Department of Paediatrics, Jinling Hospital, School of Medicine, Nanjing University, Nanjing, People's Republic of China.
Nephrology (Carlton). 2023 Sep;28(9):485-494. doi: 10.1111/nep.14194. Epub 2023 Jul 23.
Glomerular microthrombosis (GMT) was a common vascular lesion in patients with lupus nephritis (LN). The objective of this study was to investigate the relationship between serum anti-beta2-glycoprotein I antibodies (a-β2GP1) and anti-complement 1q antibodies (a-C1q) antibodies and to investigate the possible mechanism of GMT in children with LN.
The subjects were 191 children with LN diagnosed by renal biopsy in our hospital from January 2017 to January 2020. The patients were divided into GMT group and non-GMT group. The clinical manifestations, laboratory tests, renal pathology, prognosis of the two groups and the relationship between a-β2GP1 and a-C1q antibodies were observed.
In 191 children with LN, 52 cases (27.23%) presented with GMT. The value of C3, haemoglobin (Hb), estimate glomerular filtration rate (eGFR) and anticardiolipin antibody (ACA) in GMT group were lower than that of non-GMT group (p < .05, p < .01). The value of serum creatinine (Scr), 24 h proteinuria (PRO), urine red blood cells (RBC), N-acetyl-β-d-glucosidase (NAG) and retinol-binding protein (RBP), a-C1q, a-β2GP1, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and renal histopathological activity index (AI) score in GMT group were higher than that of non-GMT group (p < .05, p < .01). The positive proportions of serum a-C1q and a-β2GP1 in GMT group were higher than those in non-GMT group (p < .05). According to Spearman correlation analysis, a-C1q was positively correlated with AI score, SLEDAI, a-β2GP1, GMT, LN-III and LN-IV. Hb, eGFR and a-C1q Ab were associated with the formation of GMT in children with LN. The complete proteinuria remission and renal survival in GMT group were significantly lower than those in non-GMT group (p < .05, p < .01).
LN children with GMT had more severe clinical manifestations and renal pathologic damages, and poor outcome. Serum a-C1q level was positively correlated with a-β2GP1, and a-β2GP1 may be involved in the formation of GMT in children with LN, which might involve in the activation of complement classical pathway.
肾小球微血栓（GMT）是狼疮性肾炎（LN）患者常见的血管病变。本研究旨在探讨血清抗β2-糖蛋白 I 抗体（a-β2GP1）和抗补体 1q 抗体（a-C1q）与儿童 LN 中 GMT 形成的关系及其可能的机制。
选取 2017 年 1 月至 2020 年 1 月在我院经肾活检诊断为 LN 的 191 例患儿为研究对象。根据肾活检是否出现 GMT 将患儿分为 GMT 组和非 GMT 组。观察两组患儿的临床表现、实验室检查、肾脏病理、预后及 a-β2GP1 与 a-C1q 抗体的关系。
191 例 LN 患儿中，52 例（27.23%）出现 GMT。GMT 组患儿的 C3、血红蛋白（Hb）、估算肾小球滤过率（eGFR）和抗心磷脂抗体（ACA）水平低于非 GMT 组（p < .05，p < .01），血清肌酐（Scr）、24 小时尿蛋白（PRO）、尿红细胞（RBC）、N-乙酰-β-D-氨基葡萄糖苷酶（NAG）和视黄醇结合蛋白（RBP）、a-C1q、a-β2GP1、系统性红斑狼疮疾病活动指数（SLEDAI）和肾脏组织学活动指数（AI）评分均高于非 GMT 组（p < .05，p < .01）。GMT 组患儿血清 a-C1q 和 a-β2GP1 的阳性率高于非 GMT 组（p < .05）。Spearman 相关性分析显示，a-C1q 与 AI 评分、SLEDAI、a-β2GP1、GMT、LN-III 和 LN-IV 呈正相关。Hb、eGFR 和 a-C1q Ab 与儿童 LN 中 GMT 的形成有关。GMT 组患儿完全蛋白尿缓解和肾脏存活率明显低于非 GMT 组（p < .05，p < .01）。
伴有 GMT 的 LN 患儿临床表现和肾脏病理损伤更严重，预后更差。血清 a-C1q 水平与 a-β2GP1 呈正相关，a-β2GP1 可能参与儿童 LN 中 GMT 的形成，这可能涉及补体经典途径的激活。
