系统性红斑狼疮合并横贯性脊髓炎八例临床分析

Transverse myelitis in systemic lupus erythematosus

目的横贯性脊髓炎(transversemyelitis,TM)是系统性红斑狼疮(systemiclupuserythe-matosus,SLE)少见的严重合并症。本文报道8例并结合文献复习,对其临床表现、治疗方法、预后进行分析。方法回顾了本院1998—2005年的住院和门诊随访病例,报道8例SLE合并TM,并文献复习。结果8例(7例女性,1例男性)SLE发病年龄中位数为19岁(15￣32岁),SLE距TM发病的中位数时间为8个月(0￣12年),就诊时间距TM发病的中位数为3.3个月(0￣7年)。脊髓病变1例发生在颈髓,余7例均定位在胸髓(T1￣T9)。脊髓磁共振成像(MRI)有助于诊断,其中3/7例呈T2加权长条形病变,4/7例呈斑点状病变。其他临床表现、血清学(其中2例存在抗磷脂抗体)、脑脊液均无特征性提示。4/8例遗留明显的神经系统后遗症。其中3例进行了地塞米松鞘内注射治疗,仅1例有短暂疗效。另外4例完全恢复或基本恢复,其中3例在TM发生早期(<10d)接受了大剂量激素治疗,1例有自愈倾向。结论TM是SLE少见的严重合并症,倾向于早年、早期出现,多累及胸髓。早期积极治疗可能改善预后,肌力Ⅲ级以上者预后较好。

Objective Transverse myelitis （TM） is a rare but severe complication of systemic lupus erythematosus （SLE）. We report 8 cases from our center. The clinical features, therapeutic methods, and prognosis of this entity are summarized. Methods One thousand, nine hundred and eigty-four SLE inpatients charts duration 1998-2005 were reviewed retrospectively. Eight eases of TM in SLE were identified, Descriptive analysis and literature review were performed. Results The median onset age of SLE of the eight patients （7 female and 1 male） was 19 years old （15-32 years of age）, TM median occurrence time from onset of SLE was 8 months （0-12 years）. Except one patient had cervical spinal cord involvement, all other 7 TM patients＇ lesions were located at the thoracic region （T1-T9）. MRI was of value to the diagnosis. Two patterns of lesions, longitudinal or speckle were identified. There was no other specific clinical features, including serology （only two patients had antiphospholipid antibodies） and cerebral spinal fluid analysis that could indicate the occurrence of TM. Four patients responded poorly and had severe neurological deficits remained. Three patients had recovered completely, who had received aggressive cortieosteroid pulse therapy at the early stage （〈10 days） of the initiation of TM. One patient had a spontaneous remission tendency. Conclusion TM is a poorly understood complication of SLE, and prone to affect young patients in the early course of the disease. Thoracic spinal cord is the most frequently involved. There is no certain association of TM with antiphospholipid antibodies in our series. Prompt aggressive therapy may improve the outcome. Patients with muscle strength higher than grade Ⅲ may have a better prognosis.