塌陷型局灶节段性肾小球硬化症中的肾脏合并症：不仅仅是巧合？

Department of Pathology and Laboratory Medicine, Division of Nephropathology, University of North Carolina,hapel Hill, NC, USA.

Nephrol Dial Transplant. 2022 Jan 25;37(2):311-317. doi: 10.1093/ndt/gfaa327.

BACKGROUND

Collapsing focal segmental glomerulosclerosis (FSGS) has various underlying etiologies and often leads to renal failure. The impact of biopsy-proven renal comorbidities in promoting collapsing glomerulopathy (CG) has not been systematically evaluated in large comparative studies. Those data are reported here.

METHODS

Biopsies with the initial diagnosis of CG in native (n = 321) or transplant kidneys (n = 30) were identified in the University of North Carolina nephropathology database (1 January 2011 to 1 January 2016). Two cohorts were defined: 'sole' CG without and 'accompanied' CG with significant morphologic renal comorbidities. Tip-variant FSGS (T-FSGS) and time-matched biopsies served as control cohorts for comparative analyses.

RESULTS

CG was significantly more common in native (4.4%) and transplant biopsies (4.1%) compared with T-FSGS (0.7 and <0.1%, respectively, difference versus CG P < 0.01). 'Associated' disease was significantly more common in CG (native: 151/321; 47.0%, transplant: 21/30; 70%, P < 0.05) versus T-FSGS (native: 14/51; 27.5%, transplant: exceptional; all differences versus CG P < 0.05). In native biopsies with 'accompanied' CG but not in control groups, stenosing vasculopathies including thrombotic microangiopathies were significantly more prevalent (P < 0.01). In transplants, the high incidence of 'accompanied' CG was linked to de novo diseases, mainly rejection and vascular injury. In native kidneys, membranous glomerulopathies were prevalent in 'accompanied' T-FSGS (36%) and CG (14%) (difference versus time-matched controls P < 0.01 and P < 0.05, respectively); they were uncommon in transplants.

CONCLUSIONS

CG but not T-FSGS shows a high rate of comorbidities, with prominent vasculopathies presumably driving 'ischemic' CG-specific glomerular injury and also the disease course. These findings facilitate future studies into therapy, prognosis and reversibility of 'accompanied' CG.

背景

局灶节段性肾小球硬化症（FSGS）的病因多种多样，常导致肾衰竭。在大型对照研究中，尚未系统评估活检证实的肾合并症在促进塌陷性肾小球病（CG）中的作用。现报告这些数据。

方法

在北卡罗来纳大学肾脏病病理学数据库中（2011 年 1 月 1 日至 2016 年 1 月 1 日），确定了原发性（n=321）或移植肾（n=30）中初始诊断为 CG 的活检。定义了两个队列：“单纯”CG，无显著形态学肾合并症；“伴有”CG，有显著形态学肾合并症。尖端变异 FSGS（T-FSGS）和时间匹配的活检作为对照队列进行比较分析。

结果

与 T-FSGS（分别为 0.7%和<0.1%，与 CG 相比差异均 P<0.01）相比，CG 在原发性（4.4%）和移植肾活检（4.1%）中更为常见。“相关”疾病在 CG 中更为常见（原发性：321 例中有 151 例，47.0%；移植：30 例中有 21 例，70%，与 T-FSGS 相比差异均 P<0.05）。在原发性伴有“相关”CG 的活检中，但在对照组合中没有，狭窄性血管病变，包括血栓性微血管病，更为常见（P<0.01）。在移植中，“伴有”CG 的高发生率与新发疾病有关，主要是排斥反应和血管损伤。在原发性肾脏中，伴有“相关”T-FSGS（36%）和 CG（14%）的膜性肾小球病更为常见（与时间匹配的对照相比差异均 P<0.01 和 P<0.05）；在移植中并不常见。