Clinical Manifestations,Pathological Features And Prognosis Of Pediatric Lupus Nephritis:A Chinese Single-Center Study

Objective: To evaluate the clinical manifestations,pathological features and prognosis of pediatric lupus nephritis(LN),and analyze the risk factors that affect the prognosis of LN.Methods: We performed a retrospective review of 220 patients who were diagnosed as LN in Children’s Hospital of Chongqing Medical University during the period from January 2012 to December 2018.All data were analyzed by statistical software of SPSS 25.0.Result:(1)Of the 220 patients enrolled in this study,one hundred and seventy(77.27%)were female and fifty(22.73%)were male.The female‐to‐male ratio was 3.4:1.The mean age at diagnosis was 10.94±2.49 years,and the peak age of morbidity ranged from 9 to 15 years old.(2)The most common manifestation of LN was proteinuria(81.36%),the most common extra-renal manifestations were fever(65.45%)and rash(60.45%).Hematologic was the most common involved domain(67.27%)except the kidney.(3)The most common clinical classification of LN was nephrotic syndrome(57.14%),and the most common pathological type was Class Ⅳ(33.33%).Activity index(AI)of Class Ⅳ LN was significantly higher than Class Ⅲ(P<0.001).The 24-hour urine protein level of Class Ⅳ LN was significantly higher than Class Ⅲ(P=0.010),and the level of e-GFR of Class V LN was significantly higher than Class Ⅲ(P=0.016)and Class Ⅳ(P<0.001).(4)The 1-year,3-year,and 5-year overall cumulative survival rates were 93.5%,87.8%,and 86.5% respectively.The 5-year cumulative renal survival rate was 97.1%,the renal remission rate was 64.96% at the end of follow-up,and the renal flare rate was 23.47%.(5)Infection was the leading cause of death of LN in our cohort(50.00%),followed by neuropsychiatric lupus(30.00%).(6)At the end of follow-up,34.31% of patients achieved different levels of remission,and 43.07% of them achieved low disease activity status(LDAS).(7)The efficacy of corticosteroids combined with immunosuppressive agents was significantly better than only corticosteroids as the induction therapy for LN(P=0.010),and there was no significant difference of the efficacy between cyclophosphamide(CTX)and mycophenolate mofetil(MMF).(8)In univariate analysis,hypoproteinemia(P=0.025),serum creatinine(P=0.015),e-GFR(P=0.030),anti-doublestranded DNA antibody positive(P=0.045),nephrotic-range proteinuria(P=0.035),hypertension(P=0.018),central nervous system involvement(P=0.001),non-compliance with treatment(P<0.001),SLEDAI-2K score(P=0.011)and activity index(AI)(P=0.014)were all risk factors that affect the prognosis of LN.(9)The results of multivariate logistics regression analysis showed that hypertension,central nervous system involvement,non-compliance with treatment and e-GFR were independent risk factors that affect the prognosis of LN.Conclusion: The most common manifestation of LN was proteinuria in our cohort,and Class Ⅳ LN was the most common pathological type.The efficacy of corticosteroids combined with immunosuppressive agents was significantly better than only corticosteroids as the induction therapy for LN,and there was no significant difference of the efficacy between CTX and MMF.Infection was the leading cause of death of LN in our cohort,followed by neuropsychiatric lupus.Hypertension,central nervous system involvement,non-compliance with treatment and e-GFR were independent risk factors that affect the prognosis of LN in our cohort.