Abstract
Neutrophils are innate immune phagocytes that play a key role in immune defense against invading pathogens. The main offensive mechanisms of neutrophils are the phagocytosis of pathogens, release of granules, and production of cytokines. The formation of neutrophil extracellular traps (NETs) has been described as a novel defense mechanism in the literature. NETs are a network of fibers assembled from chromatin deoxyribonucleic acid, histones, and neutrophil granule proteins that have the ability to kill pathogens, while they can also cause toxic effects in hosts. Activated neutrophils with NET formation stimulate autoimmune responses related to a wide range of inflammatory autoimmune diseases by exposing autoantigens in susceptible individuals. The association between increased NET formation and autoimmunity was first reported in antineutrophil cytoplasmic antibody-related vasculitis, and the role of NETs in various diseases, including systemic lupus erythematosus, rheumatoid arthritis, and psoriasis, has since been elucidated in research. Herein, we discuss the mechanistic role of neutrophils, including NETs, in the pathogenesis of systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still’s disease (AOSD), and provide their clinical values as biomarkers for monitoring and prognosis.
Highlights
Serum CX3CL1 level may be used as a clinical marker to assess the disease activity of adult-onset Still’s disease (AOSD), and high serum C–X–C motif chemokine ligand (CXCL)–8 and ferritin reflected the presence of hemophagocytic syndrome
Active AOSD is associated with elevated levels of low-density granulocytes that produce IL–6
This review describes the roles of neutrophils and their release of neutrophil extracellular traps (NETs), as damage-associated molecular patterns (DAMPs), that contribute to aggravation of inflammation in the pathogenesis of AOSD and systemic juvenile idiopathic arthritis (SJIA)
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Autoinflammatory diseases are a group of rare diseases characterized by seemingly unexplained fever and inflammation affecting multiple organs, which belong to an area of clinical diseases other than autoimmune diseases [16] Both SJIA and AOSD are autoinflammatory diseases with similar clinical features and laboratory findings, with a high spiking fever, skin rash, generalized lymphadenopathy, hepatosplenomegaly, and leukocytosis [17,18]. A better understanding of the role of neutrophils and NETs in the pathogenesis of SJIA and AOSD may lead to the discovery of new biomarkers. We discuss the mechanistic role of neutrophils and NETs in the development and progression of SJIA and AOSD, and provide their clinical values as biomarkers for monitoring and prognosis
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