Application of the 2016 EULAR/ACR/PRINTO Classification Criteria for Macrophage Activation Syndrome in Patients with Adult-onset Still Disease

被引:55
作者
Ahn, Sung Soo [1 ]
Yoo, Byung-Woo [1 ]
Jung, Seung Min [1 ]
Lee, Sang-Won [1 ]
Park, Yong-Beom [1 ]
Song, Jason Jungsik [1 ,2 ]
机构
[1] Yonsei Univ, Coll Med, Div Rheumatol, Dept Internal Med, 50-1 Yonsei Ro, Seoul 03722, South Korea
[2] Yonsei Univ, Coll Med, Severance Inst Vasc & Metab Res, Seoul, South Korea
基金
新加坡国家研究基金会;
关键词
MACROPHAGE ACTIVATION SYNDROME; ADULT-ONSET STILL DISEASE; SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS; FERRITIN; HEMOPHAGOCYTIC SYNDROME; PROGNOSTIC-FACTORS; CLINICAL-FEATURES; FERRITIN LEVELS; SERUM FERRITIN; DIAGNOSIS; GUIDELINES;
D O I
10.3899/jrheum.161286
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objective. To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with adult-onset Still disease (AOSD). Methods. We performed a retrospective analysis of patients with AOSD with fever who were admitted to Severance Hospital between 2005 and 2016. The patients with AOSD were evaluated for MAS using the 2016 classification criteria for MAS. Clinical features, laboratory findings, and overall survival were analyzed. Logistic regression analysis was used to evaluate the factors associated with in-hospital mortality. Results. Among 64 patients with AOSD, 36 (56.3%) were classified as having MAS. The overall survival rate was significantly lower in patients with MAS than in those without (67% vs 100%, p < 0.001). Multivariate analysis showed that a low erythrocyte sedimentation rate, a low albumin level, an increase in ferritin of over 2 folds, and the development of MAS on admission were significantly associated with mortality in patients with AOSD. Conclusion. The 2016 EULAR/ACR/PRINTO classification criteria for MAS are potentially useful for the identification of patients with AOSD at high risk for a poor outcome. Febrile patients with AOSD should be monitored with the 2016 classification criteria for MAS in the early diagnosis and proper treatment of MAS.
引用
收藏
页码:996 / 1003
页数:8
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