Abstract: Objective　To investigate the clinical and pathological characteristics of Henoch-Schönlein purpura nephritis with diffuse capillary endothelial cell proliferation as pathological manifestation. Methods　The clinical manifestations and pathology of capillary endothelial proliferative purpura nephritis (DEP-HSPN) diagnosed by renal biopsy were retrospectively analyzed in 50 children in recent 5 years. Results　The pathological lesions in 50 cases included simple DEP-HSPN in 11 cases (7 males and 4 females) and capillary endothelial cell proliferation combined with crescents formation (non-simple DEP-HSPN) in 39 cases (27 males and 12 females). There was no significant difference in the course of disease and age between the two groups (P>0.05). The clinical type of 11 cases of simple DEP-HSPN was type III. In 39 cases of non-simple DEP-HSPN, 16 cases were type III and 23 cases were type V. All of the children had hematuria and proteinuria. The incidence of gross hematuria, urine red blood cell count, 24 h urine protein, and serum creatinine levels in children with non-simple DEP-HSPN were significantly higher than those in simple DEP-HSPN group, but the plasma albumin level was significantly lower than that in simple DEPHSPN group. It was easy to have crescent formation in DEP-HSPN, and the rate of crescent formation was 11.1% (5.0%-27.6%). The incidence of segmental lesions and renal tubular interstitial damage was low. All children had non simple IgA deposits in the mesangial area. In the 50 children treated for 1 year, 22 had complete remission, 28 had asymptomatic hematuria, and none had active nephropathy and renal insufficiency. In 32 cases of non-simple DEP-HSPN, the 24 h urinary protein, plasma albumin level, and the incidences of gross hematuria and microscopic hematuria were statistically different before treatment and 1, 3, 6, 12 months after treatment (P<0.01). The 24 h urine protein and gross hematuria gradually decreased with the prolongation of treatment, while the level of plasma albumin was gradually increased. Conclusions　DEP-HSPN is characterized by gross hematuria and proteinuria. The onset is acute and it is easy to have crescent formation. When combined with crescent formation, the clinical symptoms are more severe. The combination of strong immunosuppressive agents and long-term sequential follow-up treatment is effective in acute stage. The prognosis is good.
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