Adult-onset Still's disease
Overview
Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease characterized by recurrent high fevers, inflammatory arthritis, a distinctive salmon-colored rash, and elevated inflammatory markers. Although its exact cause remains unknown, current research recognizes AOSD and systemic juvenile idiopathic arthritis (systemic JIA) as age-specific forms of the same disease spectrum, collectively referred to as Still’s disease.
Unlike most forms of arthritis, AOSD affects the entire body and may involve the liver, spleen, lymph nodes, heart, lungs, and other organs. Disease severity varies considerably, with some people experiencing a single episode while others develop chronic or recurrent disease requiring long-term treatment.
Symptoms
High recurring fevers; joint pain (arthralgia); arthritis; salmon-colored rash; muscle pain (myalgia); sore throat; fatigue; swollen lymph nodes; enlarged liver or spleen; weight loss; chest pain caused by inflammation around the heart or lungs (pericarditis or pleuritis).
Coexisting Diseases and Conditions
Complications include macrophage activation syndrome (MAS); chronic inflammatory arthritis; pericarditis; pleuritis; interstitial lung disease; liver inflammation (hepatitis).
Risk Factors and Prevalence
Adult-onset Still’s disease is a rare disease with an estimated annual incidence of approximately 0.16 to 0.62 cases per 100,000 people worldwide and an estimated prevalence of 0.73 to 6.77 cases per 100,000 people, although more recent studies suggest the disease may be more common than previously recognized due to improved awareness and diagnosis. It affects females slightly more often than males and most commonly begins between 16 and 35 years of age, although it can occur at any age during adulthood, including in older adults. The exact cause remains unknown, but current evidence suggests that genetic susceptibility combined with environmental triggers, such as infections, may contribute to disease development.
Recent Research
- EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease (2024)
- Systemic juvenile idiopathic arthritis and adult-onset Still’s disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR/PReS recommendations for the diagnosis and management of Still’s disease (2024)
- Adult-Onset Still’s Disease (AOSD): Advances in Understanding Pathophysiology, Genetics and Emerging Treatment Options (2024)
- Adult-Onset Still’s Disease-A Complex Disease, a Challenging Treatment (2022)
Sources
- Sources
Fautrel, B., Mitrovic, S., De Matteis, A., Bindoli, S., Antón, J., Belot, A., Bracaglia, C., Constantin, T., Dagna, L., Di Bartolo, A., Feist, E., Foell, D., Gattorno, M., Georgin-Lavialle, S., Giacomelli, R., Grom, A. A., Jamilloux, Y., Laskari, K., Lazar, C., Minoia, F., … De Benedetti, F. (2024). EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease. Annals of the rheumatic diseases, 83(12), 1614–1627. https://doi.org/10.1136/ard-2024-225851
Liu Z, et al. Clinical features and prognosis of adult-onset Still’s disease: 75 cases from China. International Journal of Clinical and Experimental Medicine. 2015;8:16634
Bindoli, S., Baggio, C., Doria, A., & Sfriso, P. (2024). Adult-Onset Still’s Disease (AOSD): Advances in Understanding Pathophysiology, Genetics and Emerging Treatment Options. Drugs, 84(3), 257–274. https://doi.org/10.1007/s40265-024-01993-x
Efthimiou, P., Kontzias, A., Hur, P., Rodha, K., Ramakrishna, G. S., & Nakasato, P. (2021). Adult-onset Still’s disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Seminars in arthritis and rheumatism, 51(4), 858–874. https://doi.org/10.1016/j.semarthrit.2021.06.004
Feist, E., Mitrovic, S., & Fautrel, B. (2018). Mechanisms, biomarkers and targets for adult-onset Still’s disease. Nature reviews. Rheumatology, 14(10), 603–618. https://doi.org/10.1038/s41584-018-0081-x
Macovei, L. A., Burlui, A., Bratoiu, I., Rezus, C., Cardoneanu, A., Richter, P., Szalontay, A., & Rezus, E. (2022). Adult-Onset Still’s Disease-A Complex Disease, a Challenging Treatment. International journal of molecular sciences, 23(21), 12810. https://doi.org/10.3390/ijms232112810
De Matteis, A., Bindoli, S., De Benedetti, F., Carmona, L., Fautrel, B., & Mitrovic, S. (2024). Systemic juvenile idiopathic arthritis and adult-onset Still’s disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR/PReS recommendations for the diagnosis and management of Still’s disease. Annals of the rheumatic diseases, 83(12), 1748–1761. https://doi.org/10.1136/ard-2024-225853