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In-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome

 Sung Soo Ahn  ;  Byung-Woo Yoo  ;  Seung Min Jung  ;  Sang-Won Lee  ;  Yong-Beom Park  ;  Jason Jungsik Song 
 Seminars in Arthritis and Rheumatism, Vol.47(2) : 216-221, 2017 
Journal Title
 Seminars in Arthritis and Rheumatism 
Issue Date
Adolescent ; Adult ; Female ; Fever/complications ; Fever/mortality* ; Hospital Mortality ; Humans ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/mortality* ; Macrophage Activation Syndrome/classification* ; Macrophage Activation Syndrome/complications ; Macrophage Activation Syndrome/diagnosis ; Macrophage Activation Syndrome/mortality* ; Male ; Middle Aged ; Retrospective Studies ; Survival Rate ; Young Adult
Ferritin ; Macrophage activation syndrome ; Systemic lupus erythematosus ; Systemic onset juvenile idiopathic arthritis
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 febrile systemic lupus erythematosus (SLE). METHODS: We performed a retrospective analysis of SLE patients with fever, who were admitted to Severance Hospital between December 2005 and May 2016. Patients were evaluated for MAS using the 2016 classification criteria for MAS. Clinical features and laboratory findings were compared and overall survival rate was analyzed. Forward and backward stepwise logistic regression analysis was used to evaluate the factors associated with in-hospital mortality. RESULTS: Among 157 patients with SLE, 54 (34.3%) were considered to have MAS on admission (n = 42) and during admission (n = 12). For patients who already have MAS on admission, their baseline laboratory findings demonstrated lower CRP, platelets, total protein, albumin, complement C3, fibrinogen and higher AST, ALT, total bilirubin, ferritin, and triglyceride. The overall survival rate was significantly lower in patients with MAS than without MAS (64.8% vs. 97.0%, p < 0.001). Multivariate analysis showed that the presence of MAS was significantly associated with in-hospital mortality in febrile SLE patients (OR = 64.5; 95% CI: 7.6-544.4; p < 0.001). CONCLUSIONS: The 2016 classification criteria for MAS is useful to identify febrile SLE patients at high risk for in-hospital mortality. Monitoring febrile SLE patients with the new 2016 classification criteria might aid in the early detection of MAS.
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1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실)
Yonsei Authors
박용범(Park, Yong Beom) ; 송정식(Song, Jung Sik) ; 안성수(Ahn, Sung Soo) ; 이상원(Lee, Sang Won) ; 정승민(Jung, Seung Min )
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