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.
Key words
Purpura nephritis /
Endocapillary hyperplasia /
Pathology /
Prognosis /
Child
{{custom_sec.title}}
{{custom_sec.title}}
{{custom_sec.content}}
References
1 Xu L, Li Y, Wu X. IgA vasculitis update: epidemiology, pathogenesis, and biomarkers[J]. Front Immunol, 2022, 13: 921864. PMID: 36263029. PMCID: PMC9574357. DOI: 10.3389/fimmu.2022.921864.
2 Kim WK, Kim CJ, Yang EM. Risk factors for renal involvement in Henoch-Sch?nlein purpura[J]. J Pediatr (Rio J), 2021, 97(6): 646-650. PMID: 33722533. PMCID: PMC9432244. DOI: 10.1016/j.jped.2021.01.008.
3 高春林, 章丹, 夏正坤, 等. 2004-2017年单中心9925例儿童肾活检病理分析[J]. 中华肾脏病杂志, 2019, 35(3): 177-183. DOI: 10.3760/cma.j.issn.1001-7097.2019.03.003.
4 赵三龙, 黄松明, 张维真, 等. 儿童毛细血管内皮细胞增生性紫癜性肾炎的临床病理及预后分析[J]. 中华肾脏病杂志, 2010, 26(6): 416-421. DOI: 10.3760/cma.j.issn.1001-7097.2010.06.002.
5 程艺博, 张建江, 窦文杰, 等. 儿童弥散性毛细血管内增生性紫癜性肾炎临床病理分析[J]. 中华实用儿科临床杂志, 2021, 36(22): 1730-1735. DOI: 10.3760/cma.j.cn101070-20201105-01724.
6 秦黎, 刘雁, 李平, 等. 儿童毛细血管内皮增生性紫癜性肾炎50例临床与病理分析[J]. 临床儿科杂志, 2018, 36(1): 30-34. DOI: 10.3969/j.issn.1000-3606.2018.01.007.
7 Fu H, Mao J, Xu Y, et al. Clinical features and outcomes of diffuse endocapillary proliferation Henoch-Sch?nlein purpura nephritis in children[J]. Clinics (Sao Paulo), 2016, 71(9): 550-554. PMID: 27652838. PMCID: PMC5004580. DOI: 10.6061/clinics/2016(09)11.
8 中华医学会儿科学分会肾脏学组. 紫癜性肾炎诊治循证指南（2016）[J]. 中华儿科杂志, 2017, 55(9): 647-651. PMID: 28881507. DOI: 10.3760/cma.j.issn.0578-1310.2017.09.003.
9 Derham RJ. Prognosis of Henoch-Sch?nlein nephritis in children[J]. Br Med J, 1977, 2(6081): 262. PMID: 884464. PMCID: PMC1631330. DOI: 10.1136/bmj.2.6081.262-d.
10 Bohle A, Müller GA, Wehrmann M, et al. Pathogenesis of chronic renal failure in the primary glomerulopathies, renal vasculopathies, and chronic interstitial nephritides[J]. Kidney Int Suppl, 1996, 54: S2-S9. PMID: 8731185.
11 Koskela M, Ylinen E, Ukonmaanaho EM, et al. The ISKDC classification and a new semiquantitative classification for predicting outcomes of Henoch-Sch?nlein purpura nephritis[J]. Pediatr Nephrol, 2017, 32(7): 1201-1209. PMID: 28197887. DOI: 10.1007/s00467-017-3608-5.
12 Yu J, Ni X, Ni J, et al. Comparison of clinicopathological features and prognosis between IgA nephropathy and purpura nephritis in adults with diffuse endocapillary proliferation: a single-center cohort study[J]. Ann Palliat Med, 2020, 9(3): 795-804. PMID: 32389007. DOI: 10.21037/apm.2020.04.04.
13 李春珍, 张东风. 免疫复合物介导的儿童毛细血管内皮细胞增生性肾小球肾炎研究进展[J]. 中华儿科杂志, 2017, 55(3): 231-234. PMID: 28273713. DOI: 10.3760/cma.j.issn.0578-1310.2017.03.018.
14 Fan GZ, Li RX, Jiang Q, et al. Streptococcal infection in childhood Henoch-Sch?nlein purpura: a 5-year retrospective study from a single tertiary medical center in China, 2015-2019[J]. Pediatr Rheumatol Online J, 2021, 19(1): 79. PMID: 34078391. PMCID: PMC8173722. DOI: 10.1186/s12969-021-00569-3.
15 Yang XQ, Huang YJ, Zhai WS, et al. Correlation between endocapillary proliferative and nephrotic-range proteinuria in children with Henoch-Sch?nlein purpura nephritis[J]. Pediatr Nephrol, 2019, 34(4): 663-670. PMID: 30415419. DOI: 10.1007/s00467-018-4134-9.
16 Liu F, Wang C, Wang R, et al. Henoch-Schonlein purpura nephritis with renal interstitial lesions[J]. Open Med (Wars), 2018, 13: 597-604. PMID: 30519637. PMCID: PMC6272053. DOI: 10.1515/med-2018-0088.
17 李华荣, 陈朝英, 涂娟, 等. 儿童紫癜性肾炎预后不良相关因素分析[J]. 中华肾脏病杂志, 2020, 36(7): 535-542. DOI: 10.3760/cma.j.cn441217-20191125-00098.
18 畅晓元, 刘岩. 儿童紫癜性肾炎的相关因素及血清IgA/C3的预测价值分析[J]. 实用药物与临床, 2019, 22(9): 956-959. DOI: 10.14053/j.cnki.ppcr.201909013.
19 Kim CH, Lim BJ, Bae YS, et al. Using the Oxford classification of IgA nephropathy to predict long-term outcomes of Henoch-Sch?nlein purpura nephritis in adults[J]. Mod Pathol, 2014, 27(7): 972-982. PMID: 24390221. DOI: 10.1038/modpathol.2013.222.
20 Jelusic M, Sestan M, Cimaz R, et al. Different histological classifications for Henoch-Sch?nlein purpura nephritis: which one should be used?[J]. Pediatr Rheumatol Online J, 2019, 17(1): 10. PMID: 30819179. PMCID: PMC6393980. DOI: 10.1186/s12969-019-0311-z.
21 Arai M, Mii A, Kashiwagi T, et al. The severity of glomerular endothelial cell injury is associated with infiltrating macrophage heterogeneity in endocapillary proliferative glomerulonephritis[J]. Sci Rep, 2021, 11(1): 13339. PMID: 34172770. PMCID: PMC8233400. DOI: 10.1038/s41598-021-92655-5.
22 Inagaki K, Kaihan AB, Hachiya A, et al. Clinical impact of endocapillary proliferation according to the Oxford classification among adults with Henoch-Sch?nlein purpura nephritis: a multicenter retrospective cohort study[J]. BMC Nephrol, 2018, 19(1): 208. PMID: 30119643. PMCID: PMC6098628. DOI: 10.1186/s12882-018-1009-z.
PDF(544 KB)
