Clinicopathological And Prognostic Study Of Pediatric Primary Focal Segmental Glomerulosclerosis

ObjectIn order to find the risk factors related to prognosis,long-term renal outcome of children with primary FSG and provide evidence-based basis for clinical diagnosis and treatment,we retrospectively analyzed the clinical,pathological and follow-up data of children with primary FSGS in our center.MethodsPart 1:The clinical,pathological,and follow-up data of 274 children with primary FSGS were retrospectively analyzed.The long-term cumulative renal survival was calculated by Kaplan-Meier survival analysis,and prognostic risk factors were identified by Cox multivariate regression analysis.Part 2:The data of glomerular immune deposits in 264 children with primary FSGS were retrospectively analyzed.The effects of IgM and C3 deposition and locations on prognosis were determined by Kaplan-Meier survival analysis,Cox multivariate regression analysis and regression analysis after propensity score matching.ResultsPart 1:The median follow-up time of 274 children was 41.5(19.1-63.7)months,and 54 children(19.7%)progressed to an endpoint event.Kaplan-Meier survival analysis showed that the renal survival rates at 5,10,and 15 years were 80.7%,62.6%,and 34.7%,respectively.Multivariate Cox regression analysis showed that chronic renal tubule/interstitial lesions≥25%(HR 4.14,95%CI 1.49-11.50,P<0.01),collapse variant[(control group:non-specific variant)HR 2.16,95%CI 1.10-4.27,P=0.03]and proportion of glomerular segmental sclerosis(HR 1.03,95%CI 1.011.04,P<0.01)were independent risk factors for poor renal prognosis.ROC curve analysis suggested that Columbia variant(control group:non-specific variant),≥25%chronic renal tubular/interstitial injury and glomerular segmental sclerosis ratio could effectively predicted the diagnostic efficacy of progression to composite endpoint events,and their AUC values were 0.732,0.729,and 0.715,respectively.Part 2:The median follow-up time of 264 patients was 39.9(18.4-62.3)months.109 cases(41.3%)had glomerular IgM deposition,55 cases(20.8%)had glomerular vascular loop IgM deposition,30 cases(11.4%)had glomerular IgM and C3 codeposition,and 22 cases(8.3%)had glomerular vascular loop IgM and C3 codeposition.Cox regression analysis showed that glomerular vascular loop IgM and C3 co-deposition was an independent risk factor for poor prognosis of primary FSGS in children(HR 2.38,95%CI 1.08-5.25,P=0.03).The clinical prognostic value of glomerular vascular loop IgM and C3 co-deposition in children with primary FSGS was further verified by regression analysis after propensity score matching(PSM).ConclusionThe long-term prognosis of primary FSGS in children is poor.collapse variant,≥25%chronic renal tubular/interstitial injury,glomerular segmental sclerosis and glomerular vascular loop IgM and C3 co-deposition were the independent risk factors for poor outcomes.