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横贯性脊髓炎作为系统性红斑狼疮或狼疮样疾病的首发表现:良好的功能预后及抗磷脂抗体的相关性

Transverse myelitis as the first manifestation of systemic lupus erythematosus or lupus-like disease: good functional outcome and relevance of antiphospholipid antibodies.

作者信息

D'Cruz David P, Mellor-Pita Susana, Joven Beatriz, Sanna Giovanni, Allanson Judith, Taylor James, Khamashta Munther A, Hughes Graham R V

机构信息

Louise Coote Lupus Unit, St. Thomas' Hospital, London; Rivermead Rehabilitation Centre, Oxford, United Kingdom. david.d'

出版信息

J Rheumatol. 2004 Feb;31(2):280-5.

Abstract

OBJECTIVE

Transverse myelitis (TM) is a rare complication of systemic lupus erythematosus (SLE). Although usually a late manifestation of SLE, it can occur at presentation. We investigated the clinical presentation, treatment and outcome of 15 patients with TM as the presenting manifestation of SLE or lupus-like disease.

METHODS

All patients received corticosteroids, while 13 also received immunosuppressive therapy. Five patients were fully anticoagulated with warfarin.

RESULTS

A sensory level with spastic lower limb weakness and sphincter disturbance was the most common presentation: 14/15 patients had a thoracic or cervical sensory level. Cerebrospinal fluid examination showed high protein concentrations in 3 patients and oligoclonal bands in 8. Eleven of the 15 (73%) had antiphospholipid antibodies (aPL). Of the 15 patients, 3 had complete resolution of the symptoms, 6 had good functional improvements, 5 had good to fair outcome with some functional deficit, and one patient who received corticosteroids alone later died from pneumonia.

CONCLUSION

We describe 15 patients with TM as the presenting manifestation of SLE or lupus-like disease with a high prevalence of aPL. Our data support the view that early diagnosis and immunosuppressive therapy may be superior to corticosteroids alone in improving functional outcome. In those patients with aPL, antiplatelet agents and/or warfarin should also be considered.

摘要

目的

横贯性脊髓炎(TM)是系统性红斑狼疮(SLE)的一种罕见并发症。尽管它通常是SLE的晚期表现,但也可能在疾病初发时出现。我们调查了15例以TM为SLE或狼疮样疾病初发表现的患者的临床表现、治疗及预后情况。

方法

所有患者均接受了糖皮质激素治疗,其中13例还接受了免疫抑制治疗。5例患者使用华法林进行了充分抗凝。

结果

感觉平面伴下肢痉挛性无力及括约肌功能障碍是最常见表现:14/15例患者有胸段或颈段感觉平面。脑脊液检查显示3例患者蛋白浓度升高,8例患者有寡克隆带。15例患者中有11例(73%)存在抗磷脂抗体(aPL)。15例患者中,3例症状完全缓解,6例功能有明显改善,5例功能有一定改善但仍有部分功能缺陷,1例仅接受糖皮质激素治疗的患者后来死于肺炎。

结论

我们描述了15例以TM为SLE或狼疮样疾病初发表现且aPL患病率较高的患者。我们的数据支持这样一种观点,即早期诊断和免疫抑制治疗在改善功能预后方面可能优于单纯使用糖皮质激素。对于那些有aPL的患者,还应考虑使用抗血小板药物和/或华法林。

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