AB1114 Systemic juvenile idiopathic arthritis: a single centre experience. (12th June 2018)

• Record Type: Journal Article
• Authors: Barut, K.; Tarcın, G.; Tahaoglu, G.; Sahin, S.; Adrovic, A.; Kasapcopur, O.
• Is Part Of: Annals of the rheumatic diseases. Volume 77(2018)Supplement 2
• Page Start: 1664
• Page End: 1664
• Publication Date: 2018-06-12
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-eular.3594
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood, divided into several subgroups. The sJIA could be presented by monocyclic, polycyclic or persistent polyarticular clinical course. Macrophage activation syndrome (MAS) represents the most devastating complication that could appear during the disease course. Studies on follow up, treatment response and disease complications of the sJIA patients are spare and rare. Objectives: To evaluate demographic and clinical characteristics and to explore the long-term treatment response and disease complications in a large cohort of sJIA patients from the single centre. Methods: Demographic and clinical features of the sJIA patients were reached from the patients's archives. The frequency of disease flares, treatment response and side effects were recorded for each patient. Results: A total of 168 sJIA patients were included in the study: 87 (51.8) female, 81 (48.2) male. The clinical features are shown in table 1. Fifty-three (31.5) patients had monocyclic while 23 (13.7) patients had polycyclic clinical course (mean recurrency of attacks 2.5±2 (IQR:1–4)): in 38(. 42 Poliarticular course was present in 92 (54.8) patients. Initially diagnosis of patients were: infection in 86 (51.1), sJIA in 34 (20.4), acute rheumatic fever in 19 (11.3), urticaria in 10 (5.9), Kawasaki disease in 4 (2.4) and juvenile systemic lupus erythematosus in 2 patients. The most common disease complications were: MAS in 20 (11.9), growth retardation in 19 (11.3) and vertebral fracture due to osteoporosis in 3 (1.9) patients. Gastrointestinal symptoms secondary to methotrexate intolerance that led to cessation of treatment were present in 9 (7.1) patients. Among 5 (2.9) patients that developed tuberculosis, 4 (2.3) were under etanercept treatment. All of the patients were treated with corticosteroids: a doses of 2 mg/kg/day in 118 (70.2) patients and pulse steroids in 50 (29.8) patients with severe clinical presentation. The methotrexate was used in 126 (75), leflunomide in 5 (3), cyclosporine A in 29 (17.3), intravenous immunoglobulin in 19 (11.3), anakinra in 27 (16.1), canakinumab in 27 (16.1), tocilizumab in 18 (10.7), etanercept in 50 (29.8) and adalimumab in 7 (4.2) patients. The median time to remission after the initial treatment with corticosteroids was 4 (IQR:2–4) months. The remission off medications was achieved in 82 (48.8) while remission on medications was achieved in 83 (49.4) of patients. Conclusions: Systemic JIA is a subtype of JIA characterised by significant morbidity and mortality rate with macrophage activation syndrome being the most severe disease complication. Corticosteroids represent the main treatment modality. Biological agents should be considered in the steroid-resistant patients. The clinical remission could be achieved and chronic arthritis sequelae could be prevented in a majority of patients with biological agents. Reference: [1] Barut K, Adrovic A, Sahin S, Kasapcopur O. Juvenile Idiopathic Arthritis. Balkan medical journal. 2017;34:90–101 Disclosure of Interest: None declared