Disruption of vascular endothelial homeostasis in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The dynamic roles of angiopoietin-1 and -2 |
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| Institution: | 1. Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical, and Health Sciences, Kanazawa University, Japan;2. Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, Japan;1. Associate Professor, Department of Orthopedic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea;2. Orthopedic Resident, Department of Orthopedic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea;1. Rheumatology Unit, Department of Internal Medicine, Azienda Ospedaliera-IRCCS di Reggio Emilia, Reggio Emilia, Italy;2. Operative Unit of Pathologic Anatomy, Department of Oncology and Advanced Technology, Arcispedale Santa Maria Nuova, IRCCS, Reggio Emilia, Italy;3. Ophthalmology Unit, Department of Surgery, Arcispedale Santa Maria Nuova, IRCCS, Reggio Emilia, Italy;4. Department of Clinical and Experimental Medicine, University of Parma, Parma, Italy;1. Department of Drug Safety Sciences, Division of Clinical Pharmacology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan;2. Department of Molecular and Environmental Pathology, Institute of Health Biosciences, Tokushima University Graduate School, Tokushima 770-8503, Japan;1. Department of Biological Sciences, College of Natural Sciences, Kongju National University, 182 Shinkwan-Dong, Gongju, Chungnam 314-701, Republic of Korea;2. Department of Biochemistry and Molecular Biology, Research Institute of Oral Sciences, College of Dentistry, Gangnung-Wonju National University, Gangneung, Republic of Korea;3. Department of Biomedical Science and Research Institute of Bioscience and Biotechnology, Hallym University, Chunchon, Republic of Korea;4. Division of rheumatology, Department of Internal Medicine, Hallym University Sacred Heart Hospital, Kyunggi, Republic of Korea;5. 2nd Affiliate: Institute for Skeletal Aging, Hallym University, Chunchun, Republic of Korea;1. Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale Bracci, 16, 53100 Siena, Italy;2. Department of Medical Biotechnologies, University of Siena, 53100 Siena, Italy;1. Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University, Tokyo, Japan;2. Department of Pediatrics, Kawaguchi Municipal Medical Center, Saitama, Japan;3. Department of Pediatrics, Tokyo-Kita Medical Center, Tokyo, Japan;4. Department of Pediatrics, Soka Municipal Hospital, Saitama, Japan;5. Frontier Science Laboratory Virus Research Unit, Tokyo Medical and Dental University, Tokyo, Japan |
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| Abstract: | To assess the role of angiopoietin (Ang)-1 and Ang-2 and to investigate the clinical significance of serum levels of them in systemic juvenile idiopathic arthritis (s-JIA)-associated macrophage activation syndrome (MAS), we determined these levels in 51 patients with s-JIA, 11 patients with polyarticular JIA (poly-JIA), 12 patients with virus associated hemophagocytic syndrome (VAHS), 12 patients with Kawasaki disease (KD), and 15 age-matched healthy controls (HC). The results were compared with clinical features of MAS. During the MAS phase, serum Ang-1 levels were significantly decreased compared with those during the active and inactive phases. Serum Ang-2/1 ratio were significantly elevated during the MAS phase, compared with those during the active and inactive phases. There was a rapid increase in the Ang-2/1 ratio at the onset of MAS. Serum Ang-1 and the Ang-2/1 ratio significantly correlated with measures of disease activity, including AST and LDH. Ang-2/1 dysregulation was also observed in patients with VAHS, whereas not observed in most cases of KD. The homeostasis of vascular endothelial function by Ang-1 and Ang-2 is disrupted in MAS. Serum Ang-1 levels and the Ang-2/1 ratio might represent promising indicators of disease activity for MAS. |
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| Keywords: | Systemic juvenile idiopathic arthritis Macrophage activation syndrome Angiopoietin 1 Angiopoietin 2 Endothelial function |
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