本文選題：狼瘡性腎炎 + 抗中性粒細(xì)胞胞漿抗體��； 參考：《南京大學(xué)》2017年碩士論文

【摘要】：研究一:血清ANCA陽(yáng)性狼瘡性腎炎患者的臨床病理特征及預(yù)后研究目的:研究血清中性粒細(xì)胞胞漿抗體(ANCA)陽(yáng)性狼瘡性腎炎(LN)患者的臨床、病理特征、預(yù)后及危險(xiǎn)因素。.方法:收集本中心1985年1月至2014年12月間明確診斷為血清ANCA陽(yáng)性LN患者,根據(jù)這部分患者的血清ANCA類(lèi)型分為MPO-ANCA陽(yáng)性和PR3-ANCA陽(yáng)性,比較兩組患者的臨床、病理特征、預(yù)后及危險(xiǎn)因素。結(jié)果:符合入選標(biāo)準(zhǔn)的99例患者中,69例MPO-ANCA陽(yáng)性,26例PR3-ANCA陽(yáng)性,4例MPO-ANCA和PR3-ANCA雙陽(yáng)性。女性88例,男性11例。中位年齡34(25-43)歲。ANCA陽(yáng)性LN患者,尿沉渣紅細(xì)胞計(jì)數(shù)為162萬(wàn)/mL、血清肌酐為92.8μmol/L、SLE-DAI評(píng)分為15分;病理類(lèi)型以IV型為主,占50.5%;光鏡下,細(xì)胞/細(xì)胞纖維性新月體、間質(zhì)炎癥細(xì)胞浸潤(rùn)評(píng)分和纖維性新月體評(píng)分均較高。血清MPO-ANCA陽(yáng)性患者較PR3-ANCA陽(yáng)性患者,SCr更高(109.6 μmol/L vs.74.3μmol/L,P=0.02);病理提示慢性指數(shù)(CI)評(píng)分較高(P=0.007),其中腎小管萎縮病變(P = 0.03)和間質(zhì)纖維化病變(P = 0.001)較重。誘導(dǎo)治療后,MPO-ANCA陽(yáng)性患者的完全緩解率(69.6%vs.84.6%,P = 0.1)和總緩解率(81.2%vs.88.5%,P = 0.02)均低于PR3-ANCA陽(yáng)性患者。MPO-ANCA陽(yáng)性組有 15例患者(15.2%)發(fā)生終末期腎病(ESRD),PR3-ANCA陽(yáng)性組沒(méi)有患者發(fā)生ESRD。MPO-ANCA陽(yáng)性患者,其1年、5年、10年腎臟生存率分別為94.11、83.33、79.7%,低于PR3-ANCA陽(yáng)性患者(1年、5年、10年腎臟生存率均為100.0%),但未達(dá)到統(tǒng)計(jì)學(xué)差異(P = 0.05)。單因素分析顯示:eGFR60mL/min/per1.73m2,患者預(yù)后最好,eGFR30mL/min/per1.73m2,患者預(yù)后最差;接受嗎替麥考酚酯(MMF)治療患者預(yù)后較好;腎臟病理CI≥4分的患者預(yù)后較差;血清MPO-ANCA陽(yáng)性患者的遠(yuǎn)期預(yù)后較差。多因素分析顯示:eGFR的HR為0.96(95%CI 0.94-0.98,P=0.001),接受MMF誘導(dǎo)治療的HR為0.13(95%CI 0.03-0.59,P = 0.008)。結(jié)論:血清ANCA陽(yáng)性LN患者腎臟表現(xiàn)為大量的血尿,病理類(lèi)型以IV型LN為主,有較多新月體形成。MPO-ANCA陽(yáng)性患者腎功能較PR3-ANCA陽(yáng)性患者更差,病理提示慢性病變更重,誘導(dǎo)治療后緩解率更低,預(yù)后更差。eGFR和接受MMF誘導(dǎo)治療是這部分患者發(fā)生ESRD的獨(dú)立性保護(hù)因素。研究二:嗎替麥考酚酯與環(huán)磷酰胺治療血清ANCA陽(yáng)性狼瘡性腎炎的療效比較目的:比較嗎替麥考酚酯(MMF)與環(huán)磷酰胺(CYC)誘導(dǎo)治療血清ANCA陽(yáng)性狼瘡性腎炎(LN)患者的療效和預(yù)后。方法:1985年1月至2014年12月間明確診斷為血清ANCA陽(yáng)性LN患者99例,其中37例接受MMF誘導(dǎo)治療,34例接受CYC誘導(dǎo)治療,其余28例患者接受雷公藤多苷或其他免疫抑制劑治療�；仡櫺员容^MMF組和CYC組患者的療效和預(yù)后。結(jié)果:兩組患者治療前的基礎(chǔ)病情無(wú)差異。MMF組的完全緩解率(94.6%vs.55.9%,P= 0.001)和緩解率(97.3%vs.76.5%,P= 0.002)均高于CYC組,并且MMF組首次獲得緩解時(shí)間短于CYC組(6.4月vs.8.3月,P= 0.01)。隨訪終點(diǎn),MMF組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)、尿沉渣紅細(xì)胞計(jì)數(shù)(1 萬(wàn)/mL vs.8:5萬(wàn)/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)的檢查結(jié)果均優(yōu)于CYC組。兩組患者的不良反應(yīng)發(fā)生率無(wú)統(tǒng)計(jì)學(xué)差異。MMF組中位隨訪74.1月,CYC組中位隨訪33.4月。MMF組患者1年、5年、10年腎臟生存率分別為100%、97.2%、97.2%,明顯高于CYC組(91.0%、77.9%、69.2%)(P = 0.02)。結(jié)論:血清ANCA陽(yáng)性LN患者接受MMF誘導(dǎo)治療,其緩解率和腎臟預(yù)后均優(yōu)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..

【學(xué)位授予單位】：南京大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類(lèi)號(hào)】：R593.242
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本文編號(hào)：1825455