Little population-based data exist about adults with primary nephrotic syndrome.

To evaluate kidney, cardiovascular, and mortality outcomes in adults with primary nephrotic syndrome, we identified adults within an integrated health care delivery system (Kaiser Permanente Northern California) with nephrotic-range proteinuria or diagnosed nephrotic syndrome between 1996 and 2012. Nephrologists reviewed medical records for clinical presentation, laboratory findings, and biopsy results to confirm primary nephrotic syndrome and assigned etiology. We identified a 1:100 time-matched cohort of adults without diabetes, diagnosed nephrotic syndrome, or proteinuria as controls to compare rates of ESKD, cardiovascular outcomes, and death through 2014, using multivariable Cox regression.

We confirmed 907 patients with primary nephrotic syndrome (655 definite and 252 presumed patients with FSGS [40%], membranous nephropathy [40%], and minimal change disease [20%]). Mean age was 49 years; 43% were women. Adults with primary nephrotic syndrome had higher adjusted rates of ESKD (adjusted hazard ratio [aHR], 19.63; 95% confidence interval [95% CI], 12.76 to 30.20), acute coronary syndrome (aHR, 2.58; 95% CI, 1.89 to 3.52), heart failure (aHR, 3.01; 95% CI, 2.16 to 4.19), ischemic stroke (aHR, 1.80; 95% CI, 1.06 to 3.05), venous thromboembolism (aHR, 2.56; 95% CI, 1.35 to 4.85), and death (aHR, 1.34; 95% CI, 1.09 to 1.64) versus controls. Excess ESKD risk was significantly higher for FSGS and membranous nephropathy than for presumed minimal change disease. The three etiologies of primary nephrotic syndrome did not differ significantly in terms of cardiovascular outcomes and death.

Adults with primary nephrotic syndrome experience higher adjusted rates of ESKD, cardiovascular outcomes, and death, with significant variation by underlying etiology in the risk for developing ESKD.

关于成人原发性肾病综合征的基于人群的数据很少。

为了评估患有原发性肾病综合征的成年人的肾脏、心血管和死亡率结果，我们在 1996 年至 2012 年期间确定了综合医疗保健服务系统（北加州 Kaiser Permanente）中患有肾病范围蛋白尿或被诊断为肾病综合征的成年人。肾病学家审查了医疗记录用于临床表现、实验室检查结果和活检结果，以确认原发性肾病综合征和指定的病因。我们确定了一个 1:100 时间匹配的没有糖尿病、诊断为肾病综合征或蛋白尿的成年人队列作为对照，以使用多变量 Cox 回归比较 2014 年的 ESKD 发生率、心血管结局和死亡率。

我们确认了 907 名原发性肾病综合征患者（655 名确诊患者和 252 名推测患者患有 FSGS [40%]、膜性肾病 [40%] 和微小病变 [20%]）。平均年龄为 49 岁；43% 是女性。患有原发性肾病综合征的成人具有更高的 ESKD 调整率（调整后风险比 [aHR]，19.63；95% 置信区间 [95% CI]，12.76 至 30.20）、急性冠状动脉综合征（aHR，2.58；95% CI，1.89 至3.52）、心力衰竭（aHR，3.01；95% CI，2.16 至 4.19）、缺血性卒中（aHR，1.80；95% CI，1.06 至 3.05）、静脉血栓栓塞（aHR，2.56；95% CI，1.35 至 4.85）和死亡（aHR，1.34；95% CI，1.09 至 1.64）与对照组相比。FSGS 和膜性肾病的过度 ESKD 风险显着高于假定的微小病变。

患有原发性肾病综合征的成人经历了更高的 ESKD 调整率、心血管结局和死亡率，并且发生 ESKD 的风险因潜在病因而异。