Association between glomerular C4d deposition, proteinuria, and disease severity in children with IgA nephropathy,Pediatric Nephrology

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Association between glomerular C4d deposition, proteinuria, and disease severity in children with IgA nephropathy
Pediatric Nephrology ( IF 2.6 ) Pub Date : 2022-09-14 , DOI: 10.1007/s00467-022-05725-9
Weiran Zhou ₁ , Hui Wang ₂ , Shuzhen Sun ₃ , Ying Shen ₂ , Xuemei Liu ₁ , Junhui Zhen ₄ , Hongxia Zhang ₁ , Fan Duan ₂ , Yanyan Pan ₁ , Linlin Dong ₁

Affiliation

Background

C4d may be used as a marker to evaluate the condition and prognosis of adults with IgA nephropathy, but there have been few studies of children with IgA nephropathy.

Methods

C4d immunohistochemical staining was performed on samples from children with IgA nephropathy with C1q-negative immunofluorescence. The clinical and pathological treatment and prognostic characteristics of children in the C4d-positive and -negative groups were compared.

Results

A total of sixty-five children with IgA nephropathy were included in the study and were followed up for an average of 37 months. C4d was mainly deposited along the capillary loops. The urinary protein-to-creatinine ratio (UPCR) in the C4d-positive group was significantly higher than that in the C4d-negative group (3.97 vs. 0.81, P < 0.001), and the average integrated optical density value of each child was positively correlated with the UPCR (r = 0.441, P < 0.001). There was a significant difference in the proportions of children with mesangial hypercellularity (M1) (68.97% vs. 44.44%, P = 0.048) and segmental glomerulosclerosis (S1) (65.52% vs. 33.33%, P = 0.010) between the C4d-positive group and the C4d-negative group. The proportion of children who received immunosuppressants in the C4d-positive group was higher than that in the C4d-negative group (86.21% vs. 36.11%, P < 0.001). There was no significant difference in the proportion of children developing kidney failure between the two groups.

Conclusion

C4d was found to be associated with proteinuria, segmental lesions, and immunosuppressant treatment. Activation of the lectin pathway may reflect the severity of clinical and pathological manifestations of IgA nephropathy in children.

Graphical abstract

中文翻译：

IgA 肾病患儿肾小球 C4d 沉积、蛋白尿和疾病严重程度之间的关联

背景

C4d可作为评估成人IgA肾病病情及预后的标志物，但对儿童IgA肾病的研究较少。

方法

对来自 IgA 肾病儿童的样本进行 C4d 免疫组织化学染色，C1q 免疫荧光呈阴性。比较C4d阳性组和阴性组患儿的临床病理治疗及预后特征。

结果

共有 65 名患有 IgA 肾病的儿童被纳入研究，平均随访 37 个月。C4d 主要沿着毛细血管循环沉积。C4d阳性组尿蛋白肌酐比值（UPCR）显着高于C4d阴性组（3.97 vs. 0.81，P < 0.001），各患儿平均综合光密度值为与 UPCR 呈正相关 ( r = 0.441, P < 0.001)。系膜细胞增多症（M1）（68.97% vs. 44.44%，P = 0.048）和节段性肾小球硬化（S1）（65.52% vs. 33.33%，P = 0.010) C4d 阳性组和 C4d 阴性组之间。C4d阳性组接受免疫抑制剂治疗的患儿比例高于C4d阴性组（86.21% vs. 36.11%，P < 0.001）。两组儿童发生肾衰竭的比例无显着差异。