Pan American League of Associations for Rheumatology treatment recommendations for systemic juvenile idiopathic arthritis

We aimed to develop evidence-based Pan American League of Associations for Rheumatology recommendations for the pharmacological treatment of systemic juvenile idiopathic arthritis (sJIA). An expert panel of paediatric rheumatologists from Latin America generated clinically meaningful research questions structured using the Population, Intervention, Comparator and Outcome (PICO) format, adhering to Grading of Recommendations Assessment, Development, and Evaluation methodology. A team of methodologists conducted a systematic literature review, extracted and summarized intervention effect estimates and graded the evidence quality. The JIA expert panel voted on each research question structured using the PICO format, requiring a minimum agreement of 70% among the voting members to formulate recommendations. Four evidence-based recommendations were developed, addressing the two most common phenotypes of sJIA: with predominantly systemic features and with predominantly active synovitis. The optimal therapeutic approach emphasizes the early initiation of IL-1 or IL-6 pathway inhibition, coupled with a short-course corticosteroid regimen. For sJIA patients with predominantly systemic features and high disease activity, high-dose i.v. methylprednisolone ('pulse therapy') is advised. These recommendations highlight the importance of limiting glucocorticoid therapy to the lowest effective dose for the shortest possible duration, with gradual tapering and discontinuation within a maximum period of 6 months. These recommendations provide guidance on strategies for the use of pharmacological agents for sJIA patients.