儿童弥漫性毛细血管内增生性紫癜性肾炎的临床、病理及预后

Clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Schönlein purpura nephritis

摘要:

目的 探讨儿童弥漫性毛细血管内增生性紫癜性肾炎（diffuse endocapillary proliferative Henoch-Sch?nlein purpura nephritis，DEP-HSPN）的临床、病理及预后。方法 回顾性收集江西省儿童医院2006年1月—2021年12月经肾活检确诊的44例DEP-HSPN和765例非DEP-HSPN患儿的临床、病理及预后资料并进行比较。结果 809例紫癜性肾炎患儿中DEP-HSPN 44例（5.4%），平均年龄（8±3）岁，男29例（65.9%），女15例（34.1%）。与非DEP-HSPN组相比，DEP-HSPN组起病至肾活检时间短、呼吸道感染及肉眼血尿多见，多为肾病综合征型，24 h尿蛋白定量、尿蛋白分级、镜下血尿分级、血肌酐及血尿素氮水平高，而血浆白蛋白及补体C3水平低，病理分级重，IgA以系膜区+毛细血管襻沉积为主，改良后半定量评分系统活动性积分高（均P<0.05）。Kaplan-Meier生存曲线显示，两组患儿肾脏完全缓解率差异无统计学意义（P>0.05）。结论 DEP-HSPN患儿起病急，临床表现和病理分级重，改良后半定量评分系统活动性积分高，但预后多数良好，与非DEP-HSPN患儿的肾脏完全缓解率相似。

Abstract:

Objective To investigate the clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Sch?nlein purpura nephritis (DEP-HSPN).Methods A retrospective analysis was performed on the clinical, pathological, and prognosis data of 44 children with DEP-HSPN and 765 children without DEP-HSPN. The children with DEP-HSPN were diagnosed by renal biopsy in Jiangxi Provincial Children's Hospital from January 2006 to December 2021.Results Among the 809 children with purpura nephritis, 44 (5.4%) had DEP-HSPN, with a mean age of (8±3) years, and there were 29 boys (65.9%) and 15 girls (34.1%). Compared with the non-DEP-HSPN group, the DEP-HSPN group had a significantly shorter time from onset to renal biopsy and a significantly higher proportion of children with respiratory infection or gross hematuria, and most children had nephrotic syndrome. The DEP-HSPN group had significantly higher levels of 24-hour urinary protein, urinary protein grading, microscopic hematuria grading, serum creatinine, and blood urea nitrogen and significantly lower levels of serum albumin and complement C3 (P<0.05). The DEP-HSPN group had a higher pathological grading, with predominant deposition of IgA in the mesangial area and capillary loops, and higher activity scores in the modified semi-quantitative scoring system (P<0.05). The Kaplan-Meier survival analysis showed that there was no significant difference in the renal complete remission rate between the two groups (P>0.05).Conclusions Children with DEP-HSPN have a rapid onset, severe clinical manifestations and pathological grading, and high activity scores in the modified semi-quantitative scoring system. However, most of the children with DEP-HSPN have a good prognosis, with a comparable renal complete remission rate to the children without DEP-HSPN.