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.
YIN Ting-Ting,PENG Xiao-Jie,FU Rui et al. Clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Sch?nlein purpura nephritis[J]. CJCP, 2023, 25(8): 837-842.
YIN Ting-Ting,PENG Xiao-Jie,FU Rui et al. Clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Sch?nlein purpura nephritis[J]. CJCP, 2023, 25(8): 837-842.
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.
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.
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.
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.
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.
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.
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.
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.
