New Unified Approach to Still’s Disease Unveiled by EULAR and PReS

11/07/2024
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The European Alliance of Associations for Rheumatology (EULAR), in partnership with the Pediatric Rheumatology European Society (PReS), has introduced new recommendations to unify the diagnosis and management of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) under the single designation of Still’s disease. Previously, these conditions were classified separately based on whether symptoms appeared before or after the age of 16. This unified approach aims to improve consistency in diagnosis and access to treatment for patients of all ages.

Historically, the division between sJIA and AOSD has led to differences in treatment availability. Advanced therapies that have been approved for sJIA over the past few years have not been accessible to adults with AOSD, primarily due to the rarity of the adult-onset form, which has complicated efforts to conduct large-scale clinical trials. As a result, individuals diagnosed with sJIA have had greater access to effective therapies, while adults with the same disease have faced more limited options. The new EULAR and PReS recommendations seek to address this disparity by establishing standardized guidance for managing Still’s disease across the lifespan.

Developed through an extensive literature review and collaboration among researchers, healthcare professionals, and patient representatives, the guidelines include 14 specific recommendations and four overarching principles for diagnosis and treatment. The first principle emphasizes the recommendation to treat sJIA and AOSD as a single disease and to refer to both as Still’s disease. By adopting this universal name, EULAR and PReS aim to streamline clinical approaches and foster a unified perspective in treating this condition.

Additional principles highlight the importance of a “treat-to-target” approach, aiming for remission through shared decision-making between patients (or their caregivers) and the healthcare team. This strategy, widely used in other rheumatological conditions, is intended to individualize care and optimize outcomes, with drug-free remission as the ultimate goal. The guidelines also underscore the need for vigilance regarding macrophage activation syndrome (MAS), a potentially life-threatening complication of Still’s disease, recommending prompt detection and treatment.

EULAR and PReS anticipate that these standardized recommendations will help improve outcomes for all patients diagnosed with Still’s disease, ensuring that effective treatment strategies are not restricted by age. This consensus represents an important step in establishing consistent, evidence-based care for this complex condition.

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