关键词: 重症狼疮性肾炎, 血栓性微血管病, 双重血浆置换, 内皮细胞功能, 临床疗效

Abstract:

Ｏｂｊｅｃｔｉｖｅ：To observation the clinical manifestations of lupus nephritis (LN) patients with thrombotic microangiopathy (TMA) and the efficacy of double filtration plasmapheresis (DFPP) therapy.
Ｍｅｔｈｏｄｏｌｏｇｙ：Thirty active severe LN patients (average age 264±108 years) with TMA lesions were included.All patients were received DFPP and intravenous methylprednisolone pulse therapy,followed by oral glucocorticoids combined with immunosuppressants.The clinical manifestations and their changes of SLEDAI,SCr,indicators of immunology and endothelial function after DFPP therapy were observed.The ratio of patients free from dialysis at 3 months,remission rate and renal survival rate were analyzed.
Ｒｅｓｕｌｔｓ：Amang 30  LN patients with TMA lesions,533% of them  were initial onset  and 633% patients showed rapidly progressive glomerulonephritis with SCr 3704±1830 μmol/L,567% of them need renal replacement therapy.After DFPP treatment (27±08),SLEDAI and SCr  decreased significantly,serum albumin increased and the titer of dsDNA decreased.
The ADAMTS13 activity were found decreased in 6 of 30 patients,and 4 of those patients returned to normal levels after therapy.3 patients with positive ADAMTS13 antibody at baseline changed to megative after treatment.
The levels of Eselectin,thrombomodulin,circulating endothelial cells (CECs) and vascular cell adhesion molecule 1 (VCAM1)  decreased after DFPP treatment,but there was no statistical difference except for CECs and VCAM1.The urinary and immunological indicators were also improved significantly at 3 months.12/17 (706%) patients who need renal replacement therapy at baseline were free from dialysis at 3 months.18 patients (600%) received response at 6 months.There were 10 patients occurred renal endpoint events.Few adverse events were found during DFPP treatment
Ｃｏｎｃｌｕｓｉｏｎ：DFPP combined with immunosuppressants can significantly decrease lupus activity and improve vascular endothelial dysfunction on LN patients with TMA lesions.The efficacy outcomes of DFPP  treatment  suggests DFPP is an alternative therapy for LN patients with TMA lesions.