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Still's disease continuum from childhood to elderly: data from the international AIDA Network Still's disease
Antonio Vitale1,2, Valeria Caggiano3,2, Giuseppe Lopalco4
1Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy cantariniluca@hotmail.com vitale.reumatologia@gmail.com.
Insights
Still's disease presents similarly across pediatric, adult, and elderly onset, suggesting a unified clinical condition despite minor age-related variations in symptoms and lab findings. Treatment responses remain consistent across all age groups.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Autoammatory Diseases
Background:
- Still's disease, a rare autoinflammatory condition, is typically diagnosed in children but can manifest in adults and the elderly.
- The clinical spectrum and distinct entities of pediatric-onset, adult-onset, and elderly-onset Still's disease remain debated.
Purpose of the Study:
- To investigate demographic, clinical, and treatment response differences in Still's disease across pediatric, adult, and elderly onset groups.
- To determine if these age-related presentations represent a continuum of the same disease or distinct clinical entities.
Main Methods:
- Retrospective analysis of 411 patients with Still's disease from the International AutoInflammatory Disease Alliance Network registry.
- Comparison of demographic, clinical features (rash, arthritis, abdominal pain, pleuritis, arthralgia), laboratory data (thrombocytosis, thrombocytopenia), and treatment responses between pediatric, adult, and elderly onset groups.
Main Results:
- No significant demographic differences were found between the onset groups.
- Pediatric-onset Still's disease showed higher frequencies of salmon-colored rash, arthritis, abdominal pain, and thrombocytosis compared to adult-onset.
- Elderly-onset patients exhibited more frequent pleuritis and arthralgia than pediatric-onset patients, with a trend towards thrombocytopenia.
Conclusions:
- Overall, demographic, clinical, laboratory, and treatment aspects of Still's disease are similar across all age groups.
- Minor differences in specific symptoms and laboratory findings exist between pediatric, adult, and elderly onset.
- The findings support the classification of pediatric-onset, adult-onset, and elderly-onset Still's disease as a single clinical condition presenting at different ages.
Objective:
Still's disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still's disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still's disease.
Methods:
Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still's disease.
Results:
A total of 411 patients suffering from Still's disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still's disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p<0.0001) were significantly more frequent among elderly-onset patients compared with paediatric-onset subjects. Regarding laboratory data, thrombocytosis was significantly more frequent among paediatric patients onset compared with adult-onset subjects (p<0.0001), while thrombocytopenia was more frequent among elderly-onset patients although statistical significance was only bordered. No substantial differences were observed in the response to treatments.
Conclusions:
Despite some minor difference between groups, overall, demographic, clinical, laboratory and treatments aspects of Still's disease were similarly observed in patients at all ages. This supports that pediatric-onset, adult-onset and elderly-onset Still's disease is the same clinical condition arising in different ages.
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