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塌陷型与未另行指定的局灶节段性肾小球硬化患者的肾脏存活率比较。

Renal Survival in Patients with Collapsing Compared with Not Otherwise Specified FSGS.

作者信息

Laurin Louis-Philippe, Gasim Adil M, Derebail Vimal K, McGregor JulieAnne G, Kidd Jason M, Hogan Susan L, Poulton Caroline J, Detwiler Randal K, Jennette J Charles, Falk Ronald J, Nachman Patrick H

机构信息

University of North Carolina Kidney Center, Division of Nephrology and Hypertension and.

Division of Nephrology, Maisonneuve-Rosemont Hospital, Montreal, Quebec, Canada.

出版信息

Clin J Am Soc Nephrol. 2016 Oct 7;11(10):1752-1759. doi: 10.2215/CJN.13091215. Epub 2016 Jul 21.

Abstract

BACKGROUND AND OBJECTIVES

Idiopathic collapsing FSGS has historically been associated with poor renal outcomes. Minimal clinical data exist on the efficacy of immunosuppressive therapy. Our study sought to provide a comprehensive description of renal survival in patients with collapsing and not otherwise specified FSGS after controlling for factors affecting renal prognosis.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We performed a retrospective analysis of an inception cohort study of patients diagnosed between 1989 and 2012. All potential patients with collapsing FSGS fulfilling the inclusion criteria were identified and compared with patients with not otherwise specified FSGS (approximately 1:2 ratio) on the basis of biopsy report and record availability. Time to ESRD was analyzed using Cox proportional hazards models.

RESULTS

In total, 187 patients were studied (61 collapsing and 126 not otherwise specified), with a mean follow-up of 96 months. At baseline, patients with collapsing FSGS had higher median proteinuria (12.2 [5.6-14.8] versus 4.4 [2.3-8.1] g/d, respectively; <0.001), lower median albuminemia (2.4 [1.9-3.0] versus 2.9 [1.8-3.7] g/dl, respectively; =0.12), and lower median eGFR (48 [26-73] versus 60 [42-92] ml/min per 1.73 m, respectively; =0.01) than patients with not otherwise specified FSGS. The proportion of patients with remission of proteinuria was similar in patients with collapsing FSGS and patients with not otherwise specified FSGS (65.7% [23 of 35] versus 63.2% [72 of 114], respectively; =0.84). The overall renal outcome (ESRD defined as eGFR<15 ml/min per 1.73 m, dialysis, or transplantation) of patients with collapsing FSGS was not poorer than that of patients with not otherwise specified FSGS in multivariate analyses after adjusting for baseline characteristics and immunotherapy (hazard ratio, 1.78; 95% confidence interval, 0.92 to 3.45).

CONCLUSIONS

Compared with not otherwise specified FSGS, idiopathic collapsing FSGS presented with more severe nephrotic syndrome and lower eGFR but had a similar renal survival after controlling for exposure to immunosuppressive treatment. These results highlight the importance of early diagnosis and institution of immunosuppressive therapy in patients with collapsing FSGS.

摘要

背景与目的

特发性塌陷型局灶节段性肾小球硬化(FSGS)历来与不良肾脏预后相关。关于免疫抑制治疗疗效的临床数据极少。我们的研究旨在在控制影响肾脏预后的因素后,全面描述塌陷型及未另行指定的FSGS患者的肾脏生存率。

设计、背景、参与者及测量方法:我们对1989年至2012年间确诊患者的起始队列研究进行了回顾性分析。根据活检报告和记录可得性,识别出所有符合纳入标准的塌陷型FSGS潜在患者,并与未另行指定的FSGS患者(比例约为1:2)进行比较。使用Cox比例风险模型分析至终末期肾病(ESRD)的时间。

结果

总共研究了187例患者(61例塌陷型和126例未另行指定型),平均随访96个月。基线时,塌陷型FSGS患者的蛋白尿中位数较高(分别为12.2[5.6 - 14.8]与4.4[2.3 - 8.1]g/d;<0.001),白蛋白血症中位数较低(分别为2.4[1.9 - 3.0]与2.9[1.8 - 3.7]g/dl;=0.12),估算肾小球滤过率(eGFR)中位数较低(分别为48[26 - 73]与60[42 - 92]ml/min/1.73m²;=0.01),均低于未另行指定的FSGS患者。塌陷型FSGS患者与未另行指定的FSGS患者中蛋白尿缓解患者的比例相似(分别为65.7%[35例中的23例]与63.2%[114例中的72例];=0.84)。在调整基线特征和免疫治疗后,多变量分析中塌陷型FSGS患者的总体肾脏结局(ESRD定义为eGFR<15ml/min/1.73m²、透析或移植)并不比未另行指定的FSGS患者差(风险比,1.78;95%置信区间,0.92至3.45)。

结论

与未另行指定的FSGS相比,特发性塌陷型FSGS表现出更严重的肾病综合征和更低的eGFR,但在控制免疫抑制治疗暴露后肾脏生存率相似。这些结果凸显了塌陷型FSGS患者早期诊断和启动免疫抑制治疗的重要性。

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