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Abstract：
IgA Nephropathy (IgAN) is the most prevalent glomerular disease worldwide. Complement system activation is crucial in its pathogenesis. Few studies correlated serum C3 and C4 with disease activity and prognosis. This retrospective study investigated the prognostic value of serum complement at the time of diagnosis in patients with IgAN. Specifically we evaluated whether adding serum C3 and C4 levels to established predictive models-one based on variables related to chronic kidney disease (CKD) progression and another incorporating variables from the International IgA Prediction Tool (IntIgAPT)-enhances the accuracy of outcome prediction. A composite renal outcome was defined as 50% decline in eGFR or onset of kidney failure. 101 patients were stratified according to baseline C3 levels in three groups (Low, Medium and High). During a median follow-up of 54 months, the Low group exhibited higher incidence of primary outcome (16.3 events vs 2.9 and 1.7 events × 100 pts/year, p = 0.0026). Model-1 (M1), consisting of CKD progression variables, and Model-3 (M3), comprising IntIgANPT variables, were implemented with baseline C3 and C4 to create Model-2 (M2) and Model-4 (M4), respectively. M2 demonstrated better predictive performance over M1, showing higher discrimination (lower AIC and BIC, higher C-index and NR2). Similarly, M4 outperformed M3, showing enhanced outcome prediction when C3 and C4 levels were added. Implementation of serum C3 and C4 can enhance prediction accuracy of already-validated prognostic models in IgAN. Lower C3 and higher C4 levels were associated with poorer prognosis, highlighting a more \'Complement-Pathic\' subset of patients.
摘要：
IgA肾病(IgAN)是全球最普遍的肾小球疾病。补体系统激活在其发病机制中至关重要。很少有研究将血清C3和C4与疾病活动性和预后相关。这项回顾性研究调查了IgAN患者诊断时血清补体的预后价值。具体来说，我们评估了将血清C3和C4水平添加到已建立的预测模型中是否可以提高结果预测的准确性-一个基于与慢性肾病（CKD）进展相关的变量，另一个基于国际IgA预测工具（IntIgAPT）的变量。复合肾脏结局定义为eGFR下降50%或肾衰竭发作。根据三组的基线C3水平对101例患者进行分层（低，中等和高）。在54个月的中位随访中，低组的主要结局发生率较高(16.3事件vs2.9和1.7事件×100分/年,p=0.0026）。Model-1(M1),由CKD进展变量组成，和Model-3(M3)，包含IntIgANPT变量，用基线C3和C4实现，以创建Model-2(M2)和Model-4(M4)，分别。M2比M1表现出更好的预测性能，表现出更高的辨别力(较低的AIC和BIC，较高的C指数和NR2)。同样，M4优于M3，当添加C3和C4水平时，显示出增强的结果预测。血清C3和C4的实施可以提高IgAN中已经验证的预后模型的预测准确性。较低的C3和较高的C4水平与较差的预后相关。突出显示更多的“补体-病理”患者子集。
