2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis (selected translation) / 中华实用儿科临床杂志

The American College of Rheumatology (ACR) updated the 2011 recommendations for juvenile idiopathic arthritis (JIA) in 2013 and focused on the treatment of systemic-onset juvenile idiopathic arthritis (SOJIA).According to the clinical feature of the SOJIA,the subtype is developed to 3 phenotypes:(1) phenotype of systemic JIA with active systemic features and yarying degrees of synovitis; (2) phenotype of systemic JIA without active systemic features and with varying degrees of active synovitis ; (3) phenotype of systemic JIA with features concerning for macrophage activation syndrome (MAS).The treatment recommendation was made according each phenotype,including initial therapeutic options and therapeutic options for continued disease activity.And uncertain or inappropriate options for continued disease were listed alphabetically.Development of treatment recommendations for children with SOJIA and features of MAS is particularly challenging.However,it is anticipated that in the near future,with research of the disease process,the better recommendation may be made.

The Report of macrophage activation syndrome in 9 children / 中华风湿病学杂志

Objective To analyze the clinical characteristics of macrophage activation syndrome (MAS). Methods Clinical data was analyzed in 9 children who had been diagnosed as MAS in our hospital from Jan, 2003 to Aug, 2006. Results Seven children were boys, 2 children were girls, aged 5 months to 12 years. Clinical manifestations included long-term fever, hemophagocytic cell in bone marrow, anemia, arthritis, enlargement of lymph nodes, enlargement of liver and spleen, liver dysfunction, abnormal fat metabolism. Two cases had acute respiratory, distress syndrome(ARDS), 2 cases were complicated with multi- pie organ failure(MOF), two patients died. Glucocorticoid combined with immunosuppressive therapy were effective, HP(Plasma Exchange)was applied in one severe case and was shown to he effective. Conclusion MAS is a serious complication of JIA, especially in systemic-onset juvenile idiopathic arthritis. It is very im- portant to recognize and treat MAS earlier.