Instituto de Investigación Hospital 12 de octubre (i+12), Madrid, Spain; Department of Medicine, Universidad Complutense de Madrid, Madrid, Spain.
Department of Nephrology, Hospital Universitario 12 de Octubre, Madrid, Spain.
Am J Kidney Dis. 2021 May;77(5):684-695.e1. doi: 10.1053/j.ajkd.2020.11.011. Epub 2020 Dec 22.
RATIONALE & OBJECTIVE: A previous study that evaluated associations of kidney biopsy findings with disease progression in patients with C3 glomerulopathy (C3G) proposed a prognostic histologic index (C3G-HI) that has not yet been validated. Our objective was to validate the performance of the C3G-HI in a new patient population.
Multicenter, retrospective cohort study.
SETTING & PARTICIPANTS: 111 patients fulfilling diagnostic criteria of C3G between January 1995 and December 2019, from 33 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases (GLOSEN).
Demographic, clinical parameters, C3G-HI total activity score, and the C3G-HI total chronicity score.
Time to kidney failure.
Intraclass correlation coefficients and κ statistic were used to summarize inter-rater reproducibility for assessment of histopathology in kidney biopsies. The nonlinear relationships of risk of kidney failure with the total activity score and total chronicity score were modeled using Cox proportional hazards analysis that incorporated cubic splines.
The study group included 93 patients with C3 glomerulonephritis and 18 with dense-deposit disease. Participants had an overall meanage of 35±22 (SD) years. Forty-eight patients (43%) developed kidney failure after a mean follow-up of 65±27 months. The overall inter-rater reproducibility was very good for the total activity score (intraclass correlation coefficient [ICC]=0.63) and excellent for total chronicity score (ICC=0.89). Baseline estimated glomerular filtration rate (eGFR), 24-hour proteinuria, and treatment with immunosuppression were the main determinants of kidney failure in a model with only clinical variables. Only tubular atrophy and interstitial fibrosis were identified as predictors in a model with histological variables. When the total activity score and total chronicity score were added to the model, only the latter was identified as an independent predictor of kidney failure.
Only a subset of the kidney biopsies was centrally reviewed. Residual confounding.
We validated the performance of C3G-HI as a predictor of kidney failure in patients with C3G. The total chronicity score was the principal histologic correlate of kidney failure.
先前的研究评估了 C3 肾小球病（C3G）患者肾活检结果与疾病进展的相关性，提出了一种尚未得到验证的预后组织学指数（C3G-HI）。我们的目的是在新的患者人群中验证 C3G-HI 的性能。
多中心回顾性队列研究。
1995 年 1 月至 2019 年 12 月期间，33 个隶属于西班牙肾小球疾病研究组（GLOSEN）的肾病科共有 111 名符合 C3G 诊断标准的患者。
人口统计学、临床参数、C3G-HI 总活动评分和 C3G-HI 总慢性评分。
终点事件为肾功能衰竭。
采用组内相关系数和κ统计量来总结肾脏活检组织病理学评估的观察者间重复性。采用 Cox 比例风险分析来建立肾功能衰竭风险与总活动评分和总慢性评分之间的非线性关系，该分析采用了三次样条。
研究组包括 93 例 C3 肾小球肾炎患者和 18 例致密物沉积病患者。参与者的平均年龄为 35±22（SD）岁。平均随访 65±27 个月后，48 例（43%）患者发生肾功能衰竭。总活动评分的观察者间总体重复性非常好（组内相关系数[ICC]=0.63），总慢性评分的观察者间总体重复性极好（ICC=0.89）。在仅包含临床变量的模型中，基线估计肾小球滤过率（eGFR）、24 小时蛋白尿和免疫抑制治疗是肾功能衰竭的主要决定因素。在包含组织学变量的模型中，仅发现肾小管萎缩和间质纤维化是预测因素。当总活动评分和总慢性评分被添加到模型中时，只有后者被确定为肾功能衰竭的独立预测因素。
仅对部分肾脏活检进行了中心审查。存在残余混杂因素。
我们验证了 C3G-HI 作为 C3G 患者肾功能衰竭预测因子的性能。总慢性评分是肾功能衰竭的主要组织学相关因素。
