Review
Version 1
Preserved in Portico This version is not peer-reviewed
The Spectrum of Hyperferritinemic Syndromes
Version 1
: Received: 8 June 2023 / Approved: 9 June 2023 / Online: 9 June 2023 (09:10:35 CEST)
How to cite: Stoian, M.; Scarlat, G. The Spectrum of Hyperferritinemic Syndromes. Preprints 2023, 2023060690. https://doi.org/10.20944/preprints202306.0690.v1 Stoian, M.; Scarlat, G. The Spectrum of Hyperferritinemic Syndromes. Preprints 2023, 2023060690. https://doi.org/10.20944/preprints202306.0690.v1
Abstract
The concept of “hyperferritinemic syndrome” defines a complex pathophysiological and clinical entity, characterized by a systemic hyperinflammatory state, generally associated with elevated levels of proinflammatory cytokines (hypercytokinemia) and high serum levels of ferritin (hyperferritinemia), traditionally recognized as an iron-binding plasma protein involved in the storage of iron in a biologically available form for different physiological cellular processes. Recent studies have shown that ferritin has other important functions as well, operating both as a proinflammatory mediator and as a immunosupressive agent, its elevated levels in the serum being detected in various conditions associated with inflammatory states, such as infectious diseases, autoimmune and autoinflammatory disorders and, in some cases, in malignancies. Catastrophic antiphospholipid syndrome (cAPS), systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) and acquired forms of hemophagocytic lymphohistiocytosis (HLH), which includes macrophage activation syndrome (MAS), have been associated with hyperinflammatory states and dramatic elevations of serum ferritin, generating hyperferritinemic syndromes. Severe forms of coronavirus disease-2019 (COVID-19), in which MAS may frequently develop, has also been linked to the development of a hyperferritinemic syndrome. This review offers several insights into the physiological and pathophysiological roles of ferritin in several (hyper-)inflammatory diseases, but also into the causal entities, the underlying pathophysiological mechanisms, clinical manifestations and diagnostic features of somewhat obscure, yet potentially fatal pathological conditions, reunited under the concept of hyperferritinemic syndromes.
Keywords
hyperferritinemic syndrome; hyperinflammatory state; hypercitokinemia; immunosupression
Subject
Medicine and Pharmacology, Internal Medicine
Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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