p < 0.05). A relapse rate during follow-up was observed in 30.4% (17 of 56). Hypoglycorrhachia and hypocomplementemia seem to be protective factors for relapse. Intravenous cyclophosphamide was associated with time delay to relapse.

CONCLUSIONS

Systemic lupus erythematosus-related ATM may occur at any time of SLE course, leading to significant disability despite treatment. Relapses are infrequent and intravenous cyclophosphamide seems to delay it. Hypoglycorrhachia, hypocomplementemia, and EGS at nadir are the most important prognostic factors.

摘要

背景

急性横贯性脊髓炎(ATM)是系统性红斑狼疮(SLE)罕见但严重的并发症。本研究旨在描述与 SLE 相关的 ATM 患者的临床特征和预后因素。

方法

在这项病历回顾研究中,从 1996 年至 2017 年期间在 16 个中心就诊的符合美国风湿病学会/国际协作临床系统性红斑狼疮和工作小组诊断标准的 60 名患者中收集数据,分别为美国脊柱损伤协会损伤量表(AIS)和欧洲多发性硬化症等级量表(EGS)。

结果

纳入的患者中,95%(n=57)为女性,平均年龄为 31.6±9.6 岁。60%(n=36)在诊断为 SLE 后发生了脊髓炎。最常见的表现为对称性截瘫伴感觉减退、弛缓性瘫痪、括约肌功能障碍、AIS=A/B 和 EGS≥8。95%(n=57)使用了静脉注射甲基强的松龙,78.3%(n=47)使用了静脉注射环磷酰胺。56 例中有 75%(42 例)在 6 个月时观察到感觉运动恢复,但只有 16.1%(9 例)完全恢复。在 6 个月时,最低点的低颅压和 EGS≥7 与不良的神经预后相关(p<0.05)。在随访期间观察到复发率为 30.4%(17 例/56 例)。低颅压和低补体血症似乎是复发的保护因素。静脉注射环磷酰胺与复发的时间延迟有关。

结论

系统性红斑狼疮相关的 ATM 可能发生在 SLE 病程的任何时间,尽管治疗后仍会导致显著残疾。复发频率较低,静脉注射环磷酰胺似乎可延迟复发。最低点的低颅压、低补体血症和 EGS 是最重要的预后因素。

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