Application of the 2016 European League Against Rheumatism/American College of Rheumatology/Pediatric Rheumatology International Trials Organization classification criteria for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis in adult-onset Still disease
10.3760/cma.j.issn.1007-7480.2018.08.002
- VernacularTitle:全身型幼年特发性关节炎合并巨噬细胞活化综合征分类标准在成人斯蒂尔病中的应用探讨
- Author:
Ran WANG
1
;
Ting LI
;
Shuang YE
;
Qiong FU
;
Chunde BAO
Author Information
1. 200001,上海交通大学医学院附属仁济医院风湿科
- Keywords:
Still's disease,adult-onset;
Macrophage activation;
Arthritis,juvenile rheumatoid;
Prognosis
- From:
Chinese Journal of Rheumatology
2018;22(8):508-515
- CountryChina
- Language:Chinese
-
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＜0.01;10.7％ vs 0.9％,OR(95％CI)=13.21 (1.56,113.57),P＜0.01],also the incidence of liver dysfunction was higher in AOSD with MAS [67.8％ vs 11.5％,OR(95％CI)=0.18(7.26,36.33),P＜0.01].Among the AOSD with MAS,62.5％(35/56) of these patients received large-dose glucocorticoid therapy,5.4％ (3/56) received the glucocorticoid pulse therapy,48.2％(27/56) were treated with IVIG,and 26.8％(15/56) were treated with calcium phosphatase inhibitors.The mortality rates of AOSD with MAS was 8.9％(5/56),which was significantly higher than 1.8％(2/113) (OR =5.44,P＜0.05),the mortality rate of the AOSD without MAS.Patients who fulfilled the sJIA-MAS criteria suggested poor prognosis (OR=0.041,P=5.44),and the platelet count ≤ 181× 109/L (OR=12.17,P=0.002),alanine aminotransferase ＞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.
