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目的探讨双重血浆置换(DFPP)治疗狼疮性肾炎伴血栓性微血管病(LN-TMA)的临床疗效。方法以2010年1月至2013年6月南京军区南京总医院经肾活检确诊为LN-TMA且行DFPP治疗的21例患者为研究对象,所有患者采用免疫抑制方案联合DFPP治疗(DFPP组),以同期仅采用免疫抑制方案治疗的30例LN-TMA作为对照。比较两组患者3个月时摆脱肾脏替代治疗和血肌酐(Scr)下降50%比例、血清自身抗体水平及2年人肾存活率。结果 DFPP组21例中5例治疗2次,16例治疗3次。3个月时DFPP组13例需肾脏替代治疗的患者中11例(84.6%)摆脱了透析,对照组16例中8例(50%)摆脱透析(P=0.051),治疗初无需肾脏替代治疗的患者Scr下降50%的比例DFPP组高于对照组(62.5%对35.7%,P>0.05),DFPP组抗磷脂抗体转阴率明显高于对照组(55.6%对8.3%,P<0.05),但抗ds DNA抗体转阴率及补体水平变化两组间差异无统计学意义。随访期间两组均无死亡病例,分别有3例(14.3%)和8例(26.6%)进入终末期肾病,DFPP组和对照组2年肾存活率分别为90.5%和73.3%,差异无统计学意义(P>0.05)。治疗初需肾脏替代治疗患者的2年肾存活率DFPP组(84.6%)显著高于对照组(50%)(P=0.044)。结论免疫抑制治疗联合DFPP能增加近期摆脱肾脏替代治疗患者的比例,显著改善有严重肾功能损伤患者的远期肾脏存活率。
Objective To investigate the clinical efficacy of double plasmapheresis (DFPP) in the treatment of lupus nephritis with thrombotic microangiopathy (LN-TMA). Methods From January 2010 to June 2013, 21 patients who were diagnosed as LN-TMA by renal biopsy and treated with DFPP in Nanjing General Hospital of Nanjing Military Region from January 2010 to June 2013 were selected as study subjects. All patients received immunosuppression combined with DFPP (DFPP) Thirty LN-TMA treated with immunosuppressive regimen alone were used as controls during the same period. The two groups were compared with 50% reduction of renal replacement therapy and serum creatinine (Scr), serum autoantibodies and 2-year renal survival rate at 3 months. Results In the DFPP group, 5 cases were treated twice in 21 cases and 16 cases were treated 3 times. Eleven of the 13 patients (84.6%) in the DFPP group who required renal replacement therapy were released from dialysis at 3 months and 8 (50%) of 16 patients in the control group were released from dialysis (P = 0.051) (P <0.05). The negative rate of antiphospholipid antibody in DFPP group was significantly higher than that in control group (55.6% vs. 8.3%, P <0.05) , But there was no significant difference between the two groups in anti-dsDNA antibody negative conversion rate and complement level. During the follow-up, there were no deaths in both groups, and 3 cases (14.3%) and 8 cases (26.6%) entered end-stage renal disease respectively. The 2-year renal survival rates in DFPP group and control group were 90.5% and 73.3% Significance (P> 0.05). The 2-year renal survival in patients treated with primary renal replacement therapy was significantly higher in the DFPP group (84.6%) than in the control group (50%) (P = 0.044). Conclusions Immunosuppressive therapy combined with DFPP can increase the proportion of patients who have recently got rid of renal replacement therapy and significantly improve the long-term renal survival rate in patients with severe renal impairment.