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| C4d在IgA肾病中的生物标志物价值：激活机制、风险分层与预后评估 |
| Biomarker significance of C4d in IgA nephropathy: Activation mechanism, risk stratification, and prognostic evaluation. |
| 投稿时间：2026-04-16 修订日期：2026-07-18 |
| DOI： |
| 中文关键词: IgA肾病 C4d 补体系统 凝集素途径 替代途径 肾组织沉积 尿液C4d 无创生物标志物 风险分层 临床预后 |
| 英文关键词: IgA nephropathy C4d complement lectin pathway alternative pathway urinary biomarkers risk stratification prognosis. |
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| 中文摘要: |
| IgA肾病（IgA nephropathy，IgAN）是全球最常见的原发性肾小球肾炎，临床表现与病理进展高度异质，约20%–50%的患者可进展至肾衰竭。其经典发病机制符合“多重打击”学说：循环中半乳糖缺陷IgA1（Gd-IgA1）与抗聚糖抗体形成免疫复合物并沉积于肾小球系膜区，继而激活系膜细胞、招募炎症细胞并启动补体系统，推动肾脏损伤与疾病进展[1,2]。补体激活被认为是驱动IgAN进展的关键因素，并构成重要治疗靶点[1-3]。C4d是凝集素/经典途径激活后的稳定降解产物，其在组织中的沉积可作为补体激活的“分子足迹”。近年来，肾组织C4d沉积及尿液可溶性C4d水平均被证实与疾病活动度、病理严重程度及长期预后密切相关。本文围绕C4d在IgAN中的激活机制、组织病理学分层价值、尿液无创监测潜力及临床转化前景进行系统综述。 |
| 英文摘要: |
| IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide, characterized by highly heterogeneous clinical manifestations and pathological progression, with approximately 20%–50% of patients progressing to kidney failure. Its classic pathogenesis conforms to the "multi-hit" hypothesis: circulating galactose-deficient IgA1 (Gd-IgA1) forms immune complexes with anti-glycan antibodies, which deposit in the glomerular mesangium, subsequently activating mesangial cells, recruiting inflammatory cells, and initiating the complement system, thereby driving kidney injury and disease progression[1,2]. Complement activation is recognized as a key factor driving IgAN progression and constitutes an important therapeutic target[1-3]. C4d is a stable degradation product generated following activation of the lectin or classical pathways, and its tissue deposition serves as a "molecular footprint" of complement activation. In recent years, both renal C4d deposition and urinary soluble C4d levels have been confirmed to be closely associated with disease activity, pathological severity, and long-term prognosis. This review systematically summarizes the activation mechanisms of C4d in IgAN, its value in histopathological risk stratification, its potential as a non-invasive urinary biomarker, and its prospects for clinical translation. |
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