Sung Soo Ahn1, Byung-Woo Yoo1, Seung Min Jung1, Sang-Won Lee1, Yong-Beom Park1, Jason Jungsik Song2. 1. Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea. 2. Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea; Severance Institute for Vascular and Metabolic Research, Yonsei University College of Medicine, Seoul, South Korea. Electronic address: JSKSONG@yuhs.ac.
Abstract
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.
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 SLEpatients 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 SLEpatients (OR = 64.5; 95% CI: 7.6-544.4; p < 0.001). CONCLUSIONS: The 2016 classification criteria for MAS is useful to identify febrile SLEpatients at high risk for in-hospital mortality. Monitoring febrile SLEpatients with the new 2016 classification criteria might aid in the early detection of MAS.
Authors: Robert David Sandler; Stuart Carter; Harpreet Kaur; Sebastian Francis; Rachel Scarlett Tattersall; John Andrew Snowden Journal: Bone Marrow Transplant Date: 2019-08-27 Impact factor: 5.483
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