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

Author:

Shimizu Masaki1ORCID,Nishimura Kenichi2,Iwata Naomi3,Yasumi Takahiro4,Umebayashi Hiroaki5,Nakagishi Yasuo6,Okura Yuka7,Okamoto Nami8,Kinjo Noriko9,Mizuta Mao610,Yashiro Masato11,Yasumura Junko12,Wakiguchi Hiroyuki13ORCID,Kubota Tomohiro14,Mouri Mariko15,Kaneko Utako16,Mori Masaaki15ORCID

Affiliation:

1. Department of Child Health and Development, Graduate School of Medical and Dental Sciences Tokyo Medical and Dental University Tokyo Japan

2. Department of Pediatrics Yokohama City University Graduate School of Medicine Yokohama Japan

3. Department of Immunology and Infectious Diseases Aichi Children's Health and Medical Center Obu Japan

4. Department of Pediatrics Kyoto University Graduate School of Medicine Kyoto Japan

5. Department of Rheumatics Miyagi Children's Hospital Sendai Japan

6. Department of Pediatric Rheumatology Hyogo Prefectural Kobe Children's Hospital Kobe Japan

7. Department of Pediatrics KKR Sapporo Medical Center Sapporo Japan

8. Department of Pediatrics, School of Medicine Osaka Medical and Pharmaceutical University Takatsuki Japan

9. Department of Pediatrics, Faculty of Medicine University of the Ryukyus Nakagami‐gun Japan

10. Department of Pediatrics, Graduate School of Medical Sciences Kanazawa University Kanazawa Japan

11. Department of Pediatrics Okayama University Hospital Okayama Japan

12. Department of Pediatrics Hiroshima University Graduate School of Biomedical & Health Sciences Hiroshima Japan

13. Department of Pediatrics Yamaguchi University Graduate School of Medicine Ube Japan

14. Department of Pediatrics, Graduate School of Medical and Dental Sciences Kagoshima University Kagoshima Japan

15. Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences Tokyo Medical and Dental University Tokyo Japan

16. Department of Pediatrics Niigata University School of Medicine Niigata 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.

Publisher

Wiley

Subject

Rheumatology

Reference32 articles.

1. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

2. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders

3. The incidence of macrophage activation syndrome in children with rheumatic disorders;Moradinejad MH;Minerva Pediatr,2011

4. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis;Behrens EM;J Rheumatol,2007

5. Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: Report of five cases

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