Treatment of systemic juvenile idiopathic arthritis

Area of Science:

• Pediatric Rheumatology
• Immunology
• Internal Medicine

Background:

• Systemic juvenile idiopathic arthritis (sJIA) is a severe inflammatory disease in children, sharing features with adult-onset Still disease (AOSD).
• Patients with sJIA face risks of severe complications like macrophage activation syndrome (sJIA-MAS) and lung disease (sJIA-LD).
• Current treatments for sJIA have advanced, including biologics targeting IL-1 and IL-6 pathways.

Purpose of the Study:

• To review current treatment strategies for sJIA, sJIA-MAS, and sJIA-LD.
• To identify and discuss knowledge gaps in managing sJIA, particularly early or refractory cases.
• To provide an outlook on emerging therapies for sJIA.

Main Methods:

• Literature review of contemporary scientific insights and therapeutic approaches for sJIA.
• Analysis of current consensus treatment plans and their limitations.
• Discussion of novel agents and future research directions.

Main Results:

• Biologic agents targeting IL-1 and IL-6 are current standards of care.
• Emerging therapies include JAK inhibitors and biologics targeting IL-18, IFNγ, or IL-1β/IL-18.
• Treatment approaches for early, refractory, or complicated sJIA remain less defined.

Conclusions:

• While sJIA treatments have improved, challenges persist in managing complex cases and complications.
• Further research is needed to define optimal strategies for all sJIA patient subgroups.
• A comprehensive understanding of current and future therapies is crucial for rheumatologists worldwide.