Circulating Neutrophil Extracellular Traps Signature for Identifying Organ Involvement and Response to Glucocorticoid in Adult-Onset Still’s Disease: A Machine Learning Study

Jia, Jieshuang; Wang, Mengyan; Ma, Yuning; Teng, Jialin; Shi, Hui; Liu, Honglei; Sun, Yue; Su, Yutong; Meng, Jianfen; Chi, Huihui; Chen, Xia; Cheng, Xiaobing; Ye, Junna; Liu, Tingting; Wang, Zhihong; Wan, Lei; Zhou, Zhongzheng; Wang, Fan; Yang, Chengde; Hu, Qiongyi

doi:10.3389/fimmu.2020.563335

Cited by 22 publications

(8 citation statements)

References 46 publications

(53 reference statements)

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“…GM-CSF can up-regulate neutrophil properties, including survival, adhesion and trafficking, oxidative burst, phagocytosis, and formation of NETs [ 145–149 ]. As increased neutrophil count and NETs are key characteristics of AOSD [ 150 , 151 ], it will be of interest to see if treatment targeting GM-CSF or its receptor will be a new, highly effective way. GM-CSF binds to GM-CSF receptor α (GM-CSFRα), and then macrophage and neutrophil are enhanced in number and function in inflammatory lesions, resulting in excessive secretion of pro-inflammatory cytokines, including IL-1β, IL-6, IL-12, IL-23 and TNF [ 152–154 ].…”

Section: Other Biologic Agentsmentioning

confidence: 99%

Current and emerging biological therapy in adult-onset Still’s disease

Meng

Jia

et al. 2021

Rheumatology

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Adult-onset Still’s disease (AOSD) is a rare, but characteristic non-familial, multi-genic systemic auto-inflammatory disorder, characterized by high spiking fever, salmon-like evanescent skin rash, polyarthritis, sore throat, hyperferritinemia, and leucocytosis. The hallmark of AOSD is a cytokine storm triggered by dysregulation of inflammation. Nowadays, with advances in anti-cytokine biologic agents, the treatment of AOSD is no longer limited to nonsteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, or conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs). In this review, we focused on the roles of these cytokines in the pathogenesis of AOSD and summarized the current and emerging biological therapy.

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Section: Other Biologic Agentsmentioning

confidence: 99%

Current and emerging biological therapy in adult-onset Still’s disease

Meng

Jia

et al. 2021

Rheumatology

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“…Differential expression of LL–37 was not found according to the disease activity status of AOSD [ 111 ]. In addition, circulating NETs have the potential to discriminate the involvement of the hepatic and cardiopulmonary systems among patients with AOSD, and they may help to provide therapeutic strategies by predicting their glucocorticoid response [ 115 ].…”

Section: Nets In Sjia and Aosdmentioning

confidence: 99%

An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease

Kim

Ahn

Jung

et al. 2021

IJMS

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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.

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“…Jia et al 68 observed that high levels of four circulating neutrophil extracellular traps (NETs; cell-free DNA, myeloperoxidase-DNA, neutrophil elastase-conjugated -DNA, and citrullinated histone 3 -DNA) correlated with levels of liver enzymes and inflammatory markers, as well as cardiopulmonary manifestations, suggesting the possible utility of circulating NETs as a biomarker of disease activity in AOSD. Recently, a study by Jung et al 69 identified that chemokine (C-C motif) ligand 2 levels in serum correlated with systemic score, leukocyte and neutrophil counts, and CRP, ferritin, lactate dehydrogenase, and albumin levels in patients with AOSD.…”

Section: Still’s Disease Continuummentioning

confidence: 99%

Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as Part of the Still’s Disease Continuum

Kontzias,

Petryna,

Nakasato

et al. 2024

MJR

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Aim: We have summarised the existing evidence supporting the concept that systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) are part of the same Still’s disease spectrum. Methods: A PubMed/Embase database search was conducted using specific search strings and free text words to screen for relevant articles. The search was limited to studies in humans, published up to June 2023, in English-language. Summary: sJIA and AOSD are rare autoinflammatory disorders that have similar pathophysiological and clinical features. The clinical presentations of sJIA and AOSD are highly variable, with differential diagnoses that include a broad range of malignancies, infectious diseases, and autoimmune disorders, which contribute to delays in diagnosis. Several sets of classification exist to help diagnose patients in clinical practice; the International League of Associations for Rheumatology criteria for sJIA and the Yamaguchi and Fautrel criteria for AOSD are the most-used criteria. The therapeutic strategy for Still’s disease aims to relieve signs and symptoms, prevent irreversible joint damage and potentially life-threatening complications, and avoid deleterious side effects of treatment. Recently, targeted therapies such as interleukin (IL)-1 and IL-6 inhibitors have become available for the treatment of sJIA and AOSD. While these biologics were originally largely reserved for patients in whom non-steroidal anti-inflammatory drugs, corticosteroids and conventional synthetic disease-modifying anti-rheumatic drugs had failed, they are increasingly used earlier in the treatment paradigm. Among IL-1 inhibitors, canakinumab is the only biologic approved in the US for the treatment of both sJIA and AOSD.

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Circulating Neutrophil Extracellular Traps Signature for Identifying Organ Involvement and Response to Glucocorticoid in Adult-Onset Still’s Disease: A Machine Learning Study

Cited by 22 publications

References 46 publications

Current and emerging biological therapy in adult-onset Still’s disease

Current and emerging biological therapy in adult-onset Still’s disease

An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease

Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as Part of the Still’s Disease Continuum

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