Systemic JIA is distinguished from all other categories of juvenile idiopathic arthritis by fever, rash, and visceral involvement, and is considered by some to be an autoinflammatory disorder. This protocol covers first-line management in patients younger than 18 years who present without macrophage activation syndrome (MAS).
Systemic juvenile idiopathic arthritis in a patient under 18 years of age, without macrophage activation syndrome. The absence of MAS is a defining criterion for applicability of this initial treatment pathway.
The primary objective is resolution of systemic features, including fever and rash. Because a response that is not rapid and complete warrants prompt escalation, early treatment response is closely monitored.
Initial therapy in this setting may involve either a brief trial of an NSAID or monotherapy with a biologic agent — there is no single preferred agent across all patients, and the complete selection framework, escalation criteria, and sequencing are detailed in the full protocol.