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

• DOI: 10.1111/1756-185x.14681
• Publication Date: 2023-03-28T01:24:00Z
• Authors: Masaki Shimizu; Kenichi Nishimura; Naomi Iwata; Takahiro Yasumi; Hiroaki Umebayashi; Yasuo Nakagishi; Yuka Okura; Nami Okamoto; Noriko Kinjo; Mao Mizuta; Masato Yashiro; Junko Yasumura; Hiroyuki Wakiguchi; Tomohiro Kubota; Mariko Mouri; Utako Kaneko; Masaaki Mori
• Keywords: Macrophage Activation Syndrome

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