Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Intern Med. 2019 Mar;34(2):442-451. doi: 10.3904/kjim.2016.383. Epub 2018 Jan 5.
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.
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.
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).
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.
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。
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。
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)。
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。