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Journal of the Korean Society of... May 2024Infections and inflammatory conditions of immature musculoskeletal systems in pediatric patients also affect the adjacent muscles, connective tissues, and joints. Rapid... (Review)
Review
Infections and inflammatory conditions of immature musculoskeletal systems in pediatric patients also affect the adjacent muscles, connective tissues, and joints. Rapid diagnosis leading to appropriate treatment can significantly impact the occurrence of complications and mortality rates due to these conditions. When a radiologist becomes familiar with the imaging findings of pediatric musculoskeletal infections and inflammatory diseases, rapid differential diagnoses and more timely and appropirate treatment could be possible. In this paper, we introduce the imaging findings of infectious and inflammatory diseases affecting the immature musculoskeletal system, such as osteomyelitis, pyogenic arthritis, juvenile idiopathic arthritis, and hemophilic arthritis, based on the anatomical and pathophysiological characteristics of the immature musculoskeletal system in children.
PubMed: 38873370
DOI: 10.3348/jksr.2024.0057 -
International Journal of Paediatric... Jun 2024Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, and temporomandibular joints (TMJs) are involved in 39%-78% of patients. (Review)
Review
BACKGROUND
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, and temporomandibular joints (TMJs) are involved in 39%-78% of patients.
AIM
The aim of this systematic review was to assess the effectiveness of conservative approaches in improving TMJ arthritis in children and adolescents affected by JIA.
DESIGN
PubMed, Scopus, and Web of Science were systematically searched from the inception until February 25, 2024, to identify observational studies presenting participants with a diagnosis of JIA affecting the TMJ, rehabilitative approaches for TMJ arthritis as interventions, and clinical or radiological assessment of TMJ arthritis as outcome.
RESULTS
Of 478 papers suitable for title/abstract screening, 13 studies were included. The studies evaluated the effectiveness of intra-articular (IA) corticosteroid (CS) injections, IA infliximab injections, arthrocentesis alone or in combination with IACS injections, occlusal splint, functional appliance, and physiotherapy. The effectiveness of IACS injections was shown in eight studies. IA infliximab injections did not appear to significantly improve TMJ arthritis.
CONCLUSION
Results of this systematic review suggested that conservative treatments, especially IACS injections, might be effective in improving TMJ arthritis in patients affected by JIA. Further studies with a higher level of evidence and more representative samples should be conducted.
PubMed: 38863137
DOI: 10.1111/ipd.13225 -
European Journal of Orthodontics Aug 2024An update on the knowledge regarding the orthopedic/orthodontic role in treating JIA-related dentofacial deformities is relevant.
BACKGROUND
An update on the knowledge regarding the orthopedic/orthodontic role in treating JIA-related dentofacial deformities is relevant.
OBJECTIVES
This systematic review aimed to assess the level of evidence regarding the management of dentofacial deformity from juvenile idiopathic arthritis (JIA) with orthodontics and/or dentofacial orthopedics.
SEARCH METHODS
The following databases were searched without time or language restrictions up to 31 January 2024 (Medline, Embase, Cochrane Central Register of Controlled Trials, Scopus, Web of Science, and Latin American and Caribbean Health Sciences Literature).
SELECTION CRITERIA
Inclusion criteria were studies dealing with JIA subjects receiving treatment with orthodontic and/or dentofacial orthopedic functional appliances.
DATA COLLECTION AND ANALYSIS
After the removal of duplicate studies, data extraction, and risk of bias assessment according to ROBINS-I guidelines were conducted. Data extraction was conducted by two independent authors.
RESULTS
The electronic database search identified 397 eligible articles after the removal of duplicates. Following the application of the pre-defined inclusion and exclusion criteria, 11 articles were left for inclusion. Two trials were associated with a severe risk of bias, four trials were at moderate risk of bias, and the other five presented a low risk of bias. Various research groups employed and documented the effects of different types of appliances and methodologies. The study heterogeneity did not allow for meta-analyses. In addition, a lack of uniformity in treatment objectives was observed across the included studies. After treatment with dentofacial orthopedics skeletal improvement was demonstrated in 10 studies, and a decrease in orofacial signs and symptoms was reported in 7 studies.
CONCLUSIONS
Across the available literature, there is minor evidence to suggest that dentofacial orthopedics may be beneficial in the management of dentofacial deformities from JIA. There is little evidence to suggest that it can reduce orofacial signs and symptoms in patients with JIA. Based on current evidence, it is not possible to outline clinical recommendations for specific aspects of orthopedic management in growing subjects with JIA-related dentofacial deformity.
REGISTRATION
PROSPERO (CRD42023390746).
Topics: Humans; Arthritis, Juvenile; Dentofacial Deformities; Orthodontics, Corrective; Orthopedic Procedures; Orthodontic Appliances, Functional
PubMed: 38860748
DOI: 10.1093/ejo/cjae023 -
Rheumatology (Oxford, England) Jun 2024Previous epidemiological data of JIA in Finland are from the turn of the millennium. We aimed to determine the recent annual incidence of JIA in several consecutive...
OBJECTIVE
Previous epidemiological data of JIA in Finland are from the turn of the millennium. We aimed to determine the recent annual incidence of JIA in several consecutive years in Finland and to explore the differences in incidence between sexes, age groups, and regions.
METHODS
We analyzed all children <16 years of age who met the ILAR classification criteria for JIA. Cases from 2000-2020 were identified from two national registers: the Care Register for Health Care of the Finnish Institute for Health and Welfare and the Reimbursement Register containing medication data from the Social Insurance Institution of Finland; cases from 2016-2020 were identified from the Finnish Rheumatology Quality Register.
RESULTS
The incidence of JIA was 31.7 per 100 000 (95% CI 30.2, 33.1), according to the Care Register in 2000-2020 and peaked in 2010-2014. No considerable differences in incidence rates were observed among registers. In all age groups, incidence in girls was predominant compared with boys. The incidence in girls peaked at the ages of 2 years and 14-15 years. Decreasing incidence was observed among boys 0-3 years old during the entire study period, whereas increasing incidence was observed among teenage girls and boys 4-7 years old in 2000-2013.
CONCLUSION
The incidence of JIA is not only very high with respect to that in other parts of the world but also higher than previously reported in Finland. The incidence varied by region and year but was not higher at the end than the beginning of the study period.
PubMed: 38857448
DOI: 10.1093/rheumatology/keae322 -
Ocular Immunology and Inflammation Jun 2024To report a case of presumed fungal infection in a patient with JIA following prolong immunosuppression, and after initiation of adalimumab therapy. Method:...
PURPOSE
To report a case of presumed fungal infection in a patient with JIA following prolong immunosuppression, and after initiation of adalimumab therapy. Method: Retrospective Chart Review.
RESULT
A 20-year-old female, previously diagnosed with JIA, presented with a three-week history of blurred vision in her left eye. She had a long history of treatment with oral corticosteroids, sulfasalazine, and methotrexate, followed by tocilizumab injections and later etanercept. Recently, she was started on adalimumab injections. Fundus examination of the left eye demonstrated multifocal retinitis scattered throughout the fundus. Optical coherence tomography of the lesions showed hyperreflectivity in the inner retina with posterior shadowing and vitreous aggregates extending into the vitreous cavity. After her second adalimumab dose, she experienced blurred vision. Examination of the fundus revealed multifocal retinitis in the left eye, sparing the macula. After stopping immunomodulators and starting empirical antifungal therapy with oral fluconazole, her retinal lesions began to improve. A vitreous biopsy was performed, and intravitreal voriconazole was administered, but microbiological tests were negative. Nevertheless, her retinal lesions resolved almost completely with continued antifungal treatment. By the 6-week follow-up, her retinitis had fully resolved, maintaining excellent visual acuity.
CONCLUSION
This case underscores the need for a high index of suspicion for infection in patients with long-term immunosuppression, highlighting the importance of early therapeutic intervention.
PubMed: 38856750
DOI: 10.1080/09273948.2024.2361355 -
Best Practice & Research. Clinical... Jun 2024Juvenile Idiopathic Arthritis (JIA) is sometimes considered a diagnosis of exclusion as the name signifies that no cause is evident for this form of arthritis. Despite... (Review)
Review
Juvenile Idiopathic Arthritis (JIA) is sometimes considered a diagnosis of exclusion as the name signifies that no cause is evident for this form of arthritis. Despite this JIA has some classical clinical features and many categories are defined based on the phenotype. Since there is no diagnostic test for JIA, diseases that can mimic JIA, including Primary Immunodeficiencies (PID) can sometimes be misdiagnosed as JIA. The clues to suspecting PIDs are early age of onset, presence of family history, increased susceptibility to infections, unusual features like urticaria, interstitial lung disease, sensorineural hearing loss and poor response to conventional therapy, amongst others. This review will highlight the basics of PIDs and will discuss PIDs that can present with arthritis and hence can be confused with JIA.
PubMed: 38851969
DOI: 10.1016/j.berh.2024.101960 -
Annals of the Rheumatic Diseases Jun 2024We report the safety, tolerability and efficacy of tofacitinib in patients with juvenile idiopathic arthritis (JIA) in an ongoing long-term extension (LTE) study.
OBJECTIVES
We report the safety, tolerability and efficacy of tofacitinib in patients with juvenile idiopathic arthritis (JIA) in an ongoing long-term extension (LTE) study.
METHODS
Patients (2-<18 years) with JIA who completed phase 1/3 index studies or discontinued for reasons excluding treatment-related serious adverse events (AEs) entered the LTE study and received tofacitinib 5 mg two times per day or equivalent weight-based doses. Safety outcomes included AEs, serious AEs and AEs of special interest. Efficacy outcomes included improvement since tofacitinib initiation per the JIA-American College of Rheumatology (ACR)70/90 criteria, JIA flare rate and disease activity measured by Juvenile Arthritis Disease Activity Score (JADAS)27, with inactive disease corresponding to JADAS ≤1.0.
RESULTS
Of 225 patients with JIA (median (range) duration of treatment, 41.6 (1-103) months), 201 (89.3%) had AEs; 34 (15.1%) had serious AEs. 10 patients developed serious infections; three had herpes zoster. Two patients newly developed uveitis. Among patients with polyarticular course JIA, JIA-ACR70/90 response rates were 60.0% (78 of 130) and 33.6% (47 of 140), respectively, at month 1, and generally improved over time. JIA flare events generally occurred in <5% of patients through to month 48. Observed mean (SE) JADAS27 was 22.0 (0.6) at baseline, 6.2 (0.7) at month 1 and 2.8 (0.5) at month 48, with inactive disease in 28.8% (36 of 125) of patients at month 1 and 46.8% (29 of 82) at month 48.
CONCLUSIONS
In this interim analysis of LTE study data in patients with JIA, safety findings were consistent with the known profile of tofacitinib, and efficacy was maintained up to month 48.
TRIAL REGISTRATION NUMBER
NCT01500551.
PubMed: 38849152
DOI: 10.1136/ard-2023-225094 -
Acta Paediatrica (Oslo, Norway : 1992) Jun 2024Higher adiposity and increased risk of cardiovascular diseases have been reported in juvenile idiopathic arthritis (JIA), but body composition measurements have produced...
Increased adiposity in juvenile idiopathic arthritis - A comparison of the bioelectrical impedance analysis and dual-energy X-ray absorptiometry for the assessment of body composition.
AIM
Higher adiposity and increased risk of cardiovascular diseases have been reported in juvenile idiopathic arthritis (JIA), but body composition measurements have produced inconsistent results. This controlled cross-sectional study assessed body composition with two methods to evaluate adiposity in children with JIA.
METHODS
We measured body composition by dual- energy X-ray absorptiometry (DXA) and bioelectrical impedance analysis (BIA) from 79 JIA-patients in two Finish university hospitals in 2017-2019. Their age- and sex-matched controls (n = 79) were selected from the Physical Activity and Nutrition in Children- study and through National Registry.
RESULTS
Body fat percentage measured by BIA was higher (mean, SD) in patients compared to controls (23.1 ± 9.3% vs. 20.1 ± 7.5%, p = 0.047). Also, using DXA, there was a tendency of higher body fat percentage in patients (27.1 ± 9.1% vs. 24.6 ± 8.6, p = 0.106). BIA and DXA showed strong correlation (r from 0.810 to 0.977) in all body composition variables.
CONCLUSION
Increased adiposity was observed in patients with JIA. Evaluation of body composition should be included in the multidisciplinary care of JIA to reduce the possible risk of cardiovascular diseases in adulthood. BIA could be a useful tool for assessing body composition due to its clinical availability and safety.
PubMed: 38847445
DOI: 10.1111/apa.17318 -
Scientific Reports Jun 2024While adaptive immune responses have been studied extensively in SLE (systemic lupus erythematosus), there is limited and contradictory evidence regarding the...
While adaptive immune responses have been studied extensively in SLE (systemic lupus erythematosus), there is limited and contradictory evidence regarding the contribution of natural killer (NK) cells to disease pathogenesis. There is even less evidence about the role of NK cells in the more severe phenotype with juvenile-onset (J)SLE. In this study, analysis of the phenotype and function of NK cells in a large cohort of JSLE patients demonstrated that total NK cells, as well as perforin and granzyme A expressing NK cell populations, were significantly diminished in JSLE patients compared to age- and sex-matched healthy controls. The reduction in NK cell frequency was associated with increased disease activity, and transcriptomic analysis of NK populations from active and low disease activity JSLE patients versus healthy controls confirmed that disease activity was the main driver of differential NK cell gene expression. Pathway analysis of differentially expressed genes revealed an upregulation of interferon-α responses and a downregulation of exocytosis in active disease compared to healthy controls. Further gene set enrichment analysis also demonstrated an overrepresentation of the apoptosis pathway in active disease. This points to increased propensity for apoptosis as a potential factor contributing to NK cell deficiency in JSLE.
Topics: Humans; Killer Cells, Natural; Lupus Erythematosus, Systemic; Female; Male; Adolescent; Child; Phenotype; Granzymes; Perforin; Apoptosis; Transcriptome; Gene Expression Profiling; Case-Control Studies
PubMed: 38844784
DOI: 10.1038/s41598-024-62325-3 -
Zeitschrift Fur Rheumatologie Jun 2024Coronavirus disease 2019 (COVID-19) has influenced the world over the last 3 years. Although the risk of a severe course is low in children, it can be influenced by...
Coronavirus disease 2019 (COVID-19) has influenced the world over the last 3 years. Although the risk of a severe course is low in children, it can be influenced by chronic rheumatic diseases or treatment with immunosuppressive drugs or immunomodulatory medication. The German register for biologics in pediatric rheumatology (BIKER) documented systematic data from 68 centers on the occurrence, presentation and outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections in children with rheumatic diseases. Between March 2020 and December 2022, a total of 927 SARS-CoV‑2 infections in 884 patients could be reported and analyzed in pediatric patients with rheumatic diseases. Juvenile idiopathic arthritis (JIA) was the most frequent diagnosis (716 infections) followed by genetic autoinflammation (103 infections), systemic autoimmune diseases (78 infections), idiopathic uveitis (25 infections) and vasculitis (5 infections). Only four patients were treated as inpatients. A 3.5-year-old female patient died during the first wave from encephalopathy and respiratory failure. The patient was treated with methotrexate (MTX) and steroids for systemic JIA. Genetic tests revealed a previously unknown congenital immune defect. No other patient had to be ventilated or treated on the intensive care unit. A case of uncomplicated pediatric inflammatory multisystem syndrome (PIMS) was registered in a patient with JIA treated with MTX. At the time of the infection over 60% of the patients were treated with standard disease modifying antirheumatic drugs (DMARD) and/or biologics. Although the patients treated with MTX showed a slightly longer duration of symptoms, the antirheumatic treatment did not appear to have a negative influence on the severity or outcome of the SARS-CoV‑2 infection.
PubMed: 38844689
DOI: 10.1007/s00393-024-01515-w