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系统性红斑狼疮患者急性横贯性脊髓炎的临床特征和预后。

Clinical features and prognoses of acute transverse myelitis in patients with systemic lupus erythematosus.

机构信息

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

出版信息

Korean J Intern Med. 2019 Mar;34(2):442-451. doi: 10.3904/kjim.2016.383. Epub 2018 Jan 5.

Abstract

BACKGROUND/AIMS: Acute transverse myelitis (ATM) is a severe complication of systemic lupus erythematosus (SLE). This study evaluated the clinical factors related to outcome in patients with SLE-associated ATM.

METHODS

The medical records of patients diagnosed with SLE-associated ATM between January 1995 and January 2015 were reviewed. The patients were divided into two groups based on improvement of neurological deficits after treatment: favorable response group and unfavorable response group. During follow-up, the recurrence of ATM was also analyzed.

RESULTS

ATM was identified in 16 patients with SLE. All of the patients were treated with high doses of methylprednisolone (≥ 1 mg/kg daily). Although 12 patients (75%) recovered (favorable response group), four (25%) had persistent neurologic deficits (unfavorable response group) after the treatment. Compared to the favorable response group, significantly higher Systemic Lupus Erythematosus Disease Activity Index-2000, lower complement levels and initial severe neurologic deficits were found in the unfavorable response group. Among the 12 favorable response patients, five (41.7%) experienced recurrence of ATM during the followup. Patients (n = 5) who experienced relapse had a shorter duration of high-dose corticosteroid treatment (13.2 days vs. 32.9 days, p = 0.01) compared to patients who did not relapse. The mean duration of tapering-off the corticosteroid until 10 mg per day was significantly longer in non-relapse group (151.3 ± 60.8 days) than in relapse group (63.6 ± 39.4 days, p = 0.013).

CONCLUSION

Higher disease activity in SLE and initial severe neurologic deficits might be associated with the poor outcome of ATM. Corticosteroid slowly tapering-off therapy might be helpful in preventing the recurrence of ATM.

摘要

背景/目的:急性横贯性脊髓炎(ATM)是系统性红斑狼疮(SLE)的严重并发症。本研究评估了与 SLE 相关的 ATM 患者结局相关的临床因素。

方法

回顾了 1995 年 1 月至 2015 年 1 月期间诊断为 SLE 相关 ATM 的患者的病历。根据治疗后神经功能缺损的改善情况,将患者分为两组:治疗反应良好组和治疗反应不良组。在随访期间,还分析了 ATM 的复发情况。

结果

SLE 患者中发现 16 例 ATM。所有患者均接受大剂量甲基强的松龙(≥ 1mg/kg/d)治疗。尽管 12 例患者(75%)恢复(治疗反应良好组),但 4 例(25%)在治疗后仍存在持续的神经功能缺损(治疗反应不良组)。与治疗反应良好组相比,治疗反应不良组的系统性红斑狼疮疾病活动指数-2000 显著较高,补体水平较低,初始严重神经功能缺损。在 12 例治疗反应良好的患者中,有 5 例(41.7%)在随访期间出现 ATM 复发。与未复发的患者相比,复发患者接受大剂量皮质类固醇治疗的时间(13.2 天 vs. 32.9 天,p=0.01)更短。非复发组逐渐减少皮质类固醇至 10mg/天的平均时间(151.3±60.8 天)明显长于复发组(63.6±39.4 天,p=0.013)。

结论

SLE 疾病活动度较高和初始严重神经功能缺损可能与 ATM 预后不良有关。逐渐减少皮质类固醇的治疗可能有助于预防 ATM 的复发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/79f0/6406093/b44f5ea634d2/kjim-2016-383f1.jpg

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