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Beyond Juvenile Idiopathic Arthritis, Lupus and Juvenile Dermatomyositis: The Spectrum...
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Beyond Juvenile Idiopathic Arthritis, Lupus and Juvenile Dermatomyositis: The Spectrum of Pediatric Rheumatic Disease

A Special Issue of Children (ISSN 2227-9067).

Deadline for manuscript submissions: 5 April 2027 | Viewed by 3973

Editors

Dr. Kelly A. Rouster-Stevens

E-Mail Website
Guest Editor
1. Division of Pediatric Rheumatology, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA 30322, USA
2. Children’s Healthcare of Atlanta, Atlanta, GA 30329, USA
Interests: lupus; juvenile dermatomyositis; pediatric rheumatology
Dr. Elaine R. Flanagan

E-Mail Website
Guest Editor
1. Division of Pediatric Rheumatology, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA 30322, USA
2. Children’s Healthcare of Atlanta, Atlanta, GA 30329, USA
Interests: juvenile arthritis (JIA); uveitis; chronic non-infectious osteomyelitis (CNO)

Special Issue Information

Dear Colleagues,

Rheumatic diseases have been documented for centuries, yet the distinction between rheumatic disease in children and adults was not mentioned until 1897, when Still described a form of chronic arthritis in a child. It was not until 1976, at the inaugural American Rheumatism Association (ARA) Conference on the Rheumatic Diseases of Childhood in Park City, Utah, that pediatric rheumatology was founded. The following year, the European Union Against Rheumatism developed a classification of childhood arthritis at a conference in Oslo, Norway. Over the last 50 years, this field has greatly expanded, with significant progress being made in disease pathophysiology, therapeutics, and genetics. While disease etiology remains unclear for most pediatric rheumatologic diseases, patient outcomes are far better. This Special Issue will highlight the tremendous contributions to the field, which have resulted in improving the lives of children with pediatric rheumatologic disease. We welcome you to join us in fostering collaboration and progress in pediatric rheumatology. Your work will help us shape the future of research and inspire new directions in caring for children with rheumatic disease.

Dr. Kelly A. Rouster-Stevens
Dr. Elaine R. Flanagan
Guest Editors

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 250 words) can be sent to the Editorial Office for assessment.

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-anonymized peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). 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

  • pediatric rheumatology
  • juvenile idiopathic arthritis
  • childhood-onset systemic lupus erythematosus
  • juvenile dermatomyositis
  • autoinflammatory syndromes
  • immune dysregulation

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Published Papers (3 papers)

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Research

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13 pages, 765 KB  
Article
Assessing the Relationship Between Multidimensional Area-Level Indicators and Lupus Disease Activity in Children
by Chelsea Reynolds, Paul J. Nietert, Mileka Gilbert, Emily Vara, Natasha Ruth and Joyce Chang
Children 2026, 13(8), 1062; https://doi.org/10.3390/children13081062 - 10 Aug 2026
Viewed by 297
Abstract
Background/Objectives: Childhood-onset systemic lupus erythematosus (cSLE) is a chronic, multisystem autoimmune disease that is associated with more severe organ involvement, more intensive drug therapy, and increased long-term organ damage compared with adult-onset disease. The objectives of this study were to evaluate the performance [...] Read more.
Background/Objectives: Childhood-onset systemic lupus erythematosus (cSLE) is a chronic, multisystem autoimmune disease that is associated with more severe organ involvement, more intensive drug therapy, and increased long-term organ damage compared with adult-onset disease. The objectives of this study were to evaluate the performance of widely used small area-level multidimensional indicators of neighborhood disadvantage in a mixed urban–rural cSLE cohort against disease outcomes. Methods: This retrospective cohort study utilized electronic health records to identify and evaluate pediatric patients with cSLE across a single center in South Carolina between 1 January 2020 and 31 December 2024. Primary outcomes included disease activity at diagnosis, measured by Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K); the achievement of a low lupus disease activity state (LLDAS) by the last visit; the development of major organ involvement; and the rates of unplanned hospitalizations and emergency department visits. The associations of the census tract-level Area Deprivation Index (ADI), Social Vulnerability Index (SVI), and Childhood Opportunity Index (COI) with clinical presentation and outcomes were estimated using generalized linear models and logistic regression. Results: A total of 85 patients with cSLE were included, of which 76% reported being of Black race, and 28% lived in rural areas. Lupus disease severity was inconsistent across the metrics of area-level social vulnerability, neighborhood deprivation, child opportunity, and rurality. Patients who lived in more socially vulnerable communities were more likely to attain LLDAS during follow-up, while those who lived in lower-opportunity areas were more likely to develop CNS lupus. Baseline disease activity, renal involvement, and healthcare use were not significantly associated with area-level social disadvantages or rurality. Conclusions: In this single-center, mixed urban–rural cohort of children with cSLE, indicators of neighborhood-level disadvantage and rurality alone explained little variation in disease severity and disease control overall. The utility of available small area-level metrics is likely context-dependent and influenced by regional features, population characteristics, and the outcomes being studied. Future work should integrate individual- and area-level factors across diverse settings to better understand the drivers of health disparities. Full article
(This article belongs to the Special Issue Beyond Juvenile Idiopathic Arthritis, Lupus and Juvenile Dermatomyositis: The Spectrum of Pediatric Rheumatic Disease)
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Review

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21 pages, 642 KB  
Review
Childhood-Onset Systemic Lupus Erythematosus Research over the Past Decade in Japan
by Tasuku Tamai, Hiroyuki Wakiguchi and Kenji Ihara
Children 2026, 13(2), 250; https://doi.org/10.3390/children13020250 - 11 Feb 2026
Viewed by 2275
Abstract
Background: Childhood-onset systemic lupus erythematosus (cSLE) is a rare, serious autoimmune disease characterized by multiorgan involvement and long-term morbidity. Although several studies have examined this condition in Japan, a comprehensive summary of recent findings remains lacking. Methods: PubMed was searched for [...] Read more.
Background: Childhood-onset systemic lupus erythematosus (cSLE) is a rare, serious autoimmune disease characterized by multiorgan involvement and long-term morbidity. Although several studies have examined this condition in Japan, a comprehensive summary of recent findings remains lacking. Methods: PubMed was searched for Japanese publications on cSLE published between 2015 and 2025, including clinical studies, case reports, translational research, basic science studies, systematic reviews, clinical practice guidance, and transition care guidance. Results: Sixty publications met the inclusion criteria: 20 clinical studies, 30 case reports, 6 translational studies, 1 basic science study, 1 systematic review, 1 clinical practice guidance, and 1 transition care guidance. Most clinical studies were retrospective, although multicenter and registry-based designs have increased in recent years. Lupus nephritis remained the primary research focus, with accumulating evidence supporting mycophenolate mofetil, tacrolimus, and early belimumab as glucocorticoid (GC)-sparing approaches. Case reports illustrated the broad clinical spectrum of cSLE, with hematological and vascular complications being the most frequently reported. Translational studies highlighted the pathogenic role of type I interferon signaling and cytokine dysregulation, particularly in macrophage activation syndrome. Despite these advances, prospective studies and standardized assessment methods for pediatric-specific practice remain limited. Conclusions: Over the past decade, cSLE research in Japan has contributed to a deeper understanding of its clinical and immunological characteristics. However, treatment-related complications and long-term organ damage remain important challenges. Continued multicenter collaboration and domestic data accumulation may strengthen the evidence base, facilitate optimization of GC-sparing approaches, improve clinical management, and provide background information to support future discussions on clinical practice guidelines. Full article
(This article belongs to the Special Issue Beyond Juvenile Idiopathic Arthritis, Lupus and Juvenile Dermatomyositis: The Spectrum of Pediatric Rheumatic Disease)
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Other

Jump to: Research, Review

19 pages, 1007 KB  
Systematic Review
Comorbidities in Juvenile-Onset Rheumatic Diseases: A Systematic Review and Meta-Analysis
by Sab Siddiq, Shabnam Cheetham, Clare E. Pain, Eve M. D. Smith, Sizheng Steven Zhao, Liza J. McCann and David M. Hughes
Children 2026, 13(8), 995; https://doi.org/10.3390/children13080995 - 28 Jul 2026
Viewed by 837
Abstract
Background/Objectives: Patients with childhood-onset rheumatic diseases may be at additional risk of developing other health conditions. This systematic review aimed to (i) identify and describe the comorbidities associated with three significant childhood-onset rheumatic diseases—Juvenile Idiopathic Arthritis (JIA), Juvenile-onset Systemic Lupus Erythematosus (jSLE), [...] Read more.
Background/Objectives: Patients with childhood-onset rheumatic diseases may be at additional risk of developing other health conditions. This systematic review aimed to (i) identify and describe the comorbidities associated with three significant childhood-onset rheumatic diseases—Juvenile Idiopathic Arthritis (JIA), Juvenile-onset Systemic Lupus Erythematosus (jSLE), and Juvenile Dermatomyositis (JDM); (ii) describe comorbidity prevalence and incidence reported as apparent in childhood or adulthood, and (iii) compare these comorbidity estimates with control groups. Methods: PubMed, Web of Science, and Scopus databases were systematically searched without restrictions, in accordance with PRISMA guidelines. Where three or more studies reported the same comorbidity, a meta-analysis was performed using random-effect models. The risk of bias and study quality were assessed using an adjusted version of the Newcastle–Ottawa Scale. Results: Comorbidities were reported in 136,072 patients, of which 115,062 (84.56%) presented in childhood. There was significant heterogeneity within the results. The comorbidities presenting in childhood were uveitis (13.95%, 95% CI 11.79–16.43) in JIA patients, chronic kidney disease (48.38%, 95% CI 0.67–99.24) in jSLE patients, and calcinosis (29.70%, 95% CI 25.91–33.81) in JDM patients. The comorbidities identified in adult populations with childhood-onset rheumatic disease were uveitis (14.46%, 95% CI9.76–20.90) in JIA, hypertension (18.30%, 95% CI 7.52–38.16) in jSLE, and calcinosis (40.37%, 19.02–66.11) in JDM patients. Almost all comorbidities that were compared to control groups were more common in patients with childhood-onset rheumatic disease if statistically significant. Conclusions: Uveitis, chronic kidney disease/hypertension and calcinosis were most commonly identified among JIA, jSLE and JDM patients, respectively. Patients with the three childhood-onset rheumatic diseases evaluated in this systematic review were often found to be at higher risk of comorbidities compared to controls. This finding supports the need for the proactive, multidisciplinary management of comorbidities by clinicians and highlights the breadth of disease burden for patients. Full article
(This article belongs to the Special Issue Beyond Juvenile Idiopathic Arthritis, Lupus and Juvenile Dermatomyositis: The Spectrum of Pediatric Rheumatic Disease)
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