Correlation between endocapillary proliferative and nephrotic-range proteinuria in children with Henoch-Schönlein purpura nephritis
The endocapillary proliferative (EP) lesion is not included in the International Study of Kidney Disease in Children (ISKDC) pathological classification of Henoch-Schönlein purpura nephritis (HSPN). The main objective of the study was to determine the pathological importance of EP in the development of proteinuria in children with Henoch-Schönlein purpura nephritis (HSPN).
The pathological features of 148 HSPN children with nephrotic-range proteinuria were investigated retrospectively. Urinary IgG, transferrin, and albumin levels were measured by immunonephelometry. The correlations between EP lesion and 24-h proteinuria, urinary IgG, urinary transferrin, and urinary albumin were analyzed. Renal biopsy specimens were immunohistochemically stained for nephrin and podocalyxin.
Of the total 581 cases of children with HSPN who underwent renal biopsy, 148 cases (25.5%) presented with nephrotic-range proteinuria. The pathological types of HSPN with nephrotic-range proteinuria were categorized as IIb, IIIa, IIIb, IIIb with diffuse EP, IVb, pure focal EP type, and pure diffuse EP type. Among these types, pure diffuse EP type accounted for 7.4%. The levels of 24-h proteinuria and urinary albumin were the highest in pure diffuse EP type among all pathological types, and the percentage of EP correlated with 24-h proteinuria and urinary albumin levels. 24-h proteinuria was significantly higher in pure diffuse EP type relative to HSPN IIb type, and significantly higher in IIIb with EP, compared with HSPN IIIb. Nephrin, but not podocalyxin, was downregulated in EP segment.
EP is an independent pathogenic factor in HSPN with nephrotic-range proteinuria. Downregulation of nephrin in EP segment is a potential molecular mechanism of nephrotic-range proteinuria. Albumin is the major urinary protein component in HSPN with EP.
KeywordsHSPN Nephrotic-range proteinuria Endocapillary proliferative
This study was funded by independent innovation and applied basic research project of university basic scientific research business in 2014 (No. 2014KYYWF-ZZCX2-03) and innovation team for treatment of Henoch-Schönlein purpura nephritis in children by Integrated Chinese and Western medicine methods (No. 18IRTSTHN028).
Compliance with ethical standards
Conflicts of interest
The authors declare that they have no conflict of interest.
For this type of study formal consent is not required.
- 3.Soylemezoglu O, Ozkaya O, Ozen S, Bakkaloglu A, Dusunsel R, Peru H, Cetinyurek A, Yildiz N, Donmez O, Buyan N, Mir S, Arisoy N, Gur-Guven A, Alpay H, Ekim M, Aksu N, Soylu A, Gok F, Poyrazoglu H, Sonmez F (2009) Henoch-Schonlein nephritis: a nationwide study. Nephron Clin Pract 112:c199–c204CrossRefGoogle Scholar
- 5.Roberts IS, Cook HT, Troyanov S, Alpers CE, Amore A, Barratt J, Berthoux F, Bonsib S, Bruijn JA, Cattran DC, Coppo R, D'Agati V, D'Amico G, Emancipator S, Emma F, Feehally J, Ferrario F, Fervenza FC, Florquin S, Fogo A, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hogg RJ, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Li LS, Li PK, Liu ZH, Mackinnon B, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H (2009) The Oxford classification of IgA nephropathy: pathology definitions, correlations, and reproducibility. Kidney Int 76:546–556CrossRefGoogle Scholar
- 8.Fu G, He XQ, Hu YX, Yu LF, Yang Q, Zhuang JQ (2016) Analysis of clinicopathology and outcome of childhood Henoch-Schonlein purpura nephritis with endocapillary proliferation. J Wenzhou Med Univ 46:330–334Google Scholar
- 10.Song CD, Ding Y, Zhai ZG, Zhai WS, Ren XQ, Guo QY, Zhang X, Yang M, Zhang J (2016) The clinical and pathological features of capillary proliferative purpura nephritis in 19 children. J Clin Pediatr 34:414–417Google Scholar
- 11.Zhao S, Huang SM, Zhang WZ, Bao HY, Wu HM, Zhang AH, Chen Y, Han Y, Zhao F (20l0) Analysis of clinicopathology and prognosis of childhood HenochSchonlein purpura nephritis with diffused endothelial cell proliferation. Chin J Nephrol 26:416–421Google Scholar
- 23.Fujita E, Nagahama K, Shimizu A, Aoki M, Higo S, Yasuda F, Mii A, Fukui M, Kaneko T, Tsuruoka S (2015) Glomerular capillary and endothelial cell injury is associated with the formation of necrotizing and crescentic lesions in crescentic glomerulonephritis. J Nippon Med Sch 82:27–35CrossRefGoogle Scholar