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Clinical and Experimental Rheumatology 2021Juvenile idiopathic arthritis (JIA) is the most common chronic joint disease in paediatric rheumatology. Over the last two decades, ultrasound (US) has emerged as a tool... (Review)
Review
Juvenile idiopathic arthritis (JIA) is the most common chronic joint disease in paediatric rheumatology. Over the last two decades, ultrasound (US) has emerged as a tool with the potential to enhance disease assessment and management of JIA. This imaging modality is safe and well tolerated by children and can be easily applied bedside in the clinical setting. Owing to the lack of published studies regarding the validity and reproducibility of US in JIA and the difficulties in interpreting images of children, US was initially perceived like an art rather than a science. In recent years, a great deal of efforts has been made in order to fill the gap of scientific knowledge on US between paediatric and adult rheumatology. This has yielded significant breakthroughs, such as the achievement of valuable information about the anatomical peculiarities of the growing skeleton on US, including internationally agreed definitions on B-mode and Doppler US of components for the normal joints, and the development of a standardised scanning protocol for US examination suitable for use in children. The precise role of US in JIA, however, is yet to be fully defined. Although further research regarding the use of US in joint inflammatory pathology in paediatrics is required, this imaging modality may well possess the necessary properties to pursue the best practice in the care of children with JIA in the near future. The present review provides information on the recent advances that have made the application of US increasingly promising for the management of JIA.
Topics: Arthritis, Juvenile; Child; Humans; Musculoskeletal System; Reproducibility of Results; Rheumatology; Ultrasonography
PubMed: 33769257
DOI: 10.55563/clinexprheumatol/o4f5rk -
Pediatric Rheumatology Online Journal Dec 2022The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans (CTPs) to compare treatment initiation strategies for systemic... (Observational Study)
Observational Study
First-line options for systemic juvenile idiopathic arthritis treatment: an observational study of Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans.
BACKGROUND
The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans (CTPs) to compare treatment initiation strategies for systemic juvenile idiopathic arthritis (sJIA). First-line options for sJIA treatment (FROST) was a prospective observational study to assess CTP outcomes using the CARRA Registry.
METHODS
Patients with new-onset sJIA were enrolled if they received initial treatment according to the biologic CTPs (IL-1 or IL-6 inhibitor) or non-biologic CTPs (glucocorticoid (GC) monotherapy or methotrexate). CTPs could be used with or without systemic GC. Primary outcome was achievement of clinical inactive disease (CID) at 9 months without current use of GC. Due to the small numbers of patients in the non-biologic CTPs, no statistical comparisons were made between the CTPs.
RESULTS
Seventy-three patients were enrolled: 63 (86%) in the biologic CTPs and 10 (14%) in the non-biologic CTPs. CTP choice appeared to be strongly influenced by physician preference. During the first month of follow-up, oral GC use was observed in 54% of biologic CTP patients and 90% of non-biologic CTPs patients. Five (50%) non-biologic CTP patients subsequently received biologics within 4 months of follow-up. Overall, 30/53 (57%) of patients achieved CID at 9 months without current GC use.
CONCLUSION
Nearly all patients received treatment with biologics during the study period, and 46% of biologic CTP patients did not receive oral GC within the first month of treatment. The majority of patients had favorable short-term clinical outcomes. Increased use of biologics and decreased use of GC may lead to improved outcomes in sJIA.
Topics: Humans; Arthritis, Juvenile; Research Design
PubMed: 36482434
DOI: 10.1186/s12969-022-00768-6 -
Turkish Journal of Ophthalmology Dec 2021In this study, we aimed to describe the demographic and clinical findings of children with uveitis at a tertiary pediatric rheumatology and ophthalmology center.
OBJECTIVES
In this study, we aimed to describe the demographic and clinical findings of children with uveitis at a tertiary pediatric rheumatology and ophthalmology center.
MATERIALS AND METHODS
A retrospective cross-sectional study was conducted with 46 patients who were diagnosed with uveitis before the age of 16 years and were followed regularly for at least 6 months between January 2013 and June 2019. Demographic data, uveitis characteristics, underlying diseases, systemic treatment modalities, drug side effects, complications, and surgical intervention were evaluated.
RESULTS
Eighty-three eyes of 46 patients were included in the study. The mean age at diagnosis of uveitis was 9.2±4.5 (1.6-15.6) years, and the mean uveitis follow-up period was 54±41 (6-191) months. Twenty-one patients (45.7%) had uveitis associated with rheumatologic diseases. Juvenile idiopathic arthritis was the most common disease (23.9%). Visual acuity was categorized as moderately impaired in 6 eyes (7.2%), severely impaired in 4 eyes (4.8%), and blindness in 1 eye (1.2%). Methotrexate (87%) was the most frequently used systemic immunosuppressive agent in treatment. Adalimumab (73.9%) was added to treatment in resistant cases. Thirty-five patients (76.1%) had complications in at least 1 eye secondary to uveitis or uveitis treatment. Posterior synechiae (11 eyes, 13.2%) was the most common complication during treatment.
CONCLUSION
In order to preserve visual acuity, pediatric uveitis should be recognized early and especially persistent/chronic cases should be started on effective systemic treatment immediately.
Topics: Adolescent; Arthritis, Juvenile; Child; Cross-Sectional Studies; Follow-Up Studies; Humans; Retrospective Studies; Uveitis
PubMed: 34963262
DOI: 10.4274/tjo.galenos.2021.38585 -
BMC Oral Health Aug 2021Optimal utilization of dental caries data is crucial in epidemiological research of individuals with juvenile idiopathic arthritis (JIA). The aims were to: explore...
BACKGROUND
Optimal utilization of dental caries data is crucial in epidemiological research of individuals with juvenile idiopathic arthritis (JIA). The aims were to: explore whether caries is more prevalent among children and adolescents with JIA compared to controls; examine presence of caries according to JIA group, socio-behavioral and intraoral characteristics, and the extent to which surface-specific caries varies between and within individuals; assess whether surface-specific caries varies according to JIA group and dentition; and investigate whether disease-specific clinical features of JIA are associated with presence of caries.
METHODS
In this comparative cross-sectional study, calibrated dentists examined index teeth (primary 2. molars, 1. permanent molars) of 4-16-year-olds with JIA (n = 219) and matched controls (n = 224), using a detailed caries diagnosis system (including enamel caries). JIA-specific characteristics were assessed by pediatric rheumatologists and socio-behavioral information collected by questionnaires. Multilevel mixed-effect logistic regressions reporting odds ratios (OR) with 95% confidence interval (CI) were applied (caries at surface level as outcome variable). Potential confounders were adjusted for, and the effect of dependency of surface-specific caries data was estimated by calculating intra-class correlation coefficients (ICC).
RESULTS
At individual level, no significant difference in caries prevalence was found between individuals with JIA and controls, regardless of inclusion of enamel caries. Proportion of enamel lesions exceeded dentine lesions. JIA was not associated with presence of caries, but in both groups, low maternal educational level was associated with presence of caries (OR: 2.07, 95% CI: 1.24-3.46). Occlusal and mesial surfaces, compared to buccal surfaces, had generally higher OR according to presence of caries than distal and lingual surfaces (ICC = 0.56). Surface-specific caries in the permanent dentition differed significantly according to group affiliation. Some JIA disease-specific variables were suggested to associate with presence of caries.
CONCLUSIONS
No overall difference in caries prevalence between individuals with JIA and controls was observed, but for both groups, low maternal educational level and tooth surface associated with presence of caries. Associations between JIA disease-specific variables and presence of caries cannot be excluded. Due to predominance of enamel lesions, the potential of preventative dental strategies is considerable.
Topics: Adolescent; Arthritis, Juvenile; Child; Cross-Sectional Studies; Dental Caries; Dentition, Permanent; Humans; Multilevel Analysis; Tooth, Deciduous
PubMed: 34433437
DOI: 10.1186/s12903-021-01758-y -
Medicina (Kaunas, Lithuania) Apr 2023: Systemic juvenile idiopathic arthritis (sJIA) is a distinctive JIA subtype with mostly nonspecific systemic clinical features, which can be a diagnostic challenge....
: Systemic juvenile idiopathic arthritis (sJIA) is a distinctive JIA subtype with mostly nonspecific systemic clinical features, which can be a diagnostic challenge. This study aimed to analyze our experience with sJIA in Latvia for twelve years: assessing clinical and epidemiological characteristics, the efficacy of therapy, and disease outcomes, including the development of macrophage activation syndrome (MAS). : This is a descriptive study in which we conducted a retrospective case review of all patients with sJIA diagnosis admitted to the only pediatric tertiary centre in Latvia during the period 2009-2020. sJIA was diagnosed in 35 patients with a mean annual incidence rate of 0.85 patients per 100,000 children. Major clinical signs at the first visit were: fever, rash, arthritis, and lymphadenopathy. Almost half of the patients, 48.5%, had a monocyclic disease course, and only 20% of patients had persistent disease. MAS developed in 28.6% of patients. Biological therapy was administered to 48.6% of patients, mostly by tocilizumab, which induced remission in 75% after one year, and in 81.2% after two years without any serious therapy-related complications. In our study, none of the patients had interstitial lung disease, drug reaction with eosinophilia and systemic symptoms (DRESS)-like syndrome, or fatal disease. The incidence and clinical characteristics of sJIA correlate with the literature findings, although MAS was more common than described in other studies. There is a tendency for the persistent disease to decrease with the use of biological therapy. Tocilizumab is an efficient choice of treatment with a good safety profile.
Topics: Child; Humans; Arthritis, Juvenile; Macrophage Activation Syndrome; Retrospective Studies; Latvia; Fever
PubMed: 37109756
DOI: 10.3390/medicina59040798 -
Acta Ophthalmologica Jun 2023The purpose of this perspective was to shed light on screening of uveitis among Nordic children with juvenile idiopathic arthritis (JIA).
PURPOSE
The purpose of this perspective was to shed light on screening of uveitis among Nordic children with juvenile idiopathic arthritis (JIA).
METHODS
A literature search was conducted to review predictors of JIA-uveitis and previous JIA-uveitis screening recommendations.
RESULTS
Predictors of uveitis in JIA are younger age and positive antinuclear antibody titre at onset of JIA, specific subtypes of JIA (extended and persistent oligoarthritis, rheumatoid factor negative polyarthritis and psoriatic arthritis) and short duration of JIA. Methotrexate and monoclonal tumour necrosis factor (TNF) inhibitor treatment reduce the risk JIA-uveitis.
CONCLUSION
Children with all of the above risk factors should be screened frequently but if they receive TNF inhibitor or methotrexate therapy, they may be screened less frequently. Children with none of the risk factors do not benefit from long-term screening for uveitis. A guideline for intervals and overall length of screening was prepared considering currently known risk factors for JIA-uveitis, the Nordic population and previous guidelines.
Topics: Child; Humans; Arthritis, Juvenile; Methotrexate; Uveitis; Risk Factors; Time Factors
PubMed: 36458735
DOI: 10.1111/aos.15299 -
Reumatologia Clinica 2023To describe the methodology, objectives, and initial data of the registry of young adult patients diagnosed with Juvenile Idiopathic Arthritis (JIA), JUVENSER. The main...
OBJECTIVES
To describe the methodology, objectives, and initial data of the registry of young adult patients diagnosed with Juvenile Idiopathic Arthritis (JIA), JUVENSER. The main objective of the project is to know the sociodemographic and clinical characteristics, and disease activity of patients with JIA reaching the transition to adulthood.
MATERIAL AND METHOD
Longitudinal, prospective, multicentre study, including patients between 16 and 25 years old, with a diagnosis of JIA in any of its categories. The main objective is to determine the characteristics and activity of JIA in the young adult. It includes sociodemographic variables, clinical variables, disease activity and joint damage rates, data on the use of health resources, and treatments used. The total duration of the project will be 3 years. A cohort of 534 young adult patients was obtained.
CONCLUSIONS
The JUVENSER registry will constitute a cohort of young adults with JIA, which will allow the evaluation of the clinical characteristics and response to treatment of patients with disease onset in childhood, moving to adult clinics.
Topics: Humans; Young Adult; Adolescent; Adult; Arthritis, Juvenile; Antirheumatic Agents; Prospective Studies; Registries
PubMed: 37258400
DOI: 10.1016/j.reumae.2023.01.003 -
Arthritis Care & Research Feb 2023The aim of this work was to provide evidence of validity and reliability for 4 parent/child-reported outcome measures included in the Outcome Measures in Rheumatology...
OBJECTIVE
The aim of this work was to provide evidence of validity and reliability for 4 parent/child-reported outcome measures included in the Outcome Measures in Rheumatology juvenile idiopathic arthritis core domain set: the evaluation of the child's pain and level of disease activity, the assessment of morning stiffness duration, and an active joint count for proxy/self-assessment.
METHODS
Patients were included in the multinational study Epidemiology Treatment and Outcome of Childhood Arthritis. Criterion validity was assessed by examining the correlation of the 4 tested measures with physician measures and the clinical Juvenile Arthritis Disease Activity Score in 10 joints (cJADAS10) in the whole sample and after grouping patients by International League of Associations for Rheumatology (ILAR) category, geographic area, and education level. Reliability was assessed comparing 2 visits 7-14 days apart with intraclass correlation coefficients (ICCs).
RESULTS
A total of 8,643 parents and 6,060 patients had all the evaluations available. Correlations of tested measures were moderate (0.4-0.7) with physician-reported measures. The level of correlation with the cJADAS10 remained stable after grouping patients by ILAR category, geographic areas, and level of education of the parent filling the questionnaire. In 442 parents and 344 children, ICCs ranged between 0.79 and 0.87 for parents and 0.81 and 0.88 for children.
CONCLUSION
The 4 tested parent/child-reported outcomes showed good criterion validity and excellent reliability. These tools can be considered for remote patient assessment, when in-person evaluation might not be possible.
Topics: Humans; Arthritis, Juvenile; Reproducibility of Results; Parents; Surveys and Questionnaires; Patient Reported Outcome Measures; Quality of Life; Psychometrics; Health Status; Rheumatology; Disability Evaluation
PubMed: 35015379
DOI: 10.1002/acr.24855 -
Arthritis Care & Research Mar 2024Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is a life-threatening disease complication. Key questions remain regarding clinical course and...
OBJECTIVE
Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is a life-threatening disease complication. Key questions remain regarding clinical course and optimal treatment approaches. The objectives of the study were to detail management strategies after SJIA-LD detection, characterize overall disease courses, and measure long-term outcomes.
METHODS
This was a prospective cohort study. Clinical data were abstracted from the electronic medical record, including current clinical status and changes since diagnosis. Serum biomarkers were determined and correlated with presence of LD.
RESULTS
We enrolled 41 patients with SJIA-LD, 85% with at least one episode of macrophage activation syndrome and 41% with adverse reactions to a biologic. Although 93% of patients were alive at last follow-up (median 2.9 years), 37% progressed to requiring chronic oxygen or other ventilator support, and 65% of patients had abnormal overnight oximetry studies, which changed over time. Eighty-four percent of patients carried the HLA-DRB1*15 haplotype, significantly more than patients without LD. Patients with SJIA-LD also showed markedly elevated serum interleukin-18 (IL-18), variable C-X-C motif chemokine ligand 9 (CXCL9), and significantly elevated matrix metalloproteinase 7. Treatment strategies showed variable use of anti-IL-1/6 biologics and addition of other immunomodulatory treatments and lung-directed therapies. We found a broad range of current clinical status independent of time from diagnosis or continued biologic treatment. Multidomain measures of change showed imaging features were the least likely to improve with time.
CONCLUSION
Patients with SJIA-LD had highly varied courses, with lower mortality than previously reported but frequent hypoxia and requirement for respiratory support. Treatment strategies were highly varied, highlighting an urgent need for focused clinical trials.
Topics: Child; Humans; Arthritis, Juvenile; Prospective Studies; Lung; Lung Diseases; Macrophage Activation Syndrome; Disease Progression
PubMed: 37691306
DOI: 10.1002/acr.25234 -
Pediatric Rheumatology Online Journal Dec 2021This study aimed to perform an immunoprofiling of systemic juvenile idiopathic arthritis (sJIA) in order to define biomarkers of clinical use as well as reveal new...
BACKGROUND
This study aimed to perform an immunoprofiling of systemic juvenile idiopathic arthritis (sJIA) in order to define biomarkers of clinical use as well as reveal new immune mechanisms.
METHODS
Immunoprofiling of plasma samples from a clinically well-described cohort consisting of 21 sJIA patients as well as 60 age and sex matched healthy controls, was performed by a highly sensitive proteomic immunoassay. Based on the biomarkers being significantly up- or down-regulated in cross-sectional and paired analysis, related canonical pathways and cellular functions were explored by Ingenuity Pathway Analysis (IPA).
RESULTS
The well-studied sJIA biomarkers, IL6, IL18 and S100A12, were confirmed to be increased during active sJIA as compared to healthy controls. IL18 was the only factor found to be increased during inactive sJIA as compared to healthy controls. Novel factors, including CASP8, CCL23, CD6, CXCL1, CXCL11, CXCL5, EIF4EBP1, KITLG, MMP1, OSM, SIRT2, SULT1A1 and TNFSF11, were found to be differentially expressed in active and/or inactive sJIA and healthy controls. No significant pathway activation could be predicted based on the limited factor input to the IPA. High Mobility Group Box 1 (HMGB1), a damage associated molecular pattern being involved in a series of inflammatory diseases, was determined to be higher in active sJIA than inactive sJIA.
CONCLUSIONS
We could identify a novel set of biomarkers distinguishing active sJIA from inactive sJIA or healthy controls. Our findings enable a better understanding of the immune mechanisms active in sJIA and aid the development of future diagnostic and therapeutic strategies.
Topics: Adolescent; Arthritis, Juvenile; Biomarkers; Child; Child, Preschool; Cross-Sectional Studies; Female; Humans; Male; Proteomics
PubMed: 34963488
DOI: 10.1186/s12969-021-00660-9