Clinical characteristics and follow-up study of 192 cases of juvenile dermatomyositis

WEN Xinran; ZHANG Junmei; KUANG Weiying; DENG Jianghong; TAN Xiaohua; LI Chao; LI Shipeng; LI Caifeng

doi:10.3969/j.issn.1674-3806.2024.09.05

Abstract：Objective To study the treatment,follow-up and prognosis of pediatric patients with juvenile dermatomyositis(JDM),and to explore the factors affecting the recurrence of the disease.Methods The clinical data of pediatric patients with JDM who were diagnosed and treated in the Department of Rheumatology of Beijing Children's Hospital,Capital Medical University from June 2014 to June 2019 and were followed up for at least 6 months were retrospectively analyzed.Kaplan-Meier method was used to plot the survival curve,and Cox regression was used to analyze the factors affecting the recurrence of the disease.Results Among the 192 pediatric patients with JDM,the ratio of males to females was 1∶1.46,and the median age of the patients with onset was 65.5(43.5,98.8)months.The combination treatment of tofacitinib,thalidomide,and tocilizumab improved the disease in some refractory pediatric patients with JDM.Kaplan-Meier survival curve showed that the median time of the pediatric patients to achieve complete clinical response was 12 months.The results of multivariate Cox regression analysis showed that long time from disease onset to treatment[RR(95％CI):1.042(1.001-1.084),P=0.044]was a risk factor of disease recurrence,and positive heliotrope rash[RR(95％CI):0.208(0.049-0.894),P=0.035]at the time of disease onset was a protective factor of avoiding disease recurrence.Conclusion Most pediatric patients with JDM can achieve complete clinical response after treatment,and some of them may experience the disease recurrence.The application of thalidomide,tofacitinib and tocilizumab may improve the prognosis of the pediatric patients with refractory JDM.The long time from disease onset to treatment is a risk factor of the disease recurrence.