Treatment for macrophage activation syndrome associated with systemic juvenile idiopathic arthritis in Japan

Abstract

AbstractObjectivesTo clarify how pediatric rheumatologists treat systemic juvenile idiopathic arthritis (s‐JIA) associated macrophage activation syndrome (MAS) in the real world and to assess the efficacy and safety of dexamethasone palmitate (DEX‐P) in the treatment of s‐JIA‐associated MAS.MethodsThis multicenter, retrospective study was conducted at 13 pediatric rheumatology institutes in Japan. This study included 28 patients with s‐JIA‐associated MAS. Clinical findings, such as treatment details and adverse events, were evaluated.ResultsMethylprednisolone (mPSL) pulse therapy was selected as the first‐line treatment in more than half of the patients with MAS. Cyclosporine A (CsA) was used as first‐line therapy in combination with corticosteroids in half of the patients with MAS. DEX‐P and/or CsA were selected as the second‐line therapy in 63% of patients with corticosteroid‐resistant MAS. Plasma exchange was selected as the third‐line therapy for DEX‐P and CsA‐resistant MAS. All patients improved and there were no characteristically severe adverse events associated with DEX‐P.ConclusionsThe first‐line treatment for MAS in Japan is mPSL pulse therapy and/or CyA. DEX‐P could be an effective and safe therapeutic option for patients with corticosteroid‐resistant MAS.