Unmet Needs in Still’s disease Save

In February 2023, an expert consensus panel of pediatric and adult rheumatologists met to address challenges in Still’s disease (SD), including systemic juvenile idiopathic arthritis (sJIA) and adult-onset SD (AOSD). They addressed four main topics: (1) early recognition and diagnosis of SD; (2) pathogenetic pathways and possible biomarkers for diagnosis and response; (3) refractory disease and risk factors, and (4) treatment of SD and its complications.

They reviewed concerns regarding diagnostic criteria, biomarkers, early diagnosis, SD complications (macrophage activation syndrome and lung disease) and the identification of those at risk for refractory disease. They noted that there are significant differences between adult and pediatric populations and and across centers and countries. 

Below is a summary of expert discussions, concerns and unmet needs in the diagnosis and management of SD.

Specific Unmet Needs

• Lack of awareness of the condition both in pediatric and adult general medicine (Delays in referral)
• Lack of common and validated diagnostic criteria for both pediatric and adult SD
• Pathogenetic mechanisms underlying the disease still not completely understood
• Lack of widely available and validated biomarkers
• Limited knowledge of the role of IL-18
• Lack of a validated definition of refractory disease
• Physicians unaware of differential diagnoses
• Optimal detection and diagnosis of pulmonary involvement - Observed in children but uncommon in adults
• Use of GCs in the diagnostic phase can mask certain disease features and hamper diagnosis
• Optimal dosing and timing of anakinra administration and treatment response are unknown
• Optimal timing of anakinra administration and treatment response are unknown
• A lack of clear guidance regarding timing of discontinuation of biological therapy and GCs
• Approximately 20% of children do not respond to anti–IL-1 or anti–IL-6 therapies
• A lack of guidelines for managing non-responders
• Early initiation of therapy is important for prevention of persistent forms of the disease from becoming refractory; however, the best early treatment strategy is currently unknown
• Difficulty in accessing innovative (experimental) therapies, especially for adults
• Difficulties associated with off-label use of drugs or combinations of biological therapies