Treatment for macrophage activation syndrome associated with systemic juvenile idiopathic arthritis in Japan
© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:26 |
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| Enthalten in: |
International journal of rheumatic diseases - 26(2023), 5 vom: 27. Mai, Seite 938-945 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Shimizu, Masaki [VerfasserIn] |
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| Links: |
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| Themen: |
83HN0GTJ6D |
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| Anmerkungen: |
Date Completed 03.05.2023 Date Revised 03.05.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1111/1756-185X.14681 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM35478059X |
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| 245 | 1 | 0 | |a Treatment for macrophage activation syndrome associated with systemic juvenile idiopathic arthritis in Japan |
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| 500 | |a Date Revised 03.05.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd. | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Multicenter Study | |
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| 700 | 1 | |a Iwata, Naomi |e verfasserin |4 aut | |
| 700 | 1 | |a Yasumi, Takahiro |e verfasserin |4 aut | |
| 700 | 1 | |a Umebayashi, Hiroaki |e verfasserin |4 aut | |
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| 700 | 1 | |a Okura, Yuka |e verfasserin |4 aut | |
| 700 | 1 | |a Okamoto, Nami |e verfasserin |4 aut | |
| 700 | 1 | |a Kinjo, Noriko |e verfasserin |4 aut | |
| 700 | 1 | |a Mizuta, Mao |e verfasserin |4 aut | |
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| 700 | 1 | |a Mori, Masaaki |e verfasserin |4 aut | |
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