Ferritin is an intracellular iron storage protein and, in addition to its well-known function for the homeostasis of red-blood cells, it is produced and released in the circulation during inflammatory conditions. Furthermore, a typical 5-fold increase of ferritin levels is reported during AOSD and it is considered a useful marker to assess the activity of the disease. Erythrocyte sedimentation rate (ESR) is also reported to be associated with AOSD disease activity it is a non-specific measure of inflammation derived by the rate at which red blood cells in anticoagulated whole blood descend in a standardised tube over a period of one hour. This is a pentameric protein whose circulating concentrations rise in response to inflammation, following IL-6 secretion by macrophages and T cells. In fact, high levels of C-reactive protein (CRP) are commonly observed during disease flares. The AOSD inflammatory process may be detected and followed evaluating some specific serum biomarkers. In patients, who are inadequate responders to this therapeutic strategy, biological DMARDs (bDMARDs), mainly interleukin (IL)-1 and IL-6 inhibitors, are administered. Corticosteroids (CCSs) are considered the first-line therapy, often in combination with synthetic disease-modifying anti-rheumatic drugs (sDMARDs). The treatment of AOSD remains largely empirical, lacking validated guidelines, due to the rarity of the disease. The latter two patterns are affected by a more severe disease, sometimes requiring hospitalization in intensive care units because of life-threatening complications, mostly macrophage activation syndrome (MAS), a reactive form of haemophagocytic lymphohistocytosis (HLH). chronic pattern, affecting 40% of patients, showing a persistently active disease. polycyclic pattern, affecting 30% of patients, characterised by multiple flares alternating with remissions iii. monocyclic pattern, affecting 30% of patients, associated with a single episode and a good prognosis ii. Three clinical patterns are generally identified: i. It usually affects young adults and it is characterised by high spiking fever, arthritis, evanescent rash, and, in more severe cases, by internal organs involvement. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.ĭata Availability: All data generated by the analysis is included into the body of the present work or uploaded as supplementary materials.įunding: The authors received no specific funding for this work.Ĭompeting interests: The authors have declared that no competing interests exist.Īdult-onset Still’s disease (AOSD) is a rare and severe inflammatory disease of unknown aetiology, with a higher mortality rate. Received: MaAccepted: JPublished: July 9, 2020Ĭopyright: © 2020 Di Benedetto et al. PLoS ONE 15(7):Įditor: Massimo Cugno, Università degli Studi di Milano, ITALY Analysis of the multicentre Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale (GIRRCS) cohort. (2020) Ferritin and C-reactive protein are predictive biomarkers of mortality and macrophage activation syndrome in adult onset Still’s disease. Citation: Di Benedetto P, Cipriani P, Iacono D, Pantano I, Caso F, Emmi G, et al.
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