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The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset still's disease, and COVID-19 cytokine storm.
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 PDFPersistent urticarial eruption preceding systemic features of adult-onset Still's disease: A case report.
Australas 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 PDFDiagnostic Challenges and Therapeutic Considerations in Adult-Onset Still's Disease: A Clinical Case Report.
Cureus
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 PDFBreaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.
Proc (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 PDFAssociations between circulating interleukin-18 levels and adult-onset Still's disease: a meta-analysis.
J 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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