Consensus-based points to consider have been developed by the European Alliance of Associations for Rheumatology (EULAR) to support the creation of robust, standardised criteria for assessing disease activity in adult-onset Still’s disease, addressing a longstanding unmet need in clinical care and research.
A multinational EULAR task force comprised 23 experts from seven European countries and included rheumatologists, paediatric rheumatologists, methodologists, healthcare professionals and patient representatives.
They set out to formulate points to consider for guiding the development of criteria for assessing disease activity in adult-onset Still’s disease – a rare systemic inflammatory disorder characterised by heterogeneous clinical manifestations and disease courses.
The creation of the points to consider was underpinned by a systematic literature review, followed by a three-round Delphi exercise. Rather than enrolling a patient cohort, the study synthesised evidence from published studies and expert consensus to identify clinical, laboratory and patient-reported domains relevant to disease activity assessment in adult-onset Still’s disease.
A clear definition of adult-onset Still’s disease activity
The task force unanimously agreed on three overarching principles and developed 11 points to consider, which were published in the journal EULAR Rheumatology Open.
Key findings highlighted the importance of fever as the most frequent clinical sign of active disease, alongside evanescent skin rash, arthralgia or arthritis, systemic organ involvement such as lymphadenopathy or serositis. Laboratory abnormalities including hyperferritinaemia, elevated C-reactive protein and erythrocyte sedimentation rate, raised liver enzymes, and neutrophil-predominant leucocytosis were also noted.
Patient global assessment using a visual analogue scale was identified as the only patient-reported outcome currently suitable for inclusion.
The group also emphasised the urgent need for an objective, reproducible definition of remission and the longer-term value of developing disease-specific patient-reported outcome measures.
Professor Roberto Giacomelli, corresponding author and professor of rheumatology at the Bio-Medico Campus University Hospital in Rome, Italy, said: ‘Establishing a reproducible definition of remission could help us move towards a new therapeutic target that we can use both in trials and in the clinic. This will definitively initiate the era of treat-to-target in adult-onset Still’s disease.’
The importance of patient perspectives
Several limitations were acknowledged, including the points to consider being based on expert consensus and heterogeneous published evidence rather than prospective validation cohorts. Some proposed variables may have limited feasibility in routine practice or reduced sensitivity to change, and laboratory markers can be confounded by specific therapies or complications such as macrophage activation syndrome, the authors said.
They concluded that the EULAR points to consider provide a structured foundation for developing and validating a practical and reliable tool to assess disease activity in adult-onset Still’s disease that can be easily applied in clinical settings to improve patient management.
Indeed ‘the development of a clear definition of disease activity may permit the clinician to distinguish those patients requiring a more aggressive treatment from others, identifying active and refractory patients,’ the authors said.
These new points to consider should be read in conjunction with EULAR’s 2024 recommendations on the diagnosis and management of both children and adults with Still’s disease, they added.
The task force said future research should focus on prospective, adequately powered studies to derive and validate composite criteria, the refinement of remission definitions and the incorporation of patient perspectives, with potential implications for treat-to-target strategies, clinical trials and improved standardisation of care.
Reference
Ruscitti P et al. The EULAR points to consider regarding the development of criteria for the assessment of the disease activity in adult-onset Still’s disease. ERO 2025.