| Background and ObjectiveSystemic lupus erythematosus is a chronic inflammatory autoimmune disease involving multiple systems,which can lead to multi-tissue and multi-organ damage and has a high morbidity and mortality rate.cSLE has a more acute onset,more severe disease,a prolonged course,a high recurrence rate,and is associated with a higher mortality rate.The prevalence of initial and cumulative multiorgan system involvement tends to be higher in patients with cSLE than in adults with SLE.Despite improvements in diagnosis and treatment and a significant increase in the survival rate of SLE in children,the mortality rate of cSLE remains high.The purpose of this paper is to retrospectively analyze the clinical data of 188 children with SLE admitted to our hospital in 11 years,analyze the long-term survival rate and long-term renal survival rate,and analyze the factors affecting the prognosis to provide a basis for improving the prognosis and guiding the treatment of cSLE.MethodsClinical data of children with SLE who were treated in our pediatric renal rheumatology department from January 1,2009 to December 31,2020 were collected and followed up in clinic or by telephone..The survival rate and kidney survival rate were analyzed using the Kaplan-Meier method,and the prognostic factors were analyzed using the log-rank test and cox parametric regression analysis.Results1.188 children with SLE,40 males and 148 females,male to female ratio 1:3.7,mean age of onset 10.64±2.12 years,the median duration of illness from onset to diagnosis 30(15,60)days,no significant difference in age of onset by gender(P>0.05).2.The most common clinical manifestation was fever in 123 cases(65.4%),followed by acute or subacute rash in 129 cases(68.6%).The children were most often combined with renal damage and hematologic damage at the time of diagnosis,158(84.0%)and 118(62.8%)cases,respectively.Males were more likely to present with massive proteinuria than females,and the difference was statistically significant(P=0.044).The most common type of pathology in children with lupus nephritis was diffuse(type Ⅳ).Forty-nine cases(26.0%)were moderately active and 116 cases(61.7%)were severely active at the time of diagnosis3.Relapse and death:73 children(38.8%)had relapse during the follow-up period,mainly due to poor compliance and infection.16 children died,mainly due to poor compliance,multiple organ damage,infection,and renal insufficiency.4.Compliance:Overall 5-year follow-up rate of 85.8%.6 cases(3.8%)discontinued therapeutic drugs during the follow-up period,29 cases(18.4%)discontinued hormones,and 66 cases(41.8%)achieved different degrees of remission.5.Survival rate:the overall survival rates of children with SLE at 1,3,and 5 years were 94.1%,92.9%,and 91.7%,respectively.Cox parametric regression analysis showed that cardiovascular system damage,neurological damage,and massive proteinuria were independent risk factors for poor prognosis of SLE.6.Renal survival rate:the renal survival rates of children with lupus nephritis at 1,3 and 5 years were 98.6%,97.2%and 95.7%.cox multifactor regression analysis showed that elevated blood creatinine was an independent risk factor for poor renal prognosis,and induction of complete remission with treatment was an independent protective factor for renal prognosis.Conclusion1.The main clinical manifestations of children with SLE in this study were fever and rash most often,and systemic damage was most often renal damage and hematologic damage.2.The 5-year overall survival rate of children with SLE in this study was 91.7%.Cardiovascular system damage,neurological damage,massive proteinuria and recurrence were independent risk factors for the poor overall prognosis of SLE,and active control of organ damage,timely reduction of proteinuria and prevention of disease recurrence during treatment were the keys to improving the survival rate.3.The 5-year renal survival rate of children with SLE in this study was 95.7%.Elevated blood creatinine was an independent risk factor for poor renal prognosis,and complete remission by induction therapy was an independent protective factor for renal prognosis.Active control of renal damage during treatment and monitoring of renal function indexes to prevent progression were key to improving renal survival. |
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