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Advances in Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (JIA)

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Immunology".

Deadline for manuscript submissions: closed (31 August 2022) | Viewed by 13733

Special Issue Editor

Dr. Hyoun-Ah Kim

E-Mail Website
Guest Editor
Department of Rheumatology, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon 443-380, Korea
Interests: rheumatology; adult onset still's disease; systemic lupus erythematosus

Special Issue Information

Dear Colleagues,

Systemic juvenile idiopathic arthritis (JIA) and adult-onset Still’s disease (AOSD) are systemic inflammatory diseases characterized by fever, evanescent skin rash, pleuritis, hepatosplenomegaly, and arthritis. This extreme variability depends on a diagnostic uncertainty, especially in the early stages of the disease, which has increased the interest in finding new disease biomarkers to better understand its etiopathogenetic mechanisms, to make an early diagnosis, to predict prognosis and to aid clinical decision-making. To date, the diagnosis is based on clinical sign and symptoms interpretation and the detection of serologic nonspecific inflammatory markers of immunological activation.

This issue will bring to the forefront scientific advances in the understanding of the pathophysiology and immunobiology of AOSD and JIA.

We welcome the submission of articles that synthesize research advances in our understanding of the immune and/or inflammatory mechanisms underlying systemic JIA or AOSD. Contributors are encouraged to include findings from animal models; molecular or cellular research related to the (i) immune; (ii) inflammatory; (iii) biochemical processes and (iv) novel or established biomarkers of JIA or AOSD, underlying molecular mechanisms that could be used for disease management, from diagnosis to response to therapies. The overall goal is to highlight unique immunobiology/biology, which may provide lessons and insights into innovations in the management of JIA and AOSD.

Dr. Hyoun-Ah Kim
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

systemic inflammation; autoinflammation; damage-associated molecular pattern; pediatrics; inflammatory marker

Published Papers (4 papers)

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Review

61 pages, 1397 KiB  
Review
Biomarkers in Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome and Their Importance in COVID Era
by Laura Marinela Ailioaie, Constantin Ailioaie and Gerhard Litscher
Int. J. Mol. Sci. 2022, 23(21), 12757; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms232112757 - 22 Oct 2022
Cited by 4 | Viewed by 2810
Abstract
Systemic juvenile idiopathic arthritis (sJIA) and its complication, macrophage activation syndrome (sJIA-MAS), are rare but sometimes very serious or even critical diseases of childhood that can occasionally be characterized by nonspecific clinical signs and symptoms at onset—such as non-remitting high fever, headache, rash, [...] Read more.
Systemic juvenile idiopathic arthritis (sJIA) and its complication, macrophage activation syndrome (sJIA-MAS), are rare but sometimes very serious or even critical diseases of childhood that can occasionally be characterized by nonspecific clinical signs and symptoms at onset—such as non-remitting high fever, headache, rash, or arthralgia—and are biologically accompanied by an increase in acute-phase reactants. For a correct positive diagnosis, it is necessary to rule out bacterial or viral infections, neoplasia, and other immune-mediated inflammatory diseases. Delays in diagnosis will result in late initiation of targeted therapy. A set of biomarkers is useful to distinguish sJIA or sJIA-MAS from similar clinical entities, especially when arthritis is absent. Biomarkers should be accessible to many patients, with convenient production and acquisition prices for pediatric medical laboratories, as well as being easy to determine, having high sensitivity and specificity, and correlating with pathophysiological disease pathways. The aim of this review was to identify the newest and most powerful biomarkers and their synergistic interaction for easy and accurate recognition of sJIA and sJIA-MAS, so as to immediately guide clinicians in correct diagnosis and in predicting disease outcomes, the response to treatment, and the risk of relapses. Biomarkers constitute an exciting field of research, especially due to the heterogeneous nature of cytokine storm syndromes (CSSs) in the COVID era. They must be selected with utmost care—a fact supported by the increasingly improved genetic and pathophysiological comprehension of sJIA, but also of CSS—so that new classification systems may soon be developed to define homogeneous groups of patients, although each with a distinct disease. Full article
(This article belongs to the Special Issue Advances in Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (JIA))
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33 pages, 995 KiB  
Review
Implications of SARS-CoV-2 Infection in Systemic Juvenile Idiopathic Arthritis
by Laura Marinela Ailioaie, Constantin Ailioaie and Gerhard Litscher
Int. J. Mol. Sci. 2022, 23(8), 4268; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms23084268 - 12 Apr 2022
Cited by 9 | Viewed by 3567
Abstract
Systemic juvenile idiopathic arthritis (sJIA) is a serious multifactorial autoinflammatory disease with a significant mortality rate due to macrophage activation syndrome (MAS). Recent research has deepened the knowledge about the pathophysiological mechanisms of sJIA-MAS, facilitating new targeted treatments, and biological disease-modifying antirheumatic drugs [...] Read more.
Systemic juvenile idiopathic arthritis (sJIA) is a serious multifactorial autoinflammatory disease with a significant mortality rate due to macrophage activation syndrome (MAS). Recent research has deepened the knowledge about the pathophysiological mechanisms of sJIA-MAS, facilitating new targeted treatments, and biological disease-modifying antirheumatic drugs (bDMARDs), which significantly changed the course of the disease and prognosis. This review highlights that children are less likely to suffer severe COVID-19 infection, but at approximately 2–4 weeks, some cases of multisystem inflammatory syndrome in children (MIS-C) have been reported, with a fulminant course. Previous established treatments for cytokine storm syndrome (CSS) have guided COVID-19 therapeutics. sJIA-MAS is different from severe cases of COVID-19, a unique immune process in which a huge release of cytokines will especially flood the lungs. In this context, MIS-C should be reinterpreted as a special MAS, and long-term protection against SARS-CoV-2 infection can only be provided by the vaccine, but we do not yet have sufficient data. COVID-19 does not appear to have a substantial impact on rheumatic and musculoskeletal diseases (RMDs) activity in children treated with bDMARDs, but the clinical features, severity and outcome in these patients under various drugs are not yet easy to predict. Multicenter randomized controlled trials are still needed to determine when and by what means immunoregulatory products should be administered to patients with sJIA-MAS with a negative corticosteroid response or contraindications, to optimize their health and safety in the COVID era. Full article
(This article belongs to the Special Issue Advances in Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (JIA))
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14 pages, 644 KiB  
Review
Adult-Onset Still’s Disease: Novel Biomarkers of Specific Subsets, Disease Activity, and Relapsing Forms
by Beatrice Maranini, Giovanni Ciancio and Marcello Govoni
Int. J. Mol. Sci. 2021, 22(24), 13320; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms222413320 - 11 Dec 2021
Cited by 15 | Viewed by 3201
Abstract
Adult-onset Still’s disease (AOSD) is a systemic inflammatory disease of unknown etiology. Recent studies have demonstrated that the hallmark of AOSD is a cytokine storm, which is characterized by the excessive production of interleukin (IL)-1, IL-6, IL-18, tumor necrosis factor-α (TNF-α), and interferon-γ [...] Read more.
Adult-onset Still’s disease (AOSD) is a systemic inflammatory disease of unknown etiology. Recent studies have demonstrated that the hallmark of AOSD is a cytokine storm, which is characterized by the excessive production of interleukin (IL)-1, IL-6, IL-18, tumor necrosis factor-α (TNF-α), and interferon-γ (IFN-γ), suggesting how pro-inflammatory cytokines play an important role in the pathogenesis of this disease. Actually, a certain proportion of patients (around 17–32%) with severe clinical symptoms achieves only partial remission or is resistant to both first-line corticosteroids and second-line DMARDs. These patients are defined as refractory AOSD patients, requiring higher dosage glucocorticoids, longer treatment duration, or the simultaneous introduction of immunosuppressive drugs, further leading to AOSD relapses. In this narrative review, we will analyze the latest literature data to unravel potential pathogenetic factors associated with specific patterns of AOSD disease or relapses in order to identify biomarkers that may guide clinical decisions, eventually leading to new therapeutic options. Full article
(This article belongs to the Special Issue Advances in Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (JIA))
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16 pages, 1195 KiB  
Review
An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease
by Ji-Won Kim, Mi-Hyun Ahn, Ju-Yang Jung, Chang-Hee Suh and Hyoun-Ah Kim
Int. J. Mol. Sci. 2021, 22(23), 13038; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms222313038 - 02 Dec 2021
Cited by 20 | Viewed by 3408
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Advances in Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (JIA))
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