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IgA 肾病患者的组织学血栓性微血管病证据及其对肾脏结局的影响。

Evidences of histologic thrombotic microangiopathy and the impact in renal outcomes of patients with IgA nephropathy.

机构信息

Nephrology Division, University of São Paulo, School of Medicine, São Paulo, SP, Brazil.

Pathology Division, University of São Paulo, School of Medicine, São Paulo, SP, Brazil.

出版信息

PLoS One. 2020 Nov 4;15(11):e0233199. doi: 10.1371/journal.pone.0233199. eCollection 2020.

Abstract

INTRODUCTION

IgA nephropathy (IgAN) is the most common primary glomerulopathy worldwide. According to the Oxford Classification, changes in the kidney vascular compartment are not related with worse outcomes. This paper aims to assess the impact of thrombotic microangiopathy (TMA) in the outcomes of Brazilian patients with IgAN.

MATERIALS AND METHODS

Analysis of clinical data and kidney biopsy findings from patients with IgAN to assess the impact of TMA on renal outcomes.

RESULTS

The majority of the 118 patients included were females (54.3%); mean age of 33 years (25;43); hypertension and hematuria were observed in 67.8% and 89.8%, respectively. Median creatinine: 1.45mg/dL; eGFR: 48.8ml/min/1.73m2; 24-hour proteinuria: 2.01g; low serum C3: 12.5%. Regarding to Oxford Classification: M1: 76.3%; E1: 35.6%; S1: 70.3%; T1/T2: 38.3%; C1/C2: 28.8%. Average follow-up: 65 months. Histologic evidence of TMA were detected in 21 (17.8%) patients and those ones presented more frequently hypertension (100% vs. 61%, p <0.0001), hematuria (100% vs 87.6%, p = 0.0001), worse creatinine levels (3.8 vs. 1.38 mg/dL, p = 0.0001), eGFR (18 vs. 60 ml/min/1.73m2), p = 0.0001), low serum C3 (28.5% vs. 10.4%, p = 0.003), lower hemoglobin levels (10.6 vs. 12.7g/dL, p<0.001) and platelet counts (207,000 vs. 267,000, p = 0.001). Biopsy findings of individuals with TMA revealed only greater proportions of E1 (68% vs. 32%, p = 0.002). Individuals with TMA were followed for less time (7 vs. 65 months, p<0.0001) since they progressed more frequently to chronic kidney disease (CKD) requiring kidney replacement therapy (KRT) (71.4% vs. 21,6%, p<0.0001). Male sex, T1/T2, and TMA were independently associated with progression to CKD-KRT.

CONCLUSIONS

In this study patients with TMA had worse clinical manifestations and outcomes. In terms of histologic evidence, E1 distinguished patients with TMA from other patients. Further studies are necessary to analyze the impact of vascular lesions on IgAN prognosis.

摘要

简介

IgA 肾病(IgAN)是全球最常见的原发性肾小球疾病。根据牛津分类,肾脏血管壁的改变与预后无关。本文旨在评估巴西 IgAN 患者血栓性微血管病(TMA)对结局的影响。

材料和方法

分析 IgAN 患者的临床数据和肾活检结果,评估 TMA 对肾脏结局的影响。

结果

118 例患者中大多数为女性(54.3%);平均年龄 33 岁(25 岁至 43 岁);分别有 67.8%和 89.8%的患者患有高血压和血尿。中位数血肌酐:1.45mg/dL;eGFR:48.8ml/min/1.73m2;24 小时蛋白尿:2.01g;血清 C3 低:12.5%。根据牛津分类:M1:76.3%;E1:35.6%;S1:70.3%;T1/T2:38.3%;C1/C2:28.8%。平均随访:65 个月。21 例(17.8%)患者的肾活检有 TMA 的组织学证据,这些患者更常出现高血压(100%比 61%,p<0.0001)、血尿(100%比 87.6%,p=0.0001)、血肌酐水平升高(3.8 比 1.38mg/dL,p=0.0001)、eGFR 降低(18 比 60ml/min/1.73m2,p=0.0001)、血清 C3 低(28.5%比 10.4%,p=0.003)、血红蛋白水平降低(10.6 比 12.7g/dL,p<0.001)和血小板计数降低(207,000 比 267,000,p=0.001)。TMA 患者的活检结果仅显示 E1 比例更高(68%比 32%,p=0.002)。TMA 患者的随访时间更短(7 比 65 个月,p<0.0001),因为他们更常进展为需要肾脏替代治疗(KRT)的慢性肾脏病(CKD)(71.4%比 21.6%,p<0.0001)。男性、T1/T2 和 TMA 与进展为 CKD-KRT 独立相关。

结论

在这项研究中,TMA 患者的临床表现和结局更差。就组织学证据而言,E1 将 TMA 患者与其他患者区分开来。需要进一步研究来分析血管病变对 IgAN 预后的影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5227/7641451/f7150695f7e7/pone.0233199.g001.jpg

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