Performance of Recent PRINTO Criteria Versus Current ILAR Criteria for Systemic Juvenile Idiopathic Arthritis: A Single-Center Experience
Abstract Objectives The purpose of this study is to evaluate the performances of recently proposed Pediatric Rheumatology International Trials Organization (PRINTO) criteria versus current International League of Associations for Rheumatology (ILAR) criteria, for systemic juvenile idiopathic arthritis (sJIA). Methods The study was performed at the Department of Pediatric Rheumatology in Istanbul Faculty of Medicine with a retrospective design, covering the date range 2010 to 2021. Patients being followed-up with a diagnosis of sJIA, Kawasaki disease (control group-1), and common autoinflammatory diseases (control group-2) were included in the study. Both the ILAR and PRINTO classification criteria were applied to each patient and compared against expert rheumatologist diagnosis. Results Eighty-two patients with a diagnosis of sJIA compared against 189 [74 Kawasaki disease, 83 familial Mediterranean fever (FMF), 16 mevalonate kinase deficiency (MKD), 10 cryopyrin-associated periodic syndromes (CAPS), 6 tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS)] patients. The PRINTO criteria demonstrated higher sensitivity (62.2% vs 80.5%, p=0.003), but comparable specificity (90.5% vs 91%) as regards the ILAR criteria. Conclusions The revised criteria appear to enhance the ability to provide early recognition and pertinent classification of sJIA. The two criteria were not superior to each other in segregating sJIA from common autoinflammatory diseases and Kawasaki disease, namely in terms of specificity.