FRI0535 COMPARISON OF SERUM BIOMARKERS FOR THE DIAGNOSIS OF MACROPHAGE ACTIVATION SYNDROME COMPLICATING SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS: CLINICAL SIGNIFICANCE OF SERUM NEOPTERIN LEVELS. (June 2019)

• Record Type: Journal Article
• Authors: Irabu, Hitoshi; Shimizu, Masaki; Takakuwa, Maiko; Sakumura, Naoto; Inoue, Natsumi; Mizuta, Mao; Nakagishi, Yasuo; Yachie, Akihiro
• Is Part Of: Annals of the rheumatic diseases. Volume 78(2019)Supplement 2
• Page Start: 961
• Page End: 962
• Publication Date: 2019-06
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2019-eular.5537
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Macrophage activation syndrome (MAS) is a severe life-threatening condition that complicates systemic juvenile idiopathic arthritis (s-JIA). Differentiating MAS from these conditions is essential for selecting appropriate therapeutic interventions in a timely manner. However, there is no definite biomarkers that can effectively diagnose MAS. Objectives: Our study aimed to compare the accuracy of serum biomarkers for the diagnosis of MAS complicating s-JIA and to investigate the clinical significance of serum neopterin levels as an indicator of disease activity and diagnosis of MAS complicating s-JIA. Methods: Serum cytokine levels (neopterin, IL-18, and CXCL9 and soluble tumor necrosis factor receptor type I (sTNFR-I) and II were determined by enzyme-linked immunosorbent assay in 78 patients with s-JIA, including 21 with MAS. The accuracy of these levels for the diagnosis of MAS were compared. Next, serum neopterin levels, in total 125 patients with s-JIA, including 30 with MAS, 15 with Epstein–Barr virus-induced hemophagocytic lymphohistiocytosis (EBV-HLH), and 15 with Kawasaki disease (KD), as well as 28 healthy controls (HCs) were analysed. Results were compared with the clinical features of MAS. Results: Receiver operating characteristic curve analysis revealed area under the curve values and cut off values of neopterin, IL-18, CXCL9, sTNFR-II/I ratio and ferritin were 0.9465/19.5nmol/l, 0.8895/69250ng/ml, 0.9333/3130pg/ml, 0.9395/3.796 and 0.8671/2560ng/ml, respectively. Serum neopterin levels were significantly elevated in patients with MAS and EBV-HLH compared with those in patients with acute-phase s-JIA and KD. Serum neopterin levels profoundly and rapidly increased as MAS developed and correlated positively with disease activity. Conclusion: Serum neopterin levels may be used as a promising indicator of disease activity in s-JIA and MAS and for evaluating it. It may also be a useful marker to diagnose the transition to MAS from active-phase s-JIA. References: [1] Huber C, et al. J Exp Med1984;160:310-6. [2] Bracaglia C, et al. Ann Rheum Dis2017;76:166-72. [3] Shimizu M, et al. Clin Exp Immunol2018;191:349-355. Disclosure of Interests: None declared