Preprint / Version 1

Fever of unknown origin and marked hyperferritinemia in a young patient: a diagnostic and therapeutic challenge.

Authors

  • Maritza Daniela Diaz Carlotti Español
  • Manuel Cifrian Perez Español

DOI:

https://doi.org/10.62059/0mchs664

Keywords:

Adult-onset Still’s disease, Ferritin, Anakinra, Yamaguchi criteria, Autoinflammatory diseases

Abstract

Adult-onset Still's disease (AOSD) is a rare autoinflammatory disorder characterized by fever, rash, and arthralgia, constituting an uncommon cause of fever of unknown origin. Its diagnosis remains primarily one of exclusion. Case report: A 27-year-old woman presented with fever up to 40°C, odynophagia, and a transient salmon-colored rash. Laboratory findings were notable for neutrophilic leukocytosis, marked elevation of acute-phase reactants, and hyperferritinemia of 2800 ng/ml. After ruling out infectious, neoplastic, and autoimmune processes, the diagnosis of AOSSD was established. The patient responded rapidly to glucocorticoids and biologic treatment with anakinra, achieving clinical and laboratory remission. Conclusion: Marked hyperferritinemia and adherence to clinical criteria remain key diagnostic indicators. Early initiation of IL-1 inhibitors is currently a priority therapeutic strategy according to the EULAR/PReS 2024 recommendations.

References

1. Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P. Adult-onset Still’s disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum. 2021;51(4):858-74.

2. Fautrel B, Mitrovic S, De Matteis A, et al. EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease. Ann Rheum Dis. 2024. doi:10.1136/ard-2024-225851.

3. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19(3):424-30.

4. Tomaras S, Goetzke CC, Kallinich T, Feist E. Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach. J Clin Med. 2021;10(4):733.

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Status: Under review in Revista Argentina de Medicina

RAM is a quarterly publication edited and financed by the Argentine Society of Medicine aimed at disseminating biomedical, epidemiological and social scientific topics related to the following branches of clinical medicine: bioethics, cardiology, critical care, palliative care, dermatology, pain, education, emergentology, endocrinology, epidemiology, pharmacology and…

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Posted

2026-08-28