Adult-onset still’s disease: a case report and a comprehensive review
DOI:
https://doi.org/10.18203/2320-6012.ijrms20243738Keywords:
AOSD, Arthralgia, Fever, SteroidsAbstract
Adult-onset still disease (AOSD) is a rare inflammatory disorder affecting young adults, especially women, characterized by fever, arthralgia, skin rash, hyperleukocytosis and increased ferritin levels. It can be monocyclic, polycyclic, or chronic, with chronic cases primarily involving the joints. Ferritin, a protein that stores iron in the body, can be significantly elevated in AOSD due to systemic inflammation and the body’s response to it. Treatment typically includes corticosteroids, methotrexate, and biological therapy. Recent treatments like anakinra, tocilizumab, and TNFα-blockers have shown promise. This case report details a 26-year-old woman with a one-month history of fever, arthralgia, and weight loss. She was diagnosed with a new onset AOSD, based on elevated ferritin, liver enzymes, IL-6, and IL-18 levels. Her symptoms improved with systemic corticosteroid therapy, and she was discharged with oral steroids, NSAIDS and regular follow-ups.
Metrics
References
Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult-onset Still's disease. J Autoimmunity. 2018;93:24-36.
Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still's disease. Nature Rev Rheumatol. 2018;14(10):603-18.
Jamilloux Y, Gerfaud-Valentin M, Martinon F, Belot A, Henry T, Sève P. Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart. Immunologic Res. 2015;61(1-2):53-62.
Mert A, Ozaras R, Tabak F, Bilir M, Ozturk R, Ozdogan H, et al. Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. Clin Rheumatol. 2003;22(2):89-93.
Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still’s disease. Semin Arthritis Rheum. 2006;36:144-52.
Mitrovic S, Feist E, Fautrel B. Adult-Onset Still’s Disease. In: Cimaz, R. (eds) Periodic and Non-Periodic Fevers. Rare Diseases of the Immune System. Springer, Cham. 2020.
Gerfaud-Valentin M, Maucort-Boulch D, Hot A, Iwaz J, Ninet J, Durieu I, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Med. 2014;93(2):91-9.
Magadur-Joly G, Billaud E, Barrier JH, Pennec YL, Masson C, Renou P, et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis. 1995;54:587-90.
Daghor AK, Berrah A. Severe destructive arthritis in adult-onset Still’s disease. Rheumatol Adv Pract. 2019;3(1):rky052.
Guilpain P, Le Quellec A. About the complexity of adult onset Still’s disease and advances still required for its management. BMC Med. 2017;15:5.
Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheumatic Dis. 2006;65:564-72.
Sfriso P, Priori R, Valesini G, Rossi S, Montecucco CM, D'Ascanio A, et al. Adult-onset Still's disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients. Clin Rheumatol. 2016;35:1683-9.
Mandl LA, O’Dell JR, Case SM. Adult-onset Still’s Disease: clinical manifestations and diagnosis. UpToDate. 2024. Available at: https://www.uptodate.com/contents/adult-onset-stills-disease-clinical-manifestations-and-diagnosis?search=adult%20onset%20still%27s%20disease%20differential&source=search_result&selectedTitle=1%7E47&usage_type=default&display_rank=1#H70215716. Accessed on 12 September 2024.