Treatment for macrophage activation syndrome associated with systemic juvenile idiopathic arthritis in Japan
Corresponding Author
Masaki Shimizu
Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Correspondence
Masaki Shimizu, Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8510, Japan.
Email: [email protected]
Search for more papers by this authorKenichi Nishimura
Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan
Search for more papers by this authorNaomi Iwata
Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Obu, Japan
Search for more papers by this authorTakahiro Yasumi
Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan
Search for more papers by this authorHiroaki Umebayashi
Department of Rheumatics, Miyagi Children's Hospital, Sendai, Japan
Search for more papers by this authorYasuo Nakagishi
Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan
Search for more papers by this authorYuka Okura
Department of Pediatrics, KKR Sapporo Medical Center, Sapporo, Japan
Search for more papers by this authorNami Okamoto
Department of Pediatrics, School of Medicine, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
Search for more papers by this authorNoriko Kinjo
Department of Pediatrics, Faculty of Medicine, University of the Ryukyus, Nakagami-gun, Japan
Search for more papers by this authorMao Mizuta
Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan
Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan
Search for more papers by this authorMasato Yashiro
Department of Pediatrics, Okayama University Hospital, Okayama, Japan
Search for more papers by this authorJunko Yasumura
Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima, Japan
Search for more papers by this authorHiroyuki Wakiguchi
Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan
Search for more papers by this authorTomohiro Kubota
Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
Search for more papers by this authorMariko Mouri
Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Search for more papers by this authorUtako Kaneko
Department of Pediatrics, Niigata University School of Medicine, Niigata, Japan
Search for more papers by this authorMasaaki Mori
Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Search for more papers by this authorCorresponding Author
Masaki Shimizu
Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Correspondence
Masaki Shimizu, Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8510, Japan.
Email: [email protected]
Search for more papers by this authorKenichi Nishimura
Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan
Search for more papers by this authorNaomi Iwata
Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Obu, Japan
Search for more papers by this authorTakahiro Yasumi
Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan
Search for more papers by this authorHiroaki Umebayashi
Department of Rheumatics, Miyagi Children's Hospital, Sendai, Japan
Search for more papers by this authorYasuo Nakagishi
Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan
Search for more papers by this authorYuka Okura
Department of Pediatrics, KKR Sapporo Medical Center, Sapporo, Japan
Search for more papers by this authorNami Okamoto
Department of Pediatrics, School of Medicine, Osaka Medical and Pharmaceutical University, Takatsuki, Japan
Search for more papers by this authorNoriko Kinjo
Department of Pediatrics, Faculty of Medicine, University of the Ryukyus, Nakagami-gun, Japan
Search for more papers by this authorMao Mizuta
Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan
Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan
Search for more papers by this authorMasato Yashiro
Department of Pediatrics, Okayama University Hospital, Okayama, Japan
Search for more papers by this authorJunko Yasumura
Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima, Japan
Search for more papers by this authorHiroyuki Wakiguchi
Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan
Search for more papers by this authorTomohiro Kubota
Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
Search for more papers by this authorMariko Mouri
Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Search for more papers by this authorUtako Kaneko
Department of Pediatrics, Niigata University School of Medicine, Niigata, Japan
Search for more papers by this authorMasaaki Mori
Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Search for more papers by this authorAbstract
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.
CONFLICT OF INTEREST STATEMENT
Tokyo Medical and Dental University (TMDU) received unrestricted research grants for Department of Lifetime Clinical Immunology from AbbVie GK, Ayumi Pharmaceutical, Chugai Pharmaceutical, CSL Behring, Japan Blood Products Organization, Mitsubishi Tanabe Pharma, Nippon Kayaku, Ono Pharmaceutical, Towa Pharmaceutical, and UCB Japan. TMDU paid the salary of Masaaki Mori. The authors declare they have no competing interests.
Open Research
DATA AVAILABILITY STATEMENT
The data supporting the findings of this study are available on request from the corresponding author.
Supporting Information
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Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
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