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抗肾小球基底膜病合并系膜 IgA 沉积患者的临床特征和预后。

Clinical features and prognosis of patients with anti-GBM disease combined with mesangial IgA deposition.

机构信息

Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.

Zhengzhou University, Zhengzhou, Henan, China.

出版信息

Front Immunol. 2024 Jul 22;15:1373581. doi: 10.3389/fimmu.2024.1373581. eCollection 2024.

Abstract

INTRODUCTION

Anti-GBM diseases with IgA deposition in the mesangial region are rarely described.The factors influencing renal prognosis in patients with anti-GBM disease combined with mesangial IgA deposition are unknown.

METHODS

We searched the pathological reports of the First Affiliated Hospital of Zhengzhou University from 2015 to 2023 and found that a total of 72 patients with the anti-GBM disease and 25 patients combined with mesangial IgA deposition. We studied the clinical and pathological features, renal prognosis, and the factors affecting renal prognosis in patients with anti-GBM disease combined with mesangial IgA deposition.

RESULTS

Their median age was 44 years, and their age distribution was unimodal. The proportion of oliguria or anuria in patients with anti-GBM disease combined with mesangial IgA deposition was significantly lower than that in patients with classic anti-GBM disease (13.04 vs. 42.31%, p=0.030). Their 24-hour urinary protein excretion was significantly higher [median:3.25 vs. 1.12g/24h, Interquartile range(IQR):1.0323.945 vs. 0.631.79g/24h, p=0.020], serum creatinine (SCr) level at the initial diagnosis was lower(median:456.0 vs. 825.5μmol/L, IQR:270.0702.0 vs. 515.81231.2μmol/L, p=0.002), peak SCr level was lower (median: 601.0 vs. 907.2μmol/L, IQR: 376.5937.0 vs. 607.01361.2μmol/L, p=0.007), and their serum complement 3(C3) level was higher(median: 1.275 vs. 1.015g/L, IQR:1.0971.462 vs. 0.8501.220g/L, p=0.027). They had better renal outcomes during follow-up (p<0.001). After adjustment for hypertension, oliguria or anuria, and crescents%, IgA deposition in the mesangial region was still an independent protective factor (p=0.003) for ESRD in anti-GBM patients. Hypertension (p=0.026) and SCr levels at initial diagnosis (p=0.004) were risk factors for renal prognosis in patients with anti-GBM disease combined with mesangial IgA deposition.

DISCUSSION

Patients with anti-GBM disease combined with mesangial IgA deposition have less severe renal impairment and better renal prognosis than patients with classic anti-GBM disease.

摘要

简介

在肾小球基底膜(GBM)区域有 IgA 沉积的抗 GBM 疾病很少见。影响抗 GBM 病合并系膜 IgA 沉积患者肾脏预后的因素尚不清楚。

方法

我们检索了郑州大学第一附属医院 2015 年至 2023 年的病理报告,发现共有 72 例抗 GBM 病患者和 25 例合并系膜 IgA 沉积的患者。我们研究了抗 GBM 病合并系膜 IgA 沉积患者的临床和病理特征、肾脏预后以及影响其肾脏预后的因素。

结果

他们的中位年龄为 44 岁,年龄分布呈单峰分布。抗 GBM 病合并系膜 IgA 沉积患者少尿或无尿的比例明显低于经典抗 GBM 病患者(13.04% vs. 42.31%,p=0.030)。他们的 24 小时尿蛋白排泄量明显较高[中位数:3.25 vs. 1.12g/24h,四分位距(IQR):1.0323.945 vs. 0.631.79g/24h,p=0.020],初始诊断时的血清肌酐(SCr)水平较低(中位数:456.0 vs. 825.5μmol/L,IQR:270.0702.0 vs. 515.81231.2μmol/L,p=0.002),峰值 SCr 水平较低(中位数:601.0 vs. 907.2μmol/L,IQR:376.5937.0 vs. 607.01361.2μmol/L,p=0.007),血清补体 3(C3)水平较高(中位数:1.275 vs. 1.015g/L,IQR:1.0971.462 vs. 0.8501.220g/L,p=0.027)。他们在随访期间肾脏预后较好(p<0.001)。在调整高血压、少尿或无尿和新月体比例后,系膜区 IgA 沉积仍然是抗 GBM 病患者发生终末期肾病的独立保护因素(p=0.003)。高血压(p=0.026)和初始诊断时的 SCr 水平(p=0.004)是抗 GBM 病合并系膜 IgA 沉积患者肾脏预后的危险因素。

讨论

抗 GBM 病合并系膜 IgA 沉积患者的肾功能损害程度较轻,肾脏预后较好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0bc/11298365/5581492c13ec/fimmu-15-1373581-g001.jpg

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