Abstract
Objective In 2016, European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric rheumatology international trials organization (PRINTO) released the classification criteria for macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA). Due to the similarities of both clinical manifestations and pathogenesis between adult-onset Still dsease (AOSD) and sJIA, we hope to evaluate the 2016 sJIA-AMS classification in AOSD patients. Methods A total of 169 AOSD patients who were hospitalized in Renji Hospital were enrolled in this study. AOSD patients were divided into AOSD with MAS and AOSD without MAS, using the 2016 sJIA-MAS criteria. The data of the two groups were analyzed by Chi-square test, Mann-Whitney U test and binary Logistic analysis, and factors influencing the prognosis of patients were analyzed by Kaplan-Meier and COX regression analysis. Results According to sJIA-MAS criteria, 56 AOSD patients with MAS were identified in all the 169 AOSD cases. In AOSD patients, the incidence of splenomegaly and pericarditis/myocarditis was significantly higher in patients with MAS than in AOSD without MAS [42.9% vs 14.2%, OR(95%CI)=4.50(2.13, 9.51), P 48 U/L (OR=9.43, P=9.040) were also highly suggestive of poor prognosis. Conclusion The 2016 sJIA-MAS classification criteria are particularly valuable for early recognization of MAS in AOSD patients, and convenient to use. AOSD patients fulfilled sJIA-MAS criteria are more severe, and require larger doses of glucocorticoid and more immunosuppression therapy compared to patients without MAS, and the prognosis of these patients is also poor. Key words: Still's disease, adult-onset; Macrophage activation; Arthritis, juvenile rheumatoid; Prognosis
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
