Li-Xia Zou1, Yun Zhu2, Li Sun3, Hui-Hui Ma4, Si-Rui Yang5, Hua-Song Zeng6, Ji-Hong Xiao7, Hai-Guo Yu4, Li Guo1, Yi-Ping Xu1, Mei-Ping Lu8. 1. Department of Rheumatology Immunology and Allergy, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Lane, Hangzhou, 310003, China. 2. Department of Nephrology and Immunology, Children's Hospital of Soochow University, 92 Zhongnan Street, Suzhou, 215003, China. 3. Department of Rheumatology, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai, 201102, China. 4. Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, 72 Guangzhou Road, Nanjing, 210008, China. 5. Department of Pediatric Rheumatology and Allergy, The First Hospital of Jilin University, 71 Xinming Street, Changchun, 130021, China. 6. Pediatric Allergy, Immunology and Rheumatology Department, Guangzhou Children's Hospital, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, 318 Renminzhong Road, Guangzhou, 510120, China. 7. Department of Pediatric, The First Affiliated Hospital of Xiamen University, 55 Zhenhai Road, Xiamen, 361003, China. 8. Department of Rheumatology Immunology and Allergy, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Lane, Hangzhou, 310003, China. meipinglu@zju.edu.cn.
Abstract
BACKGROUND: Macrophage activation syndrome (MAS) is a major cause of morbidity and mortality in pediatric rheumatology. We aimed to further understand the clinical features, treatment, and outcome of MAS in China. METHODS: A multi-center cohort study was performed in seven hospitals in China from 2012 to 2018. Eighty patients with MAS were enrolled, including 53 cases with systemic juvenile idiopathic arthritis (SJIA-MAS), 10 cases of Kawasaki disease (KD-MAS), and 17 cases of connective tissue disease (CTD-MAS). The clinical and laboratory data were collected before (pre-), at onset, and during full-blown stages of MAS. We compared the data among the SJIA-MAS, KD-MAS, and CTD-MAS subjects. RESULTS: 51.2% of patients developed MAS when the underlying disease was first diagnosed. In patients with SJIA, 22.6% (12/53) were found to have hypotension before the onset of SJIA-MAS. These patients were also found to have significantly increased aspartate aminotransferase (AST) and lactate dehydrogenase (LDH), as well as decreased albumin (P < 0.05), but no difference in alanine aminotransferase, ferritin, and ratio of ferritin/erythrocyte sedimentation rate (ESR) at onset of MAS when compared to pre-MAS stages of the disease. In addition, ferritin and ratio of ferritin/ESR were significantly elevated in patients at full-blown stages of SJIA-MAS compared to pre-MAS stage. Significantly increased ferritin and ratio of ferritin/ESR were also observed in patients with SJIA compared to in KD and CTD. Receiver-operating characteristic analysis showed that 12,217.5 μg/L of ferritin and 267.5 of ferritin/ESR ratio had sensitivity (80.0% and 90.5%) and specificity (88.2% and 86.7%), respectively, for predicting full-blown SJIA-MAS. The majority of the patients received corticosteroids (79/80), while biologic agents were used in 12.5% (10/80) of cases. Tocilizumab was the most commonly selected biologic agent. The overall mortality rate was 7.5%. CONCLUSIONS: About half of MAS occurred when the underlying autoimmune diseases (SJIA, KD, and CTD) were first diagnosed. Hypotension could be an important manifestation before MAS diagnosis. Decreased albumin and increased AST, LDH, ferritin, and ratio of ferritin/ESR could predict the onset or full blown of MAS in patient with SJIA.
BACKGROUND: Macrophage activation syndrome (MAS) is a major cause of morbidity and mortality in pediatric rheumatology. We aimed to further understand the clinical features, treatment, and outcome of MAS in China. METHODS: A multi-center cohort study was performed in seven hospitals in China from 2012 to 2018. Eighty patients with MAS were enrolled, including 53 cases with systemic juvenile idiopathic arthritis (SJIA-MAS), 10 cases of Kawasaki disease (KD-MAS), and 17 cases of connective tissue disease (CTD-MAS). The clinical and laboratory data were collected before (pre-), at onset, and during full-blown stages of MAS. We compared the data among the SJIA-MAS, KD-MAS, and CTD-MAS subjects. RESULTS: 51.2% of patients developed MAS when the underlying disease was first diagnosed. In patients with SJIA, 22.6% (12/53) were found to have hypotension before the onset of SJIA-MAS. These patients were also found to have significantly increased aspartate aminotransferase (AST) and lactate dehydrogenase (LDH), as well as decreased albumin (P < 0.05), but no difference in alanine aminotransferase, ferritin, and ratio of ferritin/erythrocyte sedimentation rate (ESR) at onset of MAS when compared to pre-MAS stages of the disease. In addition, ferritin and ratio of ferritin/ESR were significantly elevated in patients at full-blown stages of SJIA-MAS compared to pre-MAS stage. Significantly increased ferritin and ratio of ferritin/ESR were also observed in patients with SJIA compared to in KD and CTD. Receiver-operating characteristic analysis showed that 12,217.5 μg/L of ferritin and 267.5 of ferritin/ESR ratio had sensitivity (80.0% and 90.5%) and specificity (88.2% and 86.7%), respectively, for predicting full-blown SJIA-MAS. The majority of the patients received corticosteroids (79/80), while biologic agents were used in 12.5% (10/80) of cases. Tocilizumab was the most commonly selected biologic agent. The overall mortality rate was 7.5%. CONCLUSIONS: About half of MAS occurred when the underlying autoimmune diseases (SJIA, KD, and CTD) were first diagnosed. Hypotension could be an important manifestation before MAS diagnosis. Decreased albumin and increased AST, LDH, ferritin, and ratio of ferritin/ESR could predict the onset or full blown of MAS in patient with SJIA.
Authors: Mark Gorelik; Ndate Fall; Mekibib Altaye; Michael G Barnes; Susan D Thompson; Alexei A Grom; Raphael Hirsch Journal: J Rheumatol Date: 2013-05-15 Impact factor: 4.666