Validation of 2016 ACR/EULAR Classification Criteria of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis in Japanese Patients

Masaki Shimizu¹, Mao Mizuta¹, Takahiro Yasumi², Naomi Iwata³, Yuka Okura⁴, Noriko Kinjo⁵, Hiroaki Umebayashi⁶, Tomohiro Kubota⁷, Yasuo Nakagishi⁸, Kenichi Nishimura⁹, Masato Yashiro¹⁰, Junko Yasumura¹¹, Kazuko Yamazaki¹², Hiroyuki Wakiguchi¹³, Nami Okamoto¹⁴ and Masaaki Mori¹⁵, ¹Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan, ²Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan, ³Department of Immunology and Infectious Diseases, Aichi Children’s Health and Medical Center, Obu, Japan, ⁴Department of Pediatrics, KKR Sapporo Medical Center, Sapporo, Japan, ⁵Department of Pediatrics, Faculty of medicine, University of the Ryukyus, Nakagami-gun, Japan, ⁶Department of Rheumatics, Miyagi Children’s Hospital, Sendai, Japan, ⁷Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan, Kagoshima, Japan, ⁸Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan, ⁹Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan, ¹⁰Department of Pediatrics, Okayama University Hospital, Okayama, Japan, ¹¹Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima, Japan, ¹²Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan, ¹³Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan, ¹⁴Department of Pediatrics, Osaka Medical College, Takatsuki, Japan, ¹⁵Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: classification criteria and macrophage activation syndrome, Systemic JIA

Session Information

Date: Sunday, November 5, 2017

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster I: Autoinflammatory Disorders and Miscellaneous

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: To validate the 2016 ACR/EULAR classification criteria of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA) in Japanese patients.

Methods: A combination of expert consensus and analysis of real patient data was conducted by a panel of 15 paediatric rheumatologists. Eighty-three profiles comprised 25 patients with s-JIA–associated MAS and 58 patients with active s-JIA without evidence of MAS. From these profiles, 19 points for full-blown MAS, 16 points for MAS onset and 58 points for acute s-JIA without MAS were evaluated.

Results: Evaluation of the classification criteria to discriminate full-blown MAS from acute s-JIA without MAS showed a sensitivity of 0.947 and specificity of 1.000 at the time of full-blown MAS. Sensitivity was 0.438 and specificity 1.000 at the time of MAS onset. The number of measurement items that fulfilled the criteria increased in full-blown MAS compared to that at MAS onset. At MAS onset, the positive rates of patients who met the criteria for platelet counts and triglycerides were low, whereas those for aspartate aminotransferase and fibrinogen were relatively high. At full-blown MAS, the number of patients who met the criteria for each measurement item increased.

Conclusion: The classification criteria for MAS complicating s-JIA had a very high diagnostic performance. However, the diagnostic sensitivity for MAS onset was relatively low. For the early diagnosis of MAS in s-JIA, the dynamics of laboratory values during the course of MAS should be further investigated.

Disclosure: M. Shimizu, None; M. Mizuta, None; T. Yasumi, None; N. Iwata, None; Y. Okura, None; N. Kinjo, None; H. Umebayashi, None; T. Kubota, None; Y. Nakagishi, None; K. Nishimura, None; M. Yashiro, None; J. Yasumura, None; K. Yamazaki, None; H. Wakiguchi, None; N. Okamoto, None; M. Mori, None.

To cite this abstract in AMA style:

Shimizu M, Mizuta M, Yasumi T, Iwata N, Okura Y, Kinjo N, Umebayashi H, Kubota T, Nakagishi Y, Nishimura K, Yashiro M, Yasumura J, Yamazaki K, Wakiguchi H, Okamoto N, Mori M. Validation of 2016 ACR/EULAR Classification Criteria of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis in Japanese Patients [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/validation-of-2016-acreular-classification-criteria-of-macrophage-activation-syndrome-complicating-systemic-juvenile-idiopathic-arthritis-in-japanese-patients/. Accessed .

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