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系统性红斑狼疮中的血栓性微血管病:依库珠单抗的疗效。

Thrombotic microangiopathy in systemic lupus erythematosus: efficacy of eculizumab.

机构信息

Division of Nephrology, University of Kentucky, Lexington, KY; Mansoura University, Egypt.

Division of Nephrology, University of Kentucky, Lexington, KY.

出版信息

Am J Kidney Dis. 2015 Jan;65(1):127-30. doi: 10.1053/j.ajkd.2014.07.031. Epub 2014 Nov 15.

Abstract

Thrombotic microangiopathy (TMA) is a severe disorder with poor outcomes. The cause is unknown for many patients, although TMA is associated with connective tissue disorders, including systemic lupus erythematosus (SLE). While uncommon, TMA is one of the most serious complications of SLE and in many cases may be resistant to therapy. We report a patient with SLE complicated by TMA that was refractory to standard therapy but responded well to eculizumab, with continued remission after 1 year of follow-up. Eculizumab might be useful in the management of resistant cases of TMA caused by SLE.

摘要

血栓性微血管病(TMA)是一种预后不良的严重疾病。尽管 TMA 与结缔组织疾病有关,包括系统性红斑狼疮(SLE),但许多患者的病因仍不清楚。虽然不常见,但 TMA 是 SLE 最严重的并发症之一,在许多情况下可能对治疗有抗性。我们报告了一例 SLE 合并 TMA 的患者,该患者对标准治疗无反应,但对依库珠单抗反应良好,在 1 年的随访后持续缓解。依库珠单抗可能对治疗由 SLE 引起的难治性 TMA 病例有用。

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