Objectives: Adult-onset Still's disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. It is characterised by fever, arthritis and, a typical salmon-coloured rash, and is accompanied by fever at nights. Currently, there is limited data on the prevalence of AOSD.
Methods: Patients diagnosed with AOSD at the Department of Rheumatology of Trakya University Medical Faculty, between 2003 to 2014 were reviewed retrospectively. Patients' clinical features, laboratory measurements, demographics, treatments, follow-up durations, disease courses, outcomes and complications were evaluated.
Results: Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. The most common findings were fever (97.6%), arthralgia (95.2%), arthritis (76.2%), rash (73.8%) and sore throat (40.5%).
Conclusions: In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.
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Sci Rep
December 2024
International Collaboration On Repair Discoveries, School of Biomedical Engineering, University of British Columbia, Vancouver, BC, Canada.
Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset Still's disease (AOSD), and COVID-19 cytokine storm (CCS) are characterized by markedly elevated inflammatory cytokines. However clinical measurement of serum cytokines is not widely available. This study examined the clinical utility of C-reactive protein (CRP) and ferritin, two inexpensive and widely available inflammatory markers, for distinguishing HLH from AOSD and CCS.
View Article and Find Full Text PDFAustralas J Dermatol
December 2024
Department of Dermatology, St Vincent's Hospital Melbourne, Fitzroy, Victoria, Australia.
Adult-onset Still's disease (AOSD) is a rare autoinflammatory systemic disorder classically characterised by inflammatory polyarthritis, daily fevers and a transient asymptomatic salmon-pink maculopapular rash that typically arises with the onset of fevers. We report a case of AOSD presenting with a severely pruritic urticarial eruption starting 6 weeks prior to the onset of fever and arthritis and complicated by macrophage activation syndrome. This case highlights the importance of early recognition of diverse cutaneous manifestations of AOSD to facilitate timely diagnosis and treatment to improve disease outcomes.
View Article and Find Full Text PDFCureus
November 2024
Diabetes and Endocrinology, United Lincolnshire Hospitals NHS Trust, Boston, GBR.
Adult-onset Still's disease (AOSD) is an uncommon systemic inflammatory disorder that presents with diverse, overlapping symptoms, complicating the diagnostic process due to its nonspecific clinical features and the absence of a definitive diagnostic test. Diagnosis is often challenging and relies on excluding other conditions while maintaining a high index of suspicion, supported by specific diagnostic criteria such as Yamaguchi or Fautrel. Prompt recognition and a multidisciplinary approach are essential, as AOSD can progress to life-threatening multiorgan dysfunction due to a hyperinflammatory response.
View Article and Find Full Text PDFProc (Bayl Univ Med Cent)
July 2024
Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated.
View Article and Find Full Text PDFJ Rheum Dis
January 2025
Division of Rheumatology, Department of Internal Medicine, Korea University Medical Center, Korea University College of Medicine, Seoul, Korea.
Objective: This study aimed to investigate the link between circulating interleukin-18 (IL-18) levels and adult-onset Still's disease (AOSD).
Methods: A thorough search was performed on MEDLINE, Embase, and Web of Science to find relevant articles. A meta-analysis was conducted to compare serum/plasma IL-18 levels in AOSD patients to those in control subjects.
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