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Published online: 2025-02-25

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AOSD vs. sepsis — diagnosis as a challenge to modern medicine and a nightmare for clinicians

Natalia Leszczyńska1, Michał Furtak1, Zuzanna Jamrozy1, Jan Kurdybacha1, Oleksii Kravets1, Monika Roszkowska1, Martyna Biadasiewicz1, Aleksandra Pytlarz1, Natalia Lekston1, Dorota Piłat1, Gabriela Zemanek1, Przemysław Kotyla2

Abstract

This article reviews the diagnostic options used in the differentiation of two almost identical diseases in terms of clinical presentation, i.e. adult-onset Still's disease (AOSD) and sepsis.

At the initial stage of the diagnostic process, differentiating between these two pathogenetically distinct disease entities is difficult and sometimes impossible. Both AOSD and sepsis are characterised by a turbulent inflammatory response, difficult to control in the early stage of the disease. High fever and serum biomarkers of inflammation do not clearly indicate a specific condition but only make it difficult for clinicians to make a correct diagnosis and apply effective treatment. Over the last dozen or so years, global literature has proposed several solutions, including determining specific serum markers to indicate a higher probability of the occurrence of a specific disease, which may facilitate further diagnostic procedures.

Among the listed biomarkers, it is worth distinguishing those whose concentration in serum is significantly higher in the case of Still's disease. These include ferritin, haem oxygenase 1, lymphocytosis, platelet morphological indices such as PMR — platelet (PLT) to mean platelet volume (MPV) ratio and PPR — platelet anisocytosis index (PDW) and platelets (PLT) to anisocytosis index (PDW) ratio, interleukin 18 (IL-18), neutrophil (n) CD64 index profile and miRNA profile expression. The markers indicative of sepsis should also be considered. Heparin-binding protein, delta neutrophil index, mean platelet volume (MPV) and red cell distribution width (RDW) show higher levels in the cases of sepsis. Correlation of multiple biomarkers and combined determination of ferritin with several parameters such as platelet hematocrit, IL-18 or sCD163 also seem to be helpful.

Currently, there is no single, highly sensitive and specific marker to reliably discriminate between sepsis and adult-onset Still's disease. Some of the discussed markers are presently still only of scientific interest and their use in routine practice may be difficult due to the high cost of determination as well as methodological and technical difficulties. Therefore, it is necessary to further search for potential biomarkers and critically interpret the currently available indicators.

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