BACKGROUND: To our knowledge, limited information is available about the differences in the characteristics of rheumatoid factor (RF)-negative polyarticular juvenile idiopathic arthritis (JIA) throughout the world. This study was aimed to compare the demographic and clinical features of patients with RF-negative polyarthritis across the world. METHODS: Patients were part of a multinational sample included in a study aimed to investigate the prevalence of disease categories, treatment regimens, and disease status in patients from different geographical areas (EPOCA Study). All patients underwent a retrospective assessment, based on the review of clinical chart, and a cross-sectional evaluation, which included assessment of physician- and parent-reported outcomes and collection of ongoing medications. RESULTS: Among the 9081 patients enrolled in the EPOCA study, 2141 patients (23.6%) with RF-negative polyarthritis were included in the present analysis. The prevalence of RF-negative polyarthritis was highest in North America and lowest in Southeast Asia (12.7%). The age at disease onset was lower in Northern and Southern Europe, where the highest prevalence of uveitis was found. Uveitis was rare in Southeast Asia, Africa & Middle East and Latin America. Patients from Eastern Europe, Latin America and Africa and Middle East presented with the highest prevalence of active joints at the visit. The combination of early onset, ANA positivity, and uveitis was observed mainly in Southern Europe (39%). CONCLUSIONS: Our results confirm the wide heterogeneity of the clinical presentation and outcome of children with RF-negative polyarticular JIA throughout the world. In particular, relevant differences in the onset age were observed across geographic areas. The group of children with early onset polyarthritis, ANA positivity, and risk of uveitis is remarkably frequent in Southern Europe.
- MeSH
- Antirheumatic Agents therapeutic use MeSH
- Global Health MeSH
- Child MeSH
- Arthritis, Juvenile * epidemiology diagnosis MeSH
- Humans MeSH
- Adolescent MeSH
- Child, Preschool MeSH
- Prevalence MeSH
- Cross-Sectional Studies MeSH
- Retrospective Studies MeSH
- Rheumatoid Factor * blood MeSH
- Uveitis epidemiology diagnosis MeSH
- Age of Onset MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Adolescent MeSH
- Male MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Geographicals
- Europe MeSH
- MeSH
- Acetazolamide therapeutic use MeSH
- Biological Therapy methods adverse effects MeSH
- Adrenal Cortex Hormones therapeutic use MeSH
- Antibodies, Monoclonal, Humanized administration & dosage adverse effects MeSH
- Arthritis, Juvenile * drug therapy complications MeSH
- Cataract drug therapy complications therapy MeSH
- Humans MeSH
- Methotrexate adverse effects therapeutic use MeSH
- Therapeutic Occlusion methods MeSH
- Child, Preschool MeSH
- Disease Progression MeSH
- Risk MeSH
- Uveitis * diagnosis drug therapy complications pathology MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Child, Preschool MeSH
- Publication type
- Case Reports MeSH
- Keywords
- upadacitinib,
- MeSH
- Dermatitis, Atopic * drug therapy genetics immunology MeSH
- Biological Therapy MeSH
- Janus Kinase 1 antagonists & inhibitors MeSH
- Arthritis, Juvenile * drug therapy MeSH
- Humans MeSH
- Treatment Outcome MeSH
- Check Tag
- Humans MeSH
- Publication type
- Case Reports MeSH
OBJECTIVES: To assess, in spondyloarthritis (SpA), the discriminative value of the Outcome Measures in Rheumatology (OMERACT) ultrasound lesions of enthesitis and their associations with clinical features in this population. METHODS: In this multicentre study involving 20 rheumatology centres, clinical and ultrasound examinations of the lower limb large entheses were performed in 413 patients with SpA (axial SpA and psoriatic arthritis) and 282 disease controls (osteoarthritis and fibromyalgia). 'Active enthesitis' was defined as (1) power Doppler (PD) at the enthesis grade ≥1 plus entheseal thickening and/or hypoechoic areas, or (2) PD grade >1 (independent of the presence of entheseal thickening and/or hypoechoic areas). RESULTS: In the univariate analysis, all OMERACT lesions except enthesophytes/calcifications showed a significant association with SpA. PD (OR=8.77, 95% CI 4.40 to 19.20, p<0.001) and bone erosions (OR=4.75, 95% CI 2.43 to 10.10, p<0.001) retained this association in the multivariate analysis. Among the lower limb entheses, only the Achilles tendon was significantly associated with SpA (OR=1.93, 95% CI 1.30 to 2.88, p<0.001) in the multivariate analyses. Active enthesitis showed a significant association with SpA (OR=9.20, 95% CI 4.21 to 23.20, p<0.001), and unlike the individual OMERACT ultrasound lesions it was consistently associated with most clinical measures of SpA disease activity and severity in the regression analyses. CONCLUSIONS: This large multicentre study assessed the value of different ultrasound findings of enthesitis in SpA, identifying the most discriminative ultrasound lesions and entheseal sites for SpA. Ultrasound could differentiate between SpA-related enthesitis and other forms of entheseal pathology (ie, mechanical enthesitis), thus improving the assessment of entheseal involvement in SpA.
- MeSH
- Achilles Tendon diagnostic imaging pathology MeSH
- Adult MeSH
- Enthesopathy * diagnostic imaging MeSH
- Middle Aged MeSH
- Humans MeSH
- Arthritis, Psoriatic diagnostic imaging complications MeSH
- Spondylarthritis * diagnostic imaging complications MeSH
- Case-Control Studies MeSH
- Severity of Illness Index MeSH
- Ultrasonography, Doppler * methods MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
Podpořeno grantem MZ ČR – RVO (FNOl, 00098892) a IGA_LF_2023_037. Vitamin/hormon D představuje látku s významnou rolí v dětské revmatologii. Mimo dlouho známé protiinfekční efekty je studována jeho protizánětlivá funkce. V randomizované dvojitě zaslepené placebem kontrolované studii u adolescentů se systémovým lupus erythematodes (SLE) bylo zjištěno, že substituce vitaminu D vedla ke snížení aktivity nemoci a redukci únavy pacientů. V případě nesystémové juvenilní idiopatické artritidy (JIA) se jedná o biomarker, který společně s dalšími klinickými a laboratorními znaky hraje roli v predikci inaktivního onemocnění. Publikace studující periodickou horečku s aftózní stomatitidou, faryngitidou a krční lymfadenitidou (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis, PFAPA) přinesla poznatek o asociaci nízkých hladin vitaminu D s delším trváním atak a kratšími intervaly mezi atakami. Toto minireview shrnuje poznatky o roli vitaminu D v dětské revmatologii a upozorňuje na možnosti substituce vitaminu D v rutinní klinické praxi.
Vitamin/hormone D plays an important role in pediatric rheumatology. Besides its antiinfectious effects, antiinflammatory function is currently studied. Randomised double-blind placebo-controlled study in adolescents with systemic lupus erythematosus (SLE) revealed, that vitamin D supplementation led to decrease in disease activity and to reduction of fatigue. In nonsystemic juvenile idiopathic arthritis (JIA), vitamin D is a biomarker, which in combination with other clinical and laboratory markers plays a role in prediction of outcome (inactive disease). Study on periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) detected association of low vitamin D levels with longer episode duration and shorter time between episodes. This paper review an information on vitamin D role in pediatric rheumatology and highlights possibilities of vitamin D supplementation in routine clinical practice.
- MeSH
- Biomarkers MeSH
- Child MeSH
- Arthritis, Juvenile diagnosis MeSH
- Clinical Studies as Topic MeSH
- Humans MeSH
- Vitamin D Deficiency pathology MeSH
- Rheumatic Diseases * diagnosis drug therapy pathology MeSH
- Lupus Erythematosus, Systemic drug therapy MeSH
- Vitamin D * analysis administration & dosage MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Publication type
- Research Support, Non-U.S. Gov't MeSH
- Review MeSH
The balance between the tumor-necrosis factor α (TNFα) and type-I interferon (T1IFN) pathways is crucial for proper immune function. Dysregulation of either pathway can contribute to autoimmune diseases development. Even though TNFα blockade has shown promising results in various autoimmune diseases, the effect on the balance between TNFα and T1IFN is elusive. We used targeted anti-TNFα therapies in juvenile idiopathic arthritis (JIA) as an experimental approach to study the cross-regulation between TNFα and type-I IFN. We found that TNFα-rich environment affected viral defense through the attenuation of T1IFN responses and affected the phenotype and distribution of myeloid dendritic cells, which are engaged in early viral infections. Anti-TNFα therapy normalized the observed deviations in JIA patients. We hypothesize that the inadequate immune response caused by a high TNFα environment could be projected to more frequent or lengthy viral infections and possibly play a role in the process of JIA disease development.
- MeSH
- Dendritic Cells MeSH
- Phenotype MeSH
- Interferon Type I * MeSH
- Arthritis, Juvenile * drug therapy MeSH
- Humans MeSH
- Necrosis MeSH
- Tumor Necrosis Factor-alpha MeSH
- Virus Diseases * MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Keywords
- tofacitinib,
- MeSH
- Janus Kinase Inhibitors pharmacology therapeutic use MeSH
- Arthritis, Juvenile * drug therapy MeSH
- Humans MeSH
- Drug-Related Side Effects and Adverse Reactions MeSH
- Piperidines pharmacology therapeutic use MeSH
- Pyrimidines pharmacology therapeutic use MeSH
- Randomized Controlled Trials as Topic MeSH
- Check Tag
- Humans MeSH
- Keywords
- tofacitinib,
- MeSH
- Janus Kinase Inhibitors pharmacokinetics therapeutic use MeSH
- Protein Kinase Inhibitors pharmacokinetics therapeutic use MeSH
- Arthritis, Juvenile * drug therapy MeSH
- Clinical Trials as Topic MeSH
- Humans MeSH
- Drug-Related Side Effects and Adverse Reactions MeSH
- Piperidines therapeutic use MeSH
- Pyrimidines therapeutic use MeSH
- Pyrroles therapeutic use MeSH
- Body Height MeSH
- Check Tag
- Humans MeSH
Článek je věnován juvenilní spondyloartritidě (JSpA) (dříve nazývané Bechtěrevova nemoc), která je dle současné klasifikace řazena do podskupiny entezopatických (enthesis-related, ERA) juvenilních idiopatických artritid (JIA). Zmiňujeme diferenciální diagnostiku s důrazem na problematiku časné diagnózy a léčby, včetně možností příspěvku praktických lékařů pro děti a dorost (PLDD) v tomto procesu. Přinášíme kazuistiky dětských pacientů se spondylartritidou jako hlavním projevem ERA. Bolesti zad jako projev spondyloartritidy nebo sakroiliitidy mohou být prvním a jediným symptomem této podskupiny JIA. Někdy bývá přítomna i periferní artritida či entezitida. Část pacientů trpí uveitidou. Popisovaní pacienti dosáhli léčbou adalimumabem remise, nicméně jeden časně relaboval. U této podskupiny JIA je bohužel výskyt relapsů po ukončení léčby vysoký. Časná diagnóza je klíčem k úspěšné léčbě.
An article is focused on juvenile spondyloarthritis (JSpA) (previously Bechterev ́s disease) in childhood. The disease is included in enthesitis-related arthritis (ERA) subgroup of JIA based on currently used classification. Differential diagnostics with stress on early diagnosis and therapy is described. It is also reviewed how general practitioners might contribute. Case reports of children with spondyloarthritis as a main symptom of ERA are presented. A back pain might be the first, and only symptom of ERA. In some patients peripheral arthritis or enthesitis are found. A proportion of patients suffers from uveitis. Here described patients reached remission using adalimumab, however one relapsed early. Unfortunately, early relapses in ERA subgroup of JIA are frequent after therapy termination. An early diagnosis is a clue to successful therapy.
- MeSH
- Adalimumab therapeutic use MeSH
- Back Pain etiology classification MeSH
- Diagnosis, Differential MeSH
- Child MeSH
- Enthesopathy diagnosis drug therapy classification MeSH
- HLA-B27 Antigen analysis immunology MeSH
- Arthritis, Juvenile * diagnostic imaging diagnosis drug therapy classification MeSH
- Humans MeSH
- Methotrexate therapeutic use MeSH
- Adolescent MeSH
- Sacroiliitis diagnosis drug therapy MeSH
- Spondylarthritis diagnosis drug therapy classification MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Adolescent MeSH
- Male MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
- Research Support, Non-U.S. Gov't MeSH
