血清抗中性粒细胞胞浆抗体阳性狼疮性肾炎患者的临床病理特征及预后

发布时间：2018-04-30 17:17

  本文选题：狼疮性肾炎 + 抗中性粒细胞胞浆抗体　； 参考：《南京大学》2017年硕士论文

【摘要】：研究一:血清ANCA阳性狼疮性肾炎患者的临床病理特征及预后研究目的:研究血清中性粒细胞胞浆抗体(ANCA)阳性狼疮性肾炎(LN)患者的临床、病理特征、预后及危险因素。.方法:收集本中心1985年1月至2014年12月间明确诊断为血清ANCA阳性LN患者,根据这部分患者的血清ANCA类型分为MPO-ANCA阳性和PR3-ANCA阳性,比较两组患者的临床、病理特征、预后及危险因素。结果:符合入选标准的99例患者中,69例MPO-ANCA阳性,26例PR3-ANCA阳性,4例MPO-ANCA和PR3-ANCA双阳性。女性88例,男性11例。中位年龄34(25-43)岁。ANCA阳性LN患者,尿沉渣红细胞计数为162万/mL、血清肌酐为92.8μmol/L、SLE-DAI评分为15分;病理类型以IV型为主,占50.5%;光镜下,细胞/细胞纤维性新月体、间质炎症细胞浸润评分和纤维性新月体评分均较高。血清MPO-ANCA阳性患者较PR3-ANCA阳性患者,SCr更高(109.6 μmol/L vs.74.3μmol/L,P=0.02);病理提示慢性指数(CI)评分较高(P=0.007),其中肾小管萎缩病变(P = 0.03)和间质纤维化病变(P = 0.001)较重。诱导治疗后,MPO-ANCA阳性患者的完全缓解率(69.6%vs.84.6%,P = 0.1)和总缓解率(81.2%vs.88.5%,P = 0.02)均低于PR3-ANCA阳性患者。MPO-ANCA阳性组有 15例患者(15.2%)发生终末期肾病(ESRD),PR3-ANCA阳性组没有患者发生ESRD。MPO-ANCA阳性患者,其1年、5年、10年肾脏生存率分别为94.11、83.33、79.7%,低于PR3-ANCA阳性患者(1年、5年、10年肾脏生存率均为100.0%),但未达到统计学差异(P = 0.05)。单因素分析显示:eGFR60mL/min/per1.73m2,患者预后最好,eGFR30mL/min/per1.73m2,患者预后最差;接受吗替麦考酚酯(MMF)治疗患者预后较好;肾脏病理CI≥4分的患者预后较差;血清MPO-ANCA阳性患者的远期预后较差。多因素分析显示:eGFR的HR为0.96(95%CI 0.94-0.98,P=0.001),接受MMF诱导治疗的HR为0.13(95%CI 0.03-0.59,P = 0.008)。结论:血清ANCA阳性LN患者肾脏表现为大量的血尿,病理类型以IV型LN为主,有较多新月体形成。MPO-ANCA阳性患者肾功能较PR3-ANCA阳性患者更差,病理提示慢性病变更重,诱导治疗后缓解率更低,预后更差。eGFR和接受MMF诱导治疗是这部分患者发生ESRD的独立性保护因素。研究二:吗替麦考酚酯与环磷酰胺治疗血清ANCA阳性狼疮性肾炎的疗效比较目的:比较吗替麦考酚酯(MMF)与环磷酰胺(CYC)诱导治疗血清ANCA阳性狼疮性肾炎(LN)患者的疗效和预后。方法:1985年1月至2014年12月间明确诊断为血清ANCA阳性LN患者99例,其中37例接受MMF诱导治疗,34例接受CYC诱导治疗,其余28例患者接受雷公藤多苷或其他免疫抑制剂治疗。回顾性比较MMF组和CYC组患者的疗效和预后。结果:两组患者治疗前的基础病情无差异。MMF组的完全缓解率(94.6%vs.55.9%,P= 0.001)和缓解率(97.3%vs.76.5%,P= 0.002)均高于CYC组,并且MMF组首次获得缓解时间短于CYC组(6.4月vs.8.3月,P= 0.01)。随访终点,MMF组SLE-DAI(2分 vs.4 分,P=0.001)、24h尿蛋白定量(0.3 g/24h vs.0.7 g/24h,P0.001)、尿沉渣红细胞计数(1 万/mL vs.8:5万/mL,P = 0.009)、Alb(43.5 g/L vs.38.4 g/L,P=0.001)、SCr(69.8 μmol/L vs.91.9 μmol/L,P = 0.006)的检查结果均优于CYC组。两组患者的不良反应发生率无统计学差异。MMF组中位随访74.1月,CYC组中位随访33.4月。MMF组患者1年、5年、10年肾脏生存率分别为100%、97.2%、97.2%,明显高于CYC组(91.0%、77.9%、69.2%)(P = 0.02)。结论:血清ANCA阳性LN患者接受MMF诱导治疗,其缓解率和肾脏预后均优于CYC。
[Abstract]:Study 1: the clinicopathological features and prognosis of serum ANCA positive lupus nephritis Objective: To study the clinical, pathological features, prognosis and risk factors of serum neutrophils cytoplasmic antibody (ANCA) positive lupus nephritis (LN) patients. Methods: the diagnosis of serum ANCA positive LN from January 1985 to December 2014 was collected. According to the type of serum ANCA in this part, the patients were divided into MPO-ANCA positive and PR3-ANCA positive. The clinical, pathological features, prognosis and risk factors of the two groups were compared. Results: of the 99 patients with the admission criteria, 69 cases were MPO-ANCA positive, 26 cases of PR3-ANCA positive, 4 cases of MPO-ANCA and PR3-ANCA double positive. 88 cases were female, 11 men were male, and the median age 34 was 34. (25-43) the red blood cell count of urine sediment was 1 million 620 thousand /mL, serum creatinine was 92.8 mol/L, and the SLE-DAI score was 15, and the pathological type was IV type, accounting for 50.5%. Under light microscope, the cell / cell fibrotic crescent body, interstitial inflammatory cell infiltration score and fibrous crescent score were higher. Serum MPO-ANCA positive patients were compared with PR3-ANCA. In positive patients, SCr was higher (109.6 mu mol/L vs.74.3 mol/L, P=0.02); pathology indicated that the chronic index (CI) score was higher (P=0.007), in which renal tubular atrophy (P = 0.03) and interstitial fibrosis (P = 0.001) were heavier. The complete remission rate (69.6%vs.84.6%, P = 0.1) and total remission rate after induction of treatment were observed. 0.02) 15 patients (15.2%) had end-stage renal disease (ESRD) in the positive group of.MPO-ANCA positive patients (15.2%), and no PR3-ANCA positive group had ESRD.MPO-ANCA positive patients. The renal survival rate was 94.11,83.33,79.7% in 1 years, 5 years and 10 years respectively, which was lower than that of PR3-ANCA positive patients (1 years, 5 years, 10 year kidney survival rates were 100%). There was no statistical difference (P = 0.05). Single factor analysis showed that eGFR60mL/min/per1.73m2, the patient had the best prognosis, eGFR30mL/min/per1.73m2, the worst prognosis; the prognosis of patients receiving malcophenol ester (MMF) was better; the prognosis of patients with renal pathological CI > 4 scores was poor; the long-term prognosis of serum MPO-ANCA positive patients was poor. The analysis showed that the HR of eGFR was 0.96 (95%CI 0.94-0.98, P=0.001), and the HR of MMF induced therapy was 0.13 (95%CI 0.03-0.59, P = 0.008). Conclusion: the kidney of the patient with ANCA positive sera showed a large number of hematuria, and the pathological type was mainly in the pathological type, and the renal function of the patients with more crescent formation was worse than that of the positive patients. Pathology mentioned that the renal function was worse. More serious chronic diseases, lower remission rate after induction of treatment, worse prognosis.EGFR and MMF induction therapy are the independent protective factors of ESRD in this part of the patients. Study two: compare the efficacy of mycophenolate mofetil and cyclophosphamide in the treatment of ANCA positive lupus nephritis: comparison of mycophenolate (MMF) and cyclophosphamide (CYC) The efficacy and prognosis of serum ANCA positive lupus nephritis (LN) patients were induced. Methods: from January 1985 to December 2014, 99 patients with serum ANCA positive LN were diagnosed, of which 37 were treated with MMF induction, 34 received CYC induction, and 28 patients received Tripterygium wilfordii or other immunosuppressive agents. A retrospective comparison of MM Outcome and prognosis of patients in group F and group CYC. Results: the total remission rate (94.6%vs.55.9%, P= 0.001) and remission rate (97.3%vs.76.5%, P= 0.002) in the two groups before treatment were higher than those in the CYC group, and the first remission time in the MMF group was shorter than that in the CYC group (6.4 months vs.8.3 month, P= 0.01). .4, P=0.001), 24h urine protein quantitative (0.3 g/24h vs.0.7 g/24h, P0.001), urine sediment red cell count (10 thousand /mL vs.8:5 million /mL, P = 0.009), Alb (69.8 mu, 0.006) examination results were superior to those of the group. There was no statistical difference in the incidence of adverse reactions between the two groups of patients. After 74.1 months of follow-up, the median follow-up of group CYC was followed up for 33.4 months in group.MMF for 1 years, 5 years, and 10 years' renal survival rate was 100%, 97.2%, 97.2%, obviously higher than group CYC (91%, 77.9%, 69.2%) (P = 0.02). Conclusion: serum ANCA positive LN patients received MMF induction therapy, and their remission rate and renal prognosis were better than CYC..

【学位授予单位】：南京大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R593.242
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本文编号：1825455