Background: The clinical manifestations and prognosis of IgA nephropathy (IgAN) are diverse. Some patients may present with kidney dysfunction lasting shorter than 3 months and meet the acute kidney disease (AKD) criteria. This study aimed to investigate the clinicopathological features, causes and prognosis of newly diagnosed cases of IgAN with AKD. Methods: 1320 IgAN patients diagnosed via kidney biopsy between January 2012 and June 2018 were included in this retrospective study, with a median follow-up period of 35 months. We analyzed the clinicopathological, etiological variables, as well as short-term and long-term prognosis. The main outcome was a composite event of 40% decline in eGFR, kidney failure or death. Results: Incidence of AKD was 8.8% in the newly diagnosed IgAN patients, and was found to be an independent risk factor affecting the short-term (HR, 7.1; 95% CI, 2.3-22.2; P = 0.001) and long-term (HR, 1.8; 95% CI, 1.2-2.6; P = 0.006) prognosis, respectively. The most common cause of AKD was malignant hypertension-related AKD (MHT-AKD; 24.1%), followed by hematuria-related AKD (H-AKD; 12.9%), nephrotoxic-drug-exposurerelated AKD (NTDE-AKD; 12.1%) and crescents-related AKD (C-AKD; 11.2%). The patients in AKD group had more severe clinicopathological characteristics and poor short-term and long-term prognosis than non-AKD group. In subgroup analysis, the MHT-AKD had the worst 5 years survival rate, followed by NTDE-AKD and C-AKD, whereas H-AKD had the best survival rate. Conclusions: AKD is not rare among IgAN patients, and is an independent risk factor for short-term and long-term prognosis. IgAN patients with AKD resulting from different causes have different prognosis.

Objective:To study the clinicopathological characteristics, treatment and prognosis in lupus nephritis (LN) patients with renal thrombotic microangiopathy (TMA), so as to provide more theoretical basis for clinicians to recognize and treat this disease.Methods:The clinical data of LN patients who underwent renal biopsy in the First Affiliated Hospital of Zhengzhou University from January 1, 2012 to May 31, 2019 were retrospectively collected and analyzed. According to renal clinicopathological examination, the patients were divided into renal TMA group and non-renal TMA group. The clinical data, laboratory examination, renal pathological examination, therapeutic measures and prognostic between the two groups were compared. Follow-up end points were defined as composite ends, including all-cause death, entry into end-stage renal disease, and estimated glomerular filtration rate decrease>50% of baseline. Kaplan-Meier survival curve and log-rank test were used to compare the difference of survival rate between the two groups, and multivariate Cox regression equation was used to analyze the risk factors of endpoint events in LN patients.Results:A total of 1 133 patients with LN were enrolled in this study. Patients with renal TMA were more likely to have hypertension ( χ2=16.310, P<0.001), higher baseline serum creatinine ( Z=-6.918, P<0.001) and 24-hour urine protein ( Z=-2.232, P=0.026), and higher renal pathology activity index (AI) score ( Z=1.957, P=0.001)and chronic index (CI) score ( Z=1.836, P=0.002). The proportions of hormone shock ( P<0.001) and plasma exchange ( P<0.001) in the renal TMA group were higher than those in non-renal TMA group. After treatment of (12±2) months, patients in the renal TMA group had a lower complete response rate ( χ2=10.455, P=0.001) and a higher non-response rate ( χ2=6.047, P=0.014) than those in non-renal TMA group, and were associated with worse prognosis (Log-rank test χ2=26.490, P<0.001). Renal TMA was an independent risk factor for poor prognosis ( HR=2.347, 95% CI 1.210-4.553, P=0.012). Conclusions:Compared with LN patients without renal TMA, LN patients with renal TMA are more likely to have hypertension, with higher serum creatinine, 24-hour urinary protein, AI and CI, suggesting poorer treatment response and renal prognosis. Moreover, renal TMA is an independent risk factor for poor prognosis in patients with LN.

目的 探讨胆红素与IgA肾病(IgAN)临床病理特征及预后的关系,探讨胆红素在IgAN中的临床价值.方法 收集2016年5月至2018年5月385例在郑州大学第一附属医院经肾穿刺活检确诊为原发性IgAN患者,根据血清总胆红素(T-Bil)水平的四分位数分组:Q1组(T-Bil<5.5μmol·L-1)、Q2组(T-Bil 5.5～<7.6μmol·L-1)、Q3组(T-Bil 7.6～<10.6μmol·L-1)、Q4组(T-Bil≥10.6μmol·L-1).比较不同T-Bil水平IgAN患者临床、病理及预后的差异.结果 4组患者中,尿素氮在Q1组与Q3组、Q1组与Q4组、Q2组与Q3组、Q2组与Q4组之间差异有统计学意义(P<0.05);肌酐(Cr)、肾小球滤过率(eGFR)、24小时尿蛋白(24 h UP)、激素/免疫抑制剂使用率、肾素-血管紧张素-醛固酮系统(RAAS)抑制剂使用率在Q1组与Q3组、Q1组与Q4组之间差异有统计学意义(P<0.05);总胆固醇(TC)在Q1组与Q4组之间差异有统计学意义(P<0.05);白蛋白(ALB)、终点事件发生率在Q1组与Q2组、Q1组与Q3组、Q1组与Q4组之间差异有统计学意义(P<0.05).Q1组与Q4组在肾小管萎缩或间质纤维化(T)方面差异有统计学意义(P<0.05).尿素氮、Cr、24 h UP与T-Bil水平呈负相关(r<0,P<0.05),eGFR与T-Bil水平呈正相关(r>0,P<0.05),该趋势在T-Bil作为连续及分组变量时均存在.随着T-Bil水平的升高,肾累积生存率逐渐升高(P<0.001).在T-Bil作为连续和分组变量时,高水平胆红素均是IgAN进入终点事件的保护因素,且在校正年龄、性别、平均动脉压、24 h UP、尿素氮、Cr、eGFR、ALB、TC、系膜细胞增生(M)、毛细血管内增生(E)、肾小球节段硬化或粘连(S)、T、细胞或纤维新月体(C)及治疗干预后该保护因素仍存在.结论 高胆红素水平的IgAN患者有更轻的临床病理指标和更好的预后,胆红素是IgAN终点事件的保护因素.

目的 探讨接受重复肾活检的IgA肾病(IgAN)患者临床、病理特点及预后.方法 收集2004年9月至2019年9月在郑州大学第一附属医院接受重复肾活检的IgAN患者共40例,比较两次肾活检时的临床及病理指标.3例患者失访,剩余37例患者采用1:1配对,筛选出同时段、年龄、性别、基线肾小球滤过率、病理表现一致的37例未接受重复肾活检IgAN患者作为对照组比较预后.结果(1)一般资料及临床指标:重复肾活检患者男17例,女23例,两次肾活检中位间隔时间为59.50个月,重复肾活检时血肌酐(Scr)较第1次肾活检升高(P<0.001),肾小球滤过率(GFR)(P<0.001)及血红蛋白(P=0.04)较第1次肾活检下降,结合病史患者多存在24小时尿蛋白定量(24 h TP)持续未转阴、短期3个月内24 h TP或Scr升高.(2)病理指标:肾小球节段硬化及粘连(S)、肾小管萎缩/间质纤维化(T)、间质炎细胞浸润、嗜复红蛋白沉积、刷状缘脱落、蛋白管型及肾小动脉透明样变性病变患者比例重复肾活检时明显增高(P<0.05);S、T、间质炎细胞浸润、嗜复红蛋白沉积、刷状缘脱落、蛋白管型、肾小动脉透明样变性加重的患者重复肾活检时均较第1次肾活检时Scr增加,GFR下降.(3)第2次肾活检后用药方案与第1次肾活检相比19例患者免疫抑制治疗加强,11例患者免疫抑制治疗减弱,10例无明显变化.(4)经Kaplan-Meier法分析,两组IgAN患者的预后无差异.结论 虽然肾活检为有创性操作,但当存在短期内24 h TP及Scr升高或24 h TP持续未缓解时,可考虑接受重复肾活检.临床指标对于病理指标改变无特异性提示作用,更加突出重复肾活检的重要性.本研究暂未能说明接受重复肾活检IgAN患者的预后更佳,但通过重复肾活检,可发现新发的活动性病理改变或原有病变的加重,对于指导治疗有积极作用.

报道1例抗肾小球基底膜（GBM）抗体及抗中性粒细胞胞质抗体（ANCA）双阳性伴膜性肾病的罕见病例。本病例为中年女性患者，其临床表现符合急进性肾小球肾炎，核周型抗中性粒细胞胞质抗体（p-ANCA）、抗GBM抗体均阳性。经肾穿刺活检诊断为ANCA相关性新月体肾炎合并抗GBM抗体阳性和Ⅰ~Ⅱ期膜性肾病（MN），给予血浆置换及免疫抑制治疗后随访观察31个月，治疗取得良好效果。