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Advances in Juvenile Idiopathic Arthritis
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Advances in Juvenile Idiopathic Arthritis

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Allergy and Immunology".

Deadline for manuscript submissions: closed (10 May 2022) | Viewed by 18254

Special Issue Editors

Dr. Giovanni Filocamo

E-Mail Website
Guest Editor
Pediatric Rheumatology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via della Commenda 9, 20122 Milan, Italy
Interests: outcome measures in rheumatology; JIA; intraarticular joint injections; biosimilars
Dr. Stefano Lanni

E-Mail Website
Guest Editor
Pediatric Rheumatology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via della Commenda 9, 20122 Milan, Italy
Interests: ultrasound in the evaluation of patients with JIA; intraarticular joint injections
Dr. Francesca Minoia

E-Mail Website
Guest Editor
Pediatric Rheumatology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via della Commenda 9, 20122 Milan, Italy
Interests: systemic JIA; macrophage activation syndrome

Special Issue Information

Dear Colleagues,

Juvenile idiopathic arthritis (JIA) is the most common chronic joint disease in pediatric rheumatology and encompasses a heterogeneous group of disorders. JIA is one of the major potential causes of permanent joint inability in childhood, and extra-articular complications such as chronic uveitis could significantly impact its morbidity. Systemic JIA is increasingly recognized as a specific entity with a peculiar crosstalk between the innate and adaptative immune system, and its life-threatening complication, the macrophage activation syndrome, requires prompt recognition and appropriate treatment.

The continuous improvements in translational research, the widespread use of intra-articular glucocorticoids, and the increasing availability of a broad spectrum of innovative therapies that are tailor-made to all different subsets of JIA have resulted in a dramatic change in the way to approach the disease and to a significant improvement of long-term outcomes.

The regular assessment of the disease through standardized outcome measures in association with feasible parent- and child-centered assessment tools became routinely adopted in the management of JIA and allowed increasing the quality of care of patients. Furthermore, coupling clinical examination with novel and sensitive imaging modalities in the evaluation of patients with JIA might foster the capacity to achieve earlier assessment of disease activity and to evaluate treatment efficacy more precisely. The goal of this Special Issue is to explore all these aspects by pointing out the state-of-the-art knowledge based on daily clinical experience and on evidence from literary reviews.

Dr. Giovanni Filocamo
Dr. Stefano Lanni
Dr. Francesca Minoia
Guest Editors

Manuscript Submission Information

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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

  • juvenile idiopathic arthritis
  • outcome measures
  • ultrasound
  • treatment
  • biologics
  • intra-articular glucocorticoids
  • macrophage activation syndrome

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Published Papers (7 papers)

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Research

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12 pages, 879 KiB  
Article
Why and How Should We Assess the Cardiovascular Risk in Patients with Juvenile Idiopathic Arthritis? A Single-Centre Experience with Carotid Intima-Media Measurements
by Marta Gruca, Krzysztof Orczyk, Justyna Zamojska, Katarzyna Niewiadomska-Jarosik, Jerzy Stańczyk and Elżbieta Smolewska
Children 2023, 10(3), 422; https://doi.org/10.3390/children10030422 - 22 Feb 2023
Viewed by 1955
Abstract
Background: Children diagnosed with juvenile idiopathic arthritis (JIA) are thought to be more likely to develop cardiovascular disease in adulthood. The factors modulating the cardiovascular risk, involving exposure to secondhand smoking, sedentary lifestyle and abnormal body mass index, might have had a stronger [...] Read more.
Background: Children diagnosed with juvenile idiopathic arthritis (JIA) are thought to be more likely to develop cardiovascular disease in adulthood. The factors modulating the cardiovascular risk, involving exposure to secondhand smoking, sedentary lifestyle and abnormal body mass index, might have had a stronger impact during the COVID-19 pandemic. The lack of reliable prognostic markers for a higher probability of cardiovascular events might be solved by carotid intima-media thickness (cIMT) measurement. The paramount goal of the study was to assess its usefulness in JIA patients. Materials and Methods: The results of cIMT measured by a single physician in 45 children diagnosed with JIA were compared to 37 age- and sex-matched healthy counterparts. The analysis also involved anthropometric parameters, laboratory tests, and a survey regarding lifestyle-related factors. Results: Four JIA patients appeared to have cIMT above the 94th percentile. A positive correlation between erythrocytes sedimentation rate (ESR) and right carotid artery percentiles was found. Passive smoking increased the cardiovascular risk regardless of JIA. Doubling the daily screen time during the pandemic led to a significant reduction in children’s physical activity. However, the number of enrolled subjects was not enough to make significant recommendations. Conclusions: cIMT measurements remain an interesting perspective for future cardiovascular screening of children with JIA. It has yet to be determined whether it should be considered in all JIA patients on a reliable basis. Full article
(This article belongs to the Special Issue Advances in Juvenile Idiopathic Arthritis)
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15 pages, 994 KiB  
Article
A Mixed Method Study: Defining the Core Learning Needs of Nurses Delivering Care to Children and Young People with Rheumatic Disease to Inform Paediatric Musculoskeletal Matters, a Free Online Educational Resource
by Nicola Smith, Christine English, Barbara Davies, Ruth Wyllie, Helen E. Foster and Tim Rapley
Children 2022, 9(6), 844; https://doi.org/10.3390/children9060844 - 7 Jun 2022
Cited by 1 | Viewed by 2096
Abstract
Children and young people with rheumatic diseases and their families are often supported by nurses who may not have had specialist training in paediatric rheumatology. The purpose of our study was to establish the core learning needs of all nurses who may encounter [...] Read more.
Children and young people with rheumatic diseases and their families are often supported by nurses who may not have had specialist training in paediatric rheumatology. The purpose of our study was to establish the core learning needs of all nurses who may encounter these children and young people in their clinical practice and use this information to inform the content and format of Paediatric Musculoskeletal Matters Nursing (PMM-Nursing) Engagement with nurses working in different roles and with various levels of experience in musculoskeletal medicine informed these learning needs and PMM-Nursing content. Mixed methods ascertained learning needs under the following themes: (1) Need for increased awareness about rheumatic disease; (2) Impact of experience and nursing role; (3) Need for increased knowledge about rheumatic disease and management. In addition, our methods informed design components for an impactful learning and information resource. Representatives from stakeholder nursing groups, social sciences, and web development used this information to create a suitable framework for PMM-Nursing. The content of PMM-Nursing is now live and freely available. Full article
(This article belongs to the Special Issue Advances in Juvenile Idiopathic Arthritis)
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13 pages, 2053 KiB  
Article
Impact of HLA-B27 and Disease Status on the Gut Microbiome of the Offspring of Ankylosing Spondylitis Patients
by Matthew L. Stoll, Kimberly DeQuattro, Zhixiu Li, Henna Sawhney, Pamela F. Weiss, Peter A. Nigrovic, Tracey B. Wright, Kenneth Schikler, Barbara Edelheit, Casey D. Morrow, John D. Reveille, Matthew A. Brown and Lianne S. Gensler
Children 2022, 9(4), 569; https://doi.org/10.3390/children9040569 - 16 Apr 2022
Cited by 13 | Viewed by 4707 | Correction
Abstract
Multiple studies have shown the microbiota to be abnormal in patients with spondyloarthritis (SpA). The purpose of this study was to explore the genetic contributions of these microbiota abnormalities. We analyzed the impact of HLA-B27 on the microbiota of children at risk for [...] Read more.
Multiple studies have shown the microbiota to be abnormal in patients with spondyloarthritis (SpA). The purpose of this study was to explore the genetic contributions of these microbiota abnormalities. We analyzed the impact of HLA-B27 on the microbiota of children at risk for SpA and compared the microbiota of HLA-B27+ pediatric offspring of ankylosing spondylitis (AS) patients with that of HLA-B27+ children with SpA. Human DNA was obtained from the offspring for determination of HLA-B27 status and polygenic risk score (PRS). Fecal specimens were collected from both groups for sequencing of the V4 region of the 16S ribosomal RNA gene. Among the offspring of AS patients, there was slight clustering by HLA-B27 status. After adjusting for multiple comparisons, five operational taxonomic units (OTUs) representing three unique taxa distinguished the HLA-B27+ from negative children: Blautia and Coprococcus were lower in the HLA-B27+ offspring, while Faecalibacterium prausnitzii was higher. HLA-B27+ offspring without arthritis were compared to children with treatment-naïve HLA-B27+ SpA. After adjustments, clustering by diagnosis was present. A total of 21 OTUs were significantly associated with diagnosis state, including Bacteroides (higher in SpA patients) and F. prausnitzii (higher in controls). Thus, our data confirmed associations with B. fragilis and F. prausnitzii with juvenile SpA, and also suggest that the mechanism by which HLA-B27 is associated with SpA may not involve alterations of the microbiota. Full article
(This article belongs to the Special Issue Advances in Juvenile Idiopathic Arthritis)
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6 pages, 512 KiB  
Communication
Ultrasonographic Assessment for Tenosynovitis in Juvenile Idiopathic Arthritis with Ankle Involvement: Diagnostic and Therapeutic Significance
by Sara Della Paolera, Serena Pastore, Alen Zabotti, Alberto Tommasini and Andrea Taddio
Children 2022, 9(4), 509; https://doi.org/10.3390/children9040509 - 3 Apr 2022
Cited by 4 | Viewed by 2054
Abstract
Background: The role of musculoskeletal ultrasound in JIA is still controversial, although there is growing evidence on its utility, especially in the diagnosis of tenosynovitis. Methods: We presented a retrospective cross-sectional study of a group of patients with JIA with ankle swelling followed [...] Read more.
Background: The role of musculoskeletal ultrasound in JIA is still controversial, although there is growing evidence on its utility, especially in the diagnosis of tenosynovitis. Methods: We presented a retrospective cross-sectional study of a group of patients with JIA with ankle swelling followed in a Pediatric Rheumatology Service of a tertiary-level pediatric hospital in Northern Italy during the follow-up period between January 1st 2003 and December 31st 2019. Preliminary results have been presented at the EULAR Congress 2021. We enrolled only patients who underwent msk-US, and we identified those with a clinical and sonographic diagnosis of tenosynovitis. For each patient, we collected data on demographics, clinical characteristics, and therapeutic strategies during the follow-up. Results: On December 31st 2019, 56 swollen ankles of 48 patients were assessed with msk-US. Twenty-two ankles showed sonographic signs of joint synovitis, sixteen ankles presented signs of both joint synovitis and tenosynovitis, and fourteen ankles presented sonographic signs of tenosynovitis only. Overall, tenosynovitis was detected on 27 (56%) out of 48 children with at least a swollen ankle. In 13 patients out of 27 with tenosynovitis (48%), there was no joint synovitis of ankle or foot. Twenty-five patients with tenosynovitis (92%) achieved clinical and radiological remission: seven patients achieved remission of tenosynovitis with methotrexate only, and fifteen patients with biological drugs alone or in combination therapy. Conclusions: We observed that more than half of the patients with ankle swelling presented a tenosynovitis, and about 50% of them did not show sonographic signs of an active joint synovitis. Among patients with tenosynovitis, biological therapy alone or in association with DMARDs showed effectiveness in inducing disease remission. Full article
(This article belongs to the Special Issue Advances in Juvenile Idiopathic Arthritis)
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8 pages, 886 KiB  
Article
Discordance between Clinical and Ultrasound Examinations in Juvenile Idiopathic Arthritis: An Experimental Approach
by Francesco Licciardi, Marco Petraz, Carlotta Covizzi, Francesca Santarelli, Carlotta Cirone, Roberta Mulatero, Francesca Robasto, Marta Dellepiane, Silvana Martino, Davide Montin and Viviana Ravagnani
Children 2022, 9(3), 333; https://doi.org/10.3390/children9030333 - 1 Mar 2022
Cited by 4 | Viewed by 1744
Abstract
Clinical examination (CE) and musculoskeletal ultrasound (MSUS) of ten joints (knee, ankle, wrist, elbow, II-MCP) and their extra-articular (EA) compartments (tendons and bursae) were performed on 35 consecutive patients with active juvenile idiopathic arthritis (JIA) (active group) to test how the extension of [...] Read more.
Clinical examination (CE) and musculoskeletal ultrasound (MSUS) of ten joints (knee, ankle, wrist, elbow, II-MCP) and their extra-articular (EA) compartments (tendons and bursae) were performed on 35 consecutive patients with active juvenile idiopathic arthritis (JIA) (active group) to test how the extension of MSUS examinations to EA changes the concordance between MSUS and CE. The overall concordance between CE and MSUS, measured with Cohen’s Kappa (k), was moderate (k = 0.43); the addition of EA MSUS increased the concordance in all joints, with the exclusion of II-MCP (k = 0.49). In the ankle and wrist, the k increase was relevant (k from 0.13 to 0.27 and 0.11 to 0.41). In the active group patients, we observed 44 subclinical synovitis; the number of subclinical synovitis per patient was correlated with JADAS-27 (p = 0.03) and was higher in a control group composed of 15 patients with persistent disease remission (1.3 vs. 0.4 p = 0.03). Our results show that EA compartments should always be evaluated during MSUS. Furthermore, we demonstrate a moderate concordance between CE and MSUS in JIA; the finding of subclinical synovitis is common in patients with active diseases and is related to disease activity. Full article
(This article belongs to the Special Issue Advances in Juvenile Idiopathic Arthritis)
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Review

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12 pages, 2909 KiB  
Review
Role of Ultrasound Evaluation of Temporomandibular Joint in Juvenile Idiopathic Arthritis: A Systematic Review
by Achille Marino, Orazio De Lucia and Roberto Caporali
Children 2022, 9(8), 1254; https://doi.org/10.3390/children9081254 - 19 Aug 2022
Cited by 3 | Viewed by 2423
Abstract
Background: Juvenile idiopathic arthritis (JIA) is childhood’s most frequent chronic rheumatic disease. JIA is a broad term that includes all arthritides starting before 16 years, lasting at least six weeks, and of unknown cause. The temporomandibular joint (TMJ) could be involved in JIA [...] Read more.
Background: Juvenile idiopathic arthritis (JIA) is childhood’s most frequent chronic rheumatic disease. JIA is a broad term that includes all arthritides starting before 16 years, lasting at least six weeks, and of unknown cause. The temporomandibular joint (TMJ) could be involved in JIA both at onset and during the disease course. The presence of TMJ synovitis might severely impair dentofacial maturation in pediatric patients. The ultrasound (US) application to detect early signs of TMJ synovitis in children with JIA has provided contradictory results. We sought to assess the current role of TMJ US in JIA through a systematic literature review. Methods: The systematic review was conducted according to the recommendations of the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA). Results: The literature search found 345 records. After duplicates removal, 253 records were screened, 20 full-text articles were reviewed to assess their eligibility, and 7 of them were included in the qualitative analysis. Joint effusion was the most recorded parameter, followed by bony condylar abnormalities. Compared to contrast enhancement MRI, the capability to detect signs of active synovitis of TMJ by US is low, especially at the early stages. Conclusion: Understanding how US may help diagnose and manage children with JIA is advisable for several reasons. MRI cannot be frequently repeated, may need sedation, and is expensive. The constant technical improvement of US will undoubtedly allow for better evaluation of what, in the past, was not clear or not even captured by sonography. So far, the role of US in the assessment of TMJ involvement in JIA is indubitably secondary to the MRI. Even so, we think that a baseline MRI of TMJ and the repetition of the sonography over time might both help the interpretation of US images and intercept significative changes. Full article
(This article belongs to the Special Issue Advances in Juvenile Idiopathic Arthritis)
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Other

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7 pages, 226 KiB  
Case Report
Infection-Triggered Hyperinflammatory Syndromes in Children
by Martina Rossano, Greta Rogani, Maria Maddalena D’Errico, Martina Cucchetti, Francesco Baldo, Sofia Torreggiani, Gisella Beretta, Stefano Lanni, Antonella Petaccia, Carlo Agostoni, Giovanni Filocamo and Francesca Minoia
Children 2022, 9(4), 564; https://doi.org/10.3390/children9040564 - 15 Apr 2022
Viewed by 2196
Abstract
An association between infectious diseases and macrophage activation syndrome (MAS) has been reported, yet the exact role of infection in MAS development is still unclear. Here, a retrospective analysis of the clinical records of patients with rheumatic diseases complicated with MAS who were [...] Read more.
An association between infectious diseases and macrophage activation syndrome (MAS) has been reported, yet the exact role of infection in MAS development is still unclear. Here, a retrospective analysis of the clinical records of patients with rheumatic diseases complicated with MAS who were treated in a pediatric tertiary care center between 2011 and 2020 was performed. Any infection documented within the 30 days preceding the onset of MAS was reported. Out of 125 children in follow-up for systemic rheumatic diseases, 12 developed MAS, with a total of 14 episodes. One patient experienced three episodes of MAS. Clinical and/or laboratory evidence of infection preceded the onset of MAS in 12 events. Clinical features, therapeutic strategies, and patient outcomes were described. The aim of this study was to evaluate the possible role of infection as a relevant trigger for MAS development in children with rheumatic conditions. The pathogenetic pathways involved in the cross-talk between uncontrolled inflammatory activity and the immune response to infection deserve further investigation. Full article
(This article belongs to the Special Issue Advances in Juvenile Idiopathic Arthritis)
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