Abstract: Objective To summarize the clinical characteristics of childhood-onset systemic lupus erythematosus(cSLE), analyze the 5-year survival rate, causes of relapse and death, and factors affecting prognosis, in order to provide a basis for improving the prognosis and guiding the treatment. Methods Clinical data of 188 SLE patients treated during Jan. 1, 2009 and Dec. 31, 2020 were retrospectively collected and the patients were followed up in clinic or with telephone. The survival rate was analyzed using Kaplan-Meier method, and the prognostic factors were analyzed using Log-rank test and cox parametric regression analysis. Results Of the 188 children, 40 were male and 148 were female, mean age of onset were(10.64±2.12)years, median duration of illness from onset to diagnosis were 30(15, 60)days. The most common clinical manifestations were fever and rash. Renal damage and hematologic damage were often observed at diagnosis. During the follow-up, 73 children(38.8%)had relapse, mainly due to poor compliance and infection; 16 children died, mainly due to poor compliance, multiple organ damage, infection, and renal insufficiency. The 1-year, 3-year and 5-year survival rates were 94.1%, 92.9%, and 91.7%, respectively. Univariate analysis showed that cardiovascular system damage(χ2=4.464, P=0.035), neurological system damage(χ2=6.545, P=0.011), hematological system damage(χ2=3.888, P=0.049), massive proteinuria(χ2=5.641, P=0.018), severe activity of the initial disease(χ2=4.679, P=0.031), induction therapy disease inactivity(χ2=7.561, P=0.006), and relapse(χ2=13.786, P≤0.001)were associated with prognosis. Cox parametric regression analysis showed that cardiovascular system damage(HR=3.361, 95%CI: 1.251-9.029, P=0.016), neurological damage(HR=2.997, 95%CI: 1.076-8.349, P=0.036), massive proteinuria(HR=2.162, 95%CI: 1.079-4.334, P=0.030), and relapse(HR=4.663, 95%CI: 1.666-13.049, P=0.003)were independent risk factors of poor prognosis. Conclusion The main clinical manifestations of SLE are fever and rash, and systemic damages are renal damage and hematologic damage. The overall 5-year prognosis for patients complicated with cardiovascular damage, neurological damage, massive proteinuria and with recurrence is poor. Active control of organ damage, timely reduction of proteinuria, and prevention of recurrence are key to improving survival.

Key words: Systemic lupus erythematosus, Children, Survival rate, Prognosis