A Long-term Follow-up Study Of Pathological Classification And Clinical Outcome Of Henoch-Sch(?)nlein Purpura Nephritis In A Single Center

Henoch-Schonlein purpura is the most common systemic vasculitis in childhood.Some children may have clinical manifestations of renal and become HenochSchonlein purpura nephritis(HSPN).The International Study of Kidney Disease in Children(ISKDC)is a commonly used method to evaluate renal histology of HSPN at home and abroad.Although it is simple and rapid to evaluate the grading and guide treatment,it has limitations in predicting prognosis.Oxford classification shows its superiority in guiding the treatment and prognosis of primary IgA nephropathy.In view of the clinical and pathophysiological similarities between HSPN and IgA nephropathy,some studies have applied Oxford classification to HSPN.However,there is currently a lack of large-sample clinical data to support the application of Oxford typing in HSPN in children.Therefore,this study retrospectively analyzed the pathological and clinical data of children with HSPN in our center since 2001.We adopted the ISKDC classification and Oxford classification to compare the advantages and disadvantages of the two methods in evaluating the prognosis.We also analyzed the prognosis of children with HSPN followed up for more than 5 years to find valuable indicators to guide the prognosis.Part1 Application of different pathological classification methods in children with Henoch-Schonlein purpura nephritis(a retrospective study)Objective:The purpose of this paper was to discuss the predictive value of ISKDC classification and Oxford Classification(MEST-C scores)in children with HSPN.Methods:We retrospectively analyzed 877 children with HSPN who were admitted to our hospital and underwent renal biopsy from January 2001 to June 2019.The pathological and clinical data were collected,and the clinicopathological features and outcomes were summarized.The primary outcome was defined as estimated glomerular filtration rate(eGFR)<90 mL/min/1.73m2.Results:A total of 877 children was enrolled in the study.The median follow-up time was 23.3(10.9-47.9)months,and 51 patients(5.8%)reached the primary endpoint event,indicating that the majority of HSPN patients had a good prognosis.As is revealed in a Kaplan-Meier plot,segmental glomerulosclerosis(S)(P<0.001)and tubular atrophy/interstitial fibrosis(T)(P<0.001)significantly predict the poor renal outcome.Other Oxford lesions and ISKDC classification,however,did not show a significant difference in a worse outcome.It also indicates that the Oxford classification is superior to the ISKDC classification in predicting prognosis.In a multivariate Cox model,eGFR(HR=2.831,95%CI=1.359-5.896),S lesion(HR=3.936,95%CI=2.0787.457)and T lesion(HR=4.002,95%CI=1.733-9.242)were independent risk factors for renal outcome.Conclusion:Oxford classification has a good application value and prospect in the pathological classification of children with HSPN and can make up for the deficiency of ISKDC classification.The decrease of EGFR,S and T are independent risk factors for predicting renal prognosis,which should be paid clinical attention.Part2 A Five-year follow-up study of the pathological and clinical study of 130 children with Henoch-Schonlein purpura nephritisObjective:To analyze the clinical features and prognosis of children with HSPN who were followed up for more than 5 years.Methods:We enrolled children who performed renal biopsy at our center and were followed up for more than 5 years between January 2001 and June 2019,and then performed the prognostic analysis.We defined the primary endpoint event as eGFR<90 mL/min/1.73m2.Results:A total of 130 children with HSPN were enrolled in our study.The median onset age was 11.7(8.6-13.3)years old,of whom 71 were male(54.6%).At a median follow-up of 100.0(75.8-119.0)months,12 of 130 children with HSPN achieved the primary endpoint event.Compared with the no outcome group,the outcome group had a higher incidence of hypertension,higher levels of 24-hour proteinuria,serum cholesterol(CHO),serum uric acid(UA),and serum creatinine(Scr),and lower levels of serum albumin(Alb)at renal biopsy.There was no statistical difference in treatment between the two groups.In terms of pathological features,more patients showed mesangial hyperplasia(M),S1,T1&T2 and Glomerulus-Bowman’s capsule adhesion in the outcome group.A univariate Cox regression analysis showed that hypertension(P=0.003),UA(P=0.001),24-hour urinary protein level(>3.5g/d)(P=0.008),M(P=0.016),S(P=0.001),T(P<0.001)were the main risk factors affecting renal prognosis.A multivariate Cox regression model showed that S was significantly associated with renal prognosis(HR=7.739,95%CI=1.422-42.114).As is revealed in a Kaplan-Meier plot,renal survival was significantly shorter in S1 patients than in S0 patients(P=0.001).The ROC curve showed S accurately predicted the outcome(area under the curve:0.7 1 0,95%CI:0.549-0.872)with 0.667(95%CI:0.349-0.901)sensitivity and 0.754(95%CI:0.6666-0.829)specificity.Conclusions:There were differences in clinical manifestations(hypertension,UA,Alb,24-hour urinary protein level)and pathological characteristics between the outcome group and no outcome group in children with HSPN at renal biopsy.S is an independent risk factor affecting renal prognosis and has a diagnostic value.