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肾脏病与透析肾移植杂志 ›› 2022, Vol. 31 ›› Issue (5): 420-426.DOI: 10.3969/j.issn.1006-298X.2022.05.004

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合并系膜溶解肾脏疾病患者的临床病理特征及预后

  

  • 出版日期:2022-10-28 发布日期:2022-10-22

Clinicopathological characteristics and prognosis of glomerular diseases presenting with mesangiolysis

  • Online:2022-10-28 Published:2022-10-22

摘要: 目的:分析系膜溶解病变的疾病谱分布特点,比较以系膜溶解为主要表现的肾小球疾病的临床病理特征及预后。
方法:回顾分析国家肾脏疾病临床医学研究中心2005年1月到2020年12月除糖尿病肾病和狼疮性肾炎外自体肾活检合并系膜溶解的患者资料,分析疾病谱并比较临床病理特征及预后。
结果:本研究纳入385例患者,系膜溶解病变发生率约为076%(385/50 488),其中局灶性、弥漫性系膜溶解分别占比8727%、1273%;急性期、慢性期和急慢性合并期分别占比883%、4727%和4390%。原发性和继发性肾小球疾病中系膜溶解发生率分别为059%和185%。继发性肾小球疾病中POEMS综合征(POEMS组,100%)、抗血管内皮生长因子(aVEGF)药物相关肾损害(aVEGF组,875%)和造血干细胞移植(HSCT)相关肾损害(HSCT组,8095%)系膜溶解发生率较高。aVEGF组尿蛋白(214 g/d)高于POEMS组和HSCT组(091 g/d、082 g/d)。aVEGF组肾小球毛细血管袢气球样变(100%)和基膜内皮下渗出样病变(857%)发生率高;POEMS组弥漫均匀性系膜溶解;HSCT组毛细血管袢融合扩张多见,平均肾小球内皮下宽度最大。三组肾脏预后总体良好。
结论:系膜溶解的疾病谱较为广泛,但总体发生率较低。以弥漫系膜溶解为表现的疾病临床、病理形态各有特点,其病理生理意义及对预后的影响,仍有待深入研究。


关键词: 系膜溶解,抗血管内皮生长因子药物相关肾损害,POEMS综合症,造血干细胞移植相关肾损害

Abstract: Objective:To clarify the disease spectrum of mesangiolyisis and compare the clinicopathological features and prognosis of glomerular diseases manifested by mesangiolyisis.
Methodology:Patients with native biopsyproven mesangiolyisis from Jan. 2005 to Dec. 2020 excluding diabetic nephropathy or lupus nephritis in the National Clinical Research Center of Kidney Disease. were analyzed retrospectively, and their clinicopathological data and followup information were evaluated.
Results:Mesangiolysis was observed in 385 (076%) cases from 50488 native renal specimen. Focal and diffuse mesangiolysis were accounted for 8727% and 1273% respectively. Mesangiolysis displayed as acute phase (883%), chronic phase (4727%), and acutechronic phase (4390%). The occurence of mesangiolyisis in primary and secondary glomerular disease was 059% and 185%, respectively. Secondary glomerular diseases included POEMS syndrome (POEMS, 100%), antivascular endothelial growth factor drugrelated renal damage (aVEGF, 875%) and hematopoietic stem cell transplantation related renal damage (HSCT, 8095%). Compare the clinicopathological and prognostic differences between the aVEGF, POEMS and HSCT groups. Patients in aVEGF group presented a higher level of protein urine (214 g/24 h) than the other two groups (091 g/24 h, 082 g/24 h). The occurence of glomerular capillary ballooning and exudative material (857%) was higher in aVEGF group. Mesangiolyisis was diffusely and evenly distributed in the glomeruli in POEMS group. Dilatation of capillary loops could be often observed in the HSCT group, and the mean average width of subendothelial space was also wider than the other two groups. Patients in the three groups had an overall good survival of kidney.
Conclusion:Mesangiolyisis was observed in a wide range of glomerular diseases, but appears to be a rather infrequent occurrence. Diseases presenting as diffuse mesangiolysis are distinctive in pathological morphology and clinical manifestations, and the role of mesangiolysis in the process of such diseases as to pathophysiological significance and its impact on prognosis remains to be explored in depth.


Key words: mesangiolyisis, anti-vascular endothelial growth factor drug-related renal damage, POEMS syndrome, hematopoietic stem cell transplantation-related renal damage