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学科主题: 临床医学
题名:
Lupus nephritis combined with renal injury due to thrombotic thrombocytopaenic purpura-haemolytic uraemic syndrome
作者: Yu, Feng; Tan, Ying; Zhao, Ming-Hui1
关键词: ADAMTS-13 ; haemolytic uraemic syndrome ; lupus nephritis ; thrombotic thrombocytopaenic purpura ; von Wilebrand factor
刊名: NEPHROLOGY DIALYSIS TRANSPLANTATION
发表日期: 2010
DOI: 10.1093/ndt/gfp421
卷: 25, 期:1, 页:145-152
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Transplantation ; Urology & Nephrology
研究领域[WOS]: Transplantation ; Urology & Nephrology
关键词[WOS]: VON-WILLEBRAND-FACTOR ; FACTOR-CLEAVING PROTEASE ; ANTIPHOSPHOLIPID SYNDROME NEPHROPATHY ; PLASMA-EXCHANGE ; INTRAVENOUS CYCLOPHOSPHAMIDE ; MYCOPHENOLATE-MOFETIL ; VASCULAR-LESIONS ; DISEASE-ACTIVITY ; ERYTHEMATOSUS ; ADAMTS13
英文摘要:

Methods. Clinical and renal histopathological data of patients with lupus nephritis were reviewed for clinical and pathological evidence of both TTP-HUS and renal thrombotic microangiopathy (TMA). Serum ADAMTS-13 activity and ADAMTS-13 autoantibodies were further studied.

Results. Seven patients with evidence of both TTP-HUS and renal TMA were identified in 353 patients with lupus nephritis. In comparison with 55 patients with lupus nephritis without TTP-HUS, those with TTP-HUS had a higher prevalence of acute renal failure and worse renal outcome. The serum ADAMTS-13 activity was significantly lower in patients with both lupus nephritis and TTP-HUS than in patients with lupus nephritis only and in normal control (40% versus 69%, P = 0.012; 40% versus 81%, P < 0.001, respectively). The prevalence of ADAMTS-13 autoantibodies was significantly higher in patients with both lupus nephritis and TTP-HUS than in patients with lupus nephritis only and in normal control (6/7, 86% versus 10/55, 18%, P < 0.001; 6/7, 86% versus 0, P < 0.001, respectively). After clinical remission, the serum ADAMTS-13 activity of the seven patients with TTP-HUS increased significantly (40% versus 63%, P < 0.001) and five out of the six patients with positive ADAMTS-13 autoantibodies turned negative.

Conclusions. ADAMTS-13 autoantibodies might play an important role in the pathogenesis of TTP-HUS associated with lupus nephritis. The long-term outcome seems to be worse in patients with both TTP-HUS and lupus nephritis than in patients with lupus nephritis alone.

语种: 英语
所属项目编号: 985-2-033-39
项目资助者: Peking University
WOS记录号: WOS:000273113100025
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/62428
Appears in Collections:北京大学第一临床医学院_肾脏内科_期刊论文

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作者单位: 1.Minist Hlth China, Key Lab Renal Dis, Beijing 100034, Peoples R China
2.Peking Univ, Inst Nephrol, Peking Univ Hosp 1, Div Renal,Dept Med, Beijing 100034, Peoples R China

Recommended Citation:
Yu, Feng,Tan, Ying,Zhao, Ming-Hui. Lupus nephritis combined with renal injury due to thrombotic thrombocytopaenic purpura-haemolytic uraemic syndrome[J]. NEPHROLOGY DIALYSIS TRANSPLANTATION,2010,25(1):145-152.
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