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

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 the efficacy and safety of dexamethasone palmitate (DEX‐P) in the treatment of s‐JIA‐associated MAS. Methods This m...

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Bibliographic Details
Published in:International journal of rheumatic diseases 2023-05, Vol.26 (5), p.938-945
Main Authors: Shimizu, Masaki, Nishimura, Kenichi, Iwata, Naomi, Yasumi, Takahiro, Umebayashi, Hiroaki, Nakagishi, Yasuo, Okura, Yuka, Okamoto, Nami, Kinjo, Noriko, Mizuta, Mao, Yashiro, Masato, Yasumura, Junko, Wakiguchi, Hiroyuki, Kubota, Tomohiro, Mouri, Mariko, Kaneko, Utako, Mori, Masaaki
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones - therapeutic use
Adverse events
Arthritis
Arthritis, Juvenile - drug therapy
Cell activation
Child
Corticosteroids
Cyclosporine
Cyclosporins
Dexamethasone
dexamethasone palmitate
Humans
Japan
macrophage activation syndrome
Macrophage Activation Syndrome - drug therapy
Macrophages
Methylprednisolone
Palmitic acid
Patients
Pediatrics
Retrospective Studies
Rheumatology
Steroids
systemic juvenile idiopathic arthritis
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Summary: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 the efficacy and safety of dexamethasone palmitate (DEX‐P) in the treatment of s‐JIA‐associated MAS. Methods This 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. Results Methylprednisolone (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. Conclusions The 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.
ISSN:1756-1841
1756-185X
DOI:10.1111/1756-185X.14681