Adult Onset Still's Disease - current state of knowledge
DOI:
https://doi.org/10.12775/JEHS.2025.82.60591Keywords
Adult-onset Still's diseaseAbstract
Introduction: Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. AOSD lies at the crossroads of innate and adaptive immunity, highlighting the complexity of its pathogenesis. The heterogeneous clinical expression and the lack of specific diagnostic markers contribute to the difficulties in achieving a rapid and accurate diagnosis, necessitating a careful differentiation to exclude malignancies, infections, and other inflammatory conditions. There is no universally established treatment protocol, as new therapeutic options continue to emerge.
Aim of the study: The study aim is to analyse and summarize current state of knowledge about diagnosis and treatment strategies of Adult Onset Still’s Disease.
Methodology: The PubMed database was searched to find scientific articles published between 2020-2025 with free full text available in which the term "Still’s disease" appears in the title or keywords.
Conclusions: Adult-Onset Still’s Disease is a complex autoinflammatory disorder driven by a dysregulated cytokine storm, with infections and genetic predispositions implicated as triggers. It presents variably, from systemic inflammation to chronic arthritis, and can lead to severe complications. While traditional treatments like NSAIDs, corticosteroids, and DMARDs have been used, biologic therapies targeting IL-1 and IL-6 have significantly improved outcomes. Ongoing research aims to refine diagnostics, identify biomarkers, and develop personalized treatments.
References
1. Maranini, B., Ciancio, G., & Govoni, M. (2021). Adult-Onset Still’s Disease: novel biomarkers of specific subsets, disease activity, and relapsing forms. International Journal of Molecular Sciences, 22(24), 13320. https://doi.org/10.3390/ijms222413320
2. Current and emerging biological therapy in adult-onset Still’s disease
Ma, Y., Meng, J., Jia, J., Wang, M., Teng, J., Zhu, D., Yang, C., & Hu, Q. (2021). Current and emerging biological therapy in adult-onset Still’s disease. Lara D. Veeken, 60(9), 3986–4000. https://doi.org/10.1093/rheumatology/keab485
3. Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as Part of the Still’s Disease Continuum
Kontzias, A., Petryna, O., Nakasato, P., & Efthimiou, P. (2024). Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as part of the Still’s Disease Continuum. Mediterranean Journal of Rheumatology, 35(Suppl 1), 45. https://doi.org/10.31138/mjr.290323.dat
4. 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
5. Mitrovic, S., & Fautrel, B. (2021). Clinical Phenotypes of Adult-Onset Still’s Disease: New Insights from Pathophysiology and Literature Findings. Journal of Clinical Medicine, 10(12), 2633. https://doi.org/10.3390/jcm10122633
6. Kim, J., Jung, J., Suh, C., & Kim, H. (2022). Flare of adult-onset Still’s disease following mRNA COVID-19 vaccination: a case report and review of literature. Clinical Rheumatology, 41(5), 1583–1589. https://doi.org/10.1007/s10067-022-06106-1
7. Matsuda, M., Asanuma, Y. F., Yokota, K., Sakai, S., Yazawa, H., Maruyama, T., Wada, T. T., Araki, Y., & Mimura, T. (2022). New-onset Adult-onset Still’s Disease Following COVID-19 vaccination: Three case reports and a literature review. Internal Medicine, 62(2), 299–305. https://doi.org/10.2169/internalmedicine.0590-22
8. Achour, T. B., Elhaj, W. B., Jridi, M., Naceur, I., Smiti, M., Ghorbel, I. B., Lamloum, M., Said, F., & Houman, M. H. (2023). Adult‐onset Still’s disease after SARS‐Cov‐2 infection. Clinical Case Reports, 11(3). https://doi.org/10.1002/ccr3.7006
9. Jia, J., Shi, H., Liu, M., Liu, T., Gu, J., Wan, L., Teng, J., Liu, H., Cheng, X., Ye, J., Su, Y., Sun, Y., Gong, W., Yang, C., & Hu, Q. (2019). Cytomegalovirus infection may trigger Adult-Onset Still’s disease onset or relapses. Frontiers in Immunology, 10. https://doi.org/10.3389/fimmu.2019.00898
10. 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
11. Rao, S., Tsang, L. S., Zhao, M., Shi, W., & Lu, Q. (2022). Adult-onset Still’s disease: A disease at the crossroad of innate immunity and autoimmunity. Frontiers in Medicine, 9. https://doi.org/10.3389/fmed.2022.881431
12. 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
13. Tomaras, S., Goetzke, C. C., Kallinich, T., & Feist, E. (2021). Adult-Onset Still’s Disease: Clinical aspects and therapeutic approach. Journal of Clinical Medicine, 10(4), 733. https://doi.org/10.3390/jcm10040733
14. 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
15. 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., . . . 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
16. Çolak, S., Tekgöz, E., Mammadov, M., Çınar, M., & Yılmaz, S. (2021). Biological treatment in resistant adult-onset Still’s disease: A single-center, retrospective cohort study. Archives of Rheumatology, 37(1), 11–18. https://doi.org/10.46497/archrheumatol.2022.8669
17. Galozzi, P., Bindoli, S., Doria, A., & Sfriso, P. (2022). Progress in Biological therapies for Adult-Onset Still’s Disease. Biologics, Volume 16, 21–34. https://doi.org/10.2147/btt.s290329
18. Mzabi, A., Fakhfakh, R., Thabet, M., Hassine, I. B., Anoun, J., Karmani, M., Fredj, F. B., & Laouani, C. (2021). Elderly-onset adult Still’s disease. Reumatologia/Rheumatology, 59(3), 188–196. https://doi.org/10.5114/reum.2021.107323
19. Saito, K., Temmoku, J., Sumichika, Y., Yoshida, S., Takano, E., Watanabe, S., Matsumoto, H., Fujita, Y., Matsuoka, N., Asano, T., Sato, S., Watanabe, H., & Migita, K. (2023). Adult-onset Still’s Disease with Acute Kidney Injury Requiring Hemodialysis: A Case Report and Literature Review. Internal Medicine, 62(19), 2901–2906. https://doi.org/10.2169/internalmedicine.1026-22
20. Orentas, M., Patel, N., Rodby, R., & Hassan, S. (2022). The association between adult-onset Still’s disease and collapsing glomerulopathy: a case report. Journal of Medical Case Reports, 16(1). https://doi.org/10.1186/s13256-022-03606-1
21. Kato, R., Ikeuchi, T., Tomita, K., & Yamasaki, A. (2020). Adult-onset Still’s disease with concurrent thrombotic thrombocytopenic purpura: case report and literature review. BMJ Case Reports, 13(9), e235786. https://doi.org/10.1136/bcr-2020-235786
22. Oka, K., Yuya, Y., Yasuda, M., & Otsuka, F. (2020). Adult‐onset Still’s disease accompanying noninfective endocarditis. Journal of General and Family Medicine, 21(6), 268–269. https://doi.org/10.1002/jgf2.339
23. Farina, N., Arroyo-Sánchez, D., Campochiaro, C., Dagna, L., & Tomelleri, A. (2023). Adult-onset Still’s disease complicated by Guillain-Barré syndrome. Lara D. Veeken, 62(10), e295–e296. https://doi.org/10.1093/rheumatology/kead125
24. Milne, I., Kanwar, R., Martin, W., Egert, D., Leisgang, A., Albano-Aluquin, S. A., Henao, M. P., Kreider, C., & Ssentongo, P. (2024). Adult-onset Still’s disease masquerading as acute coronary syndrome: a case report and review of the literature. Journal of Medical Case Reports, 18(1). https://doi.org/10.1186/s13256-024-04799-3
25. Huang, Z., Xu, H., Min, Q., Li, Z., Bi, J., Liu, L., & Liang, Y. (2021b). Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review. Diagnostic Pathology, 16(1). https://doi.org/10.1186/s13000-021-01159-3
26. Kedor, C., Tomaras, S., Baeumer, D., & Feist, E. (2021). Update on the therapy of adult-onset Still’s disease with a focus on IL-1-inhibition: a systematic review. Therapeutic Advances in Musculoskeletal Disease, 13. https://doi.org/10.1177/1759720x211059598
27. Fautrel, B., Patterson, J., Bowe, C., Arber, M., Glanville, J., Mealing, S., Canon-Garcia, V., Fagerhed, L., Rabijns, H., & Giacomelli, R. (2022). Systematic review on the use of biologics in adult-onset still’s disease. Seminars in Arthritis and Rheumatism, 58, 152139. https://doi.org/10.1016/j.semarthrit.2022.152139
Leavis, H. L., Van Daele, P. L. A., Mulders-Manders, C., Michels, R., Rutgers, A., Legger, E., Bijl, M., Hak, E. A., Lam-Tse, W., Bonte-Mineur, F., Fretter, P., Simon, A., Van Paassen, P., Van Der Goes, M. C., Flendrie, M., Vercoutere, W., Van Lieshout, A. W. T., Leek, A., Vastert, S. J., & Tas, S. W. (2023). Management of adult-onset Still’s disease: evidence- and consensus-based recommendations by experts. Lara D. Veeken, 63(6), 1656–1663. https://doi.org/10.1093/rheumatology/kead461
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Anna Centkowska, Natalia Katarzyna Wagner-Bieleń, Martyna Zwierzchowska, Agata Antoniak, Anna Maria Jocz, Joanna Karina Banaśkiewicz, Aleksandra Maria Żyta, Gabriela Helena Dąbrowska, Maria Anna Żmijewska, Jakub Ziółkowski
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
The periodical offers access to content in the Open Access system under the Creative Commons Attribution-NonCommercial-ShareAlike 4.0
Stats
Number of views and downloads: 14
Number of citations: 0
