Date: Sunday, November 5, 2017
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.
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 September 25, 2021.
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