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Juvenile Idiopathic Arthritis (juvenile + idiopathic_arthritis)
Kinds of Juvenile Idiopathic Arthritis Selected AbstractsPseudoporphyria and Nonsteroidal Antiinflammatory Agents in Children with Juvenile Idiopathic ArthritisPEDIATRIC DERMATOLOGY, Issue 6 2000Bernadette De Silva M.R.C.P. Nonsteroidal antiinflammatory drugs (NSAIDs) are implicated in the etiology of this condition. In a 1-year prospective study of children attending the pediatric rheumatology clinic in Edinburgh we found a prevalence of pseudoporphyria of 10.9% in children taking NSAIDs for juvenile idiopathic arthritis. Naproxen was the most commonly implicated NSAID, independent of dosage. Blue/gray eye color was an independent risk factor for the development of pseudoporphyria. We would advise caution in prescribing naproxen in these children to prevent disfiguring facial scarring. [source] Associations between the American College of Rheumatology pediatric response measures and the continuous measures of disease activity used in adult rheumatoid arthritis: A secondary analysis of clinical trial data from children with Polyarticular-Course Juvenile Idiopathic ArthritisARTHRITIS & RHEUMATISM, Issue 12 2009Sarah Ringold Objective To measure associations between the American College of Rheumatology (ACR) pediatric criteria for improvement and the continuous measures of disease activity used for rheumatoid arthritis in adult patients with polyarticular-course juvenile idiopathic arthritis (JIA). Methods In this retrospective analysis of 2 etanercept trials, disease activity was calculated at baseline, 3 months, and 6 months using the Disease Activity Score (DAS), the DAS based on 28 joints (DAS28), the Simplified Disease Activity Index (SDAI), and the Clinical Disease Activity Index (CDAI). The ACR pediatric response and the European League Against Rheumatism (EULAR) response were also determined for the 3-month and 6-month evaluations. Data were analyzed in 94 patients with JIA independent of the treatment arm. Correlation coefficients between measures were calculated for each visit. The areas under the receiver operating characteristic curve (AUC of ROC) were calculated to assess the discriminative properties of the scores for the ACR pediatric response measures. Results The mean DAS, DAS28, CDAI score, and SDAI score were 3.7, 4.7, 30.8, and 36.4, respectively, at baseline, corresponding to high levels of disease activity (CDAI/SDAI) or moderate levels of disease activity (DAS/DAS28). At 3 months, the mean scores corresponded to low (DAS/DAS28) or moderate (CDAI/SDAI) disease activity. At 6 months, the mean scores corresponded to low disease activity (DAS/DAS28/CDAI) or moderate disease activity (SDAI). Most children met the criteria for a good or moderate EULAR response at 3 months and 6 months. The correlation between continuous outcome measures and each pediatric core set component was moderate to very good. The AUC of ROC values for each measure were high (range 0.76,0.98). Conclusion Good correlation and discriminative abilities were seen between the DAS, DAS28, CDAI, and SDAI for the ACR pediatric criteria for improvement. These disease activity measures may be useful for research and clinical care in polyarticular-course JIA. [source] Contribution of OCT to evaluate macular disease in JIA associated uveitisACTA OPHTHALMOLOGICA, Issue 2008B BODAGHI Purpose To examine the frequency and characteristics of macular lesions observed in Juvenile Idiopathic Arthritis (JIA) uveitis, using Optical Coherence Tomography (OCT). Methods In this cross-sectional study, 38 consecutive patients were recruited from a tertiary referral center in uveitis. All eyes with JIA uveitis underwent complete ophthalmic examination including OCT 3. Exclusion criterion was the inability to obtain OCT scans. Flare and visual acuity were also analyzed by using linear regression. Results We analyzed foveal thickness (FT) and central foveal thickness (CFT) using the software mapping, to describe macular lesions in 61 eyes. Maculopathy was observed in 51 eyes (84%), compared to 12% in the literature (P<0.0001) and comprised four types: perifoveolar thickening in 45 eyes (74%), macular edema in 29 eyes (48%), foveal detachment in 11 eyes (18%), and atrophic changes in 6 eyes (10%). Only 4 eyes did not demonstrate any lesion. Conclusion Among children with JIA-uveitis, macular involvement is frequent, and characterized by perifoveolar thickening and serous retinal detachment. OCT is a non-invasive instrument. It may easily show this maculopathy, which could impair visual function, and conditioned a therapeutic intensification. [source] State of the art: Juvenile idiopathic arthritisINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 1 2002Prudence J. Manners Abstract Juvenile idiopathic arthritis (JIA) is the most common of the autoimmune musculoskeletal conditions in children. As awareness of this condition increases, so too does the apparent prevalence reported from countries around the world, suggesting that significant numbers of children with JIA have previously gone undiagnosed. Prevalence varies with race and possibly geography. However, JIA should no longer be considered as a rare condition. In the past decade, there have been definite advances in understanding the pathogenesis of JIA, and there have been parallel advances in therapies. There have been fairly modest advances in the classification of JIA, but there is at least heightened international debate on the issue, which will lead to progress. It is estimated that nearly one-third of children with this condition continue into adult life with inflammatory joint disease, and therefore the burden of disease remains significant. [source] Juvenile idiopathic arthritis profile in Turkish childrenPEDIATRICS INTERNATIONAL, Issue 2 2008Mustafa Yilmaz Abstract Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders. Publications from different countries point to differences in the disease manifestation of JIA among different populations. The aim of the present paper was to evaluate the clinical and laboratory features of JIA in Turkish children. Methods: A total of 196 JIA patients who fulfilled International League of Associations for Rheumatology (ILAR) diagnostic criteria were included in this retrospective study. The data collected were age, gender, age at disease onset and at diagnosis, and follow-up duration. Antinuclear antibody (ANA), rheumatoid factor (RF), and human leukocyte antigen B-27 were evaluated for each patient. Results: There were 102 boys and 94 girls with a mean duration of disease of 4.1 years. The mean age at the first visit was 8.8 years, and the mean age at onset of disease was 6.8 years (range, 8 months,15 years). Polyarticular JIA was the most frequent onset type (37.2%). Other subtypes included oligoarthritis (34.2%), systemic arthritis (15.3%), psoriatic arthritis (1%), enthesitis-related arthritis (9.7%), and other arthritis (2.2%). ANA was positive in 28 patients (14.2%). Chronic uveitis occurred in two patients with oligoarthritis; and two patients with enthesitis-related arthritis had acute uveitis. Three patients (1.4%) developed amyloidosis. Conclusion: Compared to reports from Western countries, remarkably different features of JIA were found in Turkish children, which included higher frequency of polyarticular JIA, higher prevalence among boys, lower rate of ANA positivity and uveitis. Further studies are required to understand how genetic and environmental differences affect JIA expression. [source] Juvenile idiopathic arthritis and HLA Class I and Class II interactions and age-at-onset effectsARTHRITIS & RHEUMATISM, Issue 6 2010Jill A. Hollenbach Objective The aim of this study was to quantitate risk and to examine heterogeneity for HLA at high resolution in patients with the most common subtypes of juvenile idiopathic arthritis (JIA), IgM rheumatoid factor,negative polyarticular JIA and oligoarticular JIA. Use of 4-digit comprehensive HLA typing enabled great precision, and a large cohort allowed for consideration of both age at disease onset and disease subtype. Methods Polymerase chain reaction,based high-resolution HLA typing for class I and class II loci was accomplished for 820 patients with JIA and 273 control subjects. Specific HLA epitopes, potential interactions of alleles at specific loci and between loci (accounting for linkage disequilibrium and haplotypic associations), and an assessment of the current International League of Associations for Rheumatology classification criteria were considered. Results An HLA,DRB1/DQB1 effect was shown to be exclusively attributable to DRB1 and was similar between patients with oligoarticular JIA and a younger subgroup of patients with polyarticular JIA. Furthermore, patients with polyarticular JIA showed age-specific related effects, with disease susceptibility in the group older than age 6 years limited to an effect of the HLA,DRB1*08 haplotype, which is markedly different from the additional susceptibility haplotypes, HLA,DRB1*1103/1104, found in the group with oligoarticular JIA and the group of younger patients with polyarticular JIA. Also in contrast to findings for oligoarticular JIA, patients with polyarticular arthritis had no evidence of an HLA class I effect. Markers associated with a reduced risk of disease included DRB1*1501, DRB1*0401, and DRB1*0701. DRB1*1501 was shown to reduce risk across the whole cohort, whereas DRB1*0401 and DRB1*0701 were protective for selected JIA subtypes. Surprisingly, the disease predisposition mediated by DPB1*0201 in individuals without any disease-predisposing DRB1 alleles was great enough to overcome even the very strong protective effect observed for DRB1*1501. Conclusion Inherited HLA factors in JIA show similarities overall as well as differences between JIA subtypes. [source] Synovial fluid proteins differentiate between the subtypes of juvenile idiopathic arthritisARTHRITIS & RHEUMATISM, Issue 6 2010Margalit E. Rosenkranz Objective Juvenile idiopathic arthritis (JIA) is a heterogeneous group of inflammatory diseases, and no clinically useful prognostic markers to predict disease outcome in children with JIA are currently available. Synovial fluid likely reflects the proteins present in the inflamed synovium. The purpose of this study was to delineate the synovial fluid proteome and determine whether protein expression differs in the different subtypes of JIA. Methods Synovial fluid samples obtained from children with oligoarticular JIA, polyarticular JIA, or systemic JIA were compared. Two-dimensional gel electrophoresis for protein separation and matrix-assisted laser desorption ionization,time-of-flight mass spectrometry and quadripole time-of-flight mass spectrometry for protein identification were used for this study. Synovial fluid cells were analyzed by polymerase chain reaction (PCR) for the presence of haptoglobin messenger RNA (mRNA). Results The synovial fluid proteome of the samples was delineated. The majority of proteins showed overexpression in JIA synovial fluid as compared with noninflammatory control samples. There were 24 statistically significantly differentially expressed spots (>2-fold change; P < 0.05) between the subtypes of JIA. PCR analysis revealed haptoglobin mRNA, suggesting that haptoglobin is locally produced in an inflamed joint in JIA. Conclusion Despite the similar histologic appearance of inflamed joints in patients with different subtypes of JIA, there are differences in protein expression according to the subtype of JIA. Haptoglobin is differentially expressed between the subtypes of JIA and is locally produced in an inflamed joint in JIA. Haptoglobin and other differentially expressed proteins may be potential biomarkers in JIA. [source] Increased frequency of extremely skewed X chromosome inactivation in juvenile idiopathic arthritisARTHRITIS & RHEUMATISM, Issue 11 2009Elif Uz Objective Juvenile idiopathic arthritis (JIA) is a childhood rheumatic disease of unknown etiology. Two subgroups of JIA, i.e., oligoarticular and polyarticular, are thought to have an autoimmune component, and show a higher female:male ratio. Skewed X chromosome inactivation (XCI) has previously been shown to be associated with scleroderma and autoimmune thyroiditis, 2 autoimmune disorders occurring predominantly in females. This study was undertaken to extend the analysis to the pediatric age group and to determine the XCI profiles of patients with JIA. Methods A polymorphic repeat in the androgen receptor gene was genotyped to determine XCI status in 81 female patients with JIA (21 with polyarticular disease and 60 with oligoarticular disease) and 211 healthy female controls. DNA obtained from venous blood samples was used for this analysis. Results Informative data were obtained on 62 JIA patients and 155 controls. Skewed XCI was observed in 14 patients (22.6%) and 11 controls (7.1%) (P = 0.0036), and extreme skewing was apparent in 8 patients (12.9%) and 2 controls (1.3%) (P = 0.0008). Conclusion Our findings in the present study indicate that skewed XCI may be a risk factor for the occurrence of autoimmune disorders, including JIA. [source] Association of the autoimmunity locus 4q27 with juvenile idiopathic arthritisARTHRITIS & RHEUMATISM, Issue 3 2009H. M. Albers Objective Juvenile idiopathic arthritis (JIA) is characterized by chronic arthritis and an autoimmune etiology. In several autoimmune diseases, including rheumatoid arthritis (RA), an association with the 4q27 locus has been reported. We undertook this study to investigate the possible role of the 4q27 locus in JIA. Methods A case,control association study was conducted, with a total of 655 Caucasian JIA patients and 791 healthy controls divided into 2 independent sample sets. The rs6822844 marker in the 4q27 locus was genotyped. Results In the first and larger sample set, a 5% decrease in T allele frequency was observed in patients compared with controls (allelic odds ratio [OR] 0.72 [95% confidence interval 0.55,0.95], P = 0.019), and in the second set, a 3% decrease was observed (allelic OR 0.81 [95% confidence interval 0.61,1.09], P = 0.169). The combined data set generated an OR of 0.76 (95% confidence interval 0.62,0.93, P = 7.08 × 10,3). When the different JIA subtypes were analyzed individually, significant decreases were seen in the subtypes with a polyarticular course of disease (extended oligoarthritis [P = 0.019] and rheumatoid factor,negative polyarthritis [P = 0.038]). Conclusion Our findings suggest that the 4q27 locus, previously reported to be associated with RA, type 1 diabetes mellitus, celiac disease, and psoriatic arthritis, is also associated with susceptibility to JIA. [source] Identification of a novel susceptibility locus for juvenile idiopathic arthritis by genome-wide association analysisARTHRITIS & RHEUMATISM, Issue 1 2009Anne Hinks Objective Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease of childhood. Two well-established genetic factors known to contribute to JIA susceptibility, HLA and PTPN22, account for less than half of the genetic susceptibility to disease; therefore, additional genetic factors have yet to be identified. The purpose of this study was to perform a systematic search of the genome to identify novel susceptibility loci for JIA. Methods A genome-wide association study using Affymetrix GeneChip 100K arrays was performed in a discovery cohort (279 cases and 184 controls). Single-nucleotide polymorphisms (SNPs) showing the most significant differences between cases and controls were then genotyped in a validation sample of cases (n = 321) and controls, combined with control data from the 1958 UK birth cohort (n = 2,024). In one region in which association was confirmed, fine-mapping was performed (654 cases and 1,847 controls). Results Of the 112 SNPs that were significantly associated with JIA in the discovery cohort, 6 SNPs were associated with JIA in the independent validation cohort. The most strongly associated SNP mapped to the HLA region, while the second strongest association was with a SNP within the VTCN1 gene. Fine-mapping of that gene was performed, and 10 SNPs were found to be associated with JIA. Conclusion This study is the first to successfully apply a SNP-based genome-wide association approach to the investigation of JIA. The replicated association with markers in the VTCN1 gene defined an additional susceptibility locus for JIA and implicates a novel pathway in the pathogenesis of this chronic disease of childhood. [source] Premature aging of the immune system in children with juvenile idiopathic arthritisARTHRITIS & RHEUMATISM, Issue 7 2008Martina Prelog Objective Juvenile idiopathic arthritis (JIA) is an autoimmune disease of the young. The pathogenesis is not completely understood. Premature aging, associated thymic involution, and compensatory autoproliferation could play important roles in the pathogenesis of autoimmunity. We undertook this study to determine whether patients with JIA demonstrate premature immunosenescence. Methods To test this hypothesis, we measured 3 indicators of aging: the percentages and total counts of peripheral blood naive T cells, the frequency of T cell receptor excision circles (TRECs) in naive T cells, and telomeric erosion and Ki-67 expression as estimates of the replicative history of homeostatic proliferation. Results JIA patients showed an accelerated loss of CD4+,CD45RA+,CD62L+ naive T cells with advancing age and a compensatory increase in the number of CD4+,CD45RO+ memory T cells. JIA patients demonstrated a significantly decreased frequency of TRECs in CD4+,CD45RA+ naive T cells compared with age-matched healthy donors (P = 0.002). TREC numbers correlated with age only in healthy donors (P = 0.0001). Telomeric erosion in CD4+,CD45RA+ naive T cells was increased in JIA patients (P = 0.01). The percentages of Ki-67,positive CD4+,CD45RA+ naive T cells were increased in JIA patients (P = 0.001) and correlated with disease duration (P = 0.003), which was also an independent factor contributing to telomeric erosion (P = 0.04). Conclusion Our findings suggest that age-inappropriate T cell senescence and disturbed T cell homeostasis may contribute to the development of JIA. In patients with JIA, dysfunction in the ability to reconstitute the T cell compartment should be considered when exploring new therapeutic strategies. [source] Intraoral condition in children with juvenile idiopathic arthritis compared to controlsINTERNATIONAL JOURNAL OF PAEDIATRIC DENTISTRY, Issue 6 2008EVA LEKSELL Aims. The aims of this study were to compare the periodontal conditions in children and adolescents with juvenile idiopathic arthritis (JIA) in comparison to age-matched healthy individuals, and to describe intraoral health in relation to medical assessments. Design. Forty-one JIA patients, 10,19 years old, were compared to 41 controls. Plaque, calculus, probing depth, bleeding on probing, clinical attachment loss, as well as mucosal lesions were registered. Marginal bone level was recorded on radiographs. A questionnaire was included. Data were analysed with chi-squared test, Fisher's exact test, and Mann,Whitney U -test (P < 0.05). Results. The JIA patients reported pain from jaws (P = 0.001), hands (P = 0.001), and oral ulcers (P = 0.015) more often than controls. They avoided certain types of food because of oral ulcers (P = 0.037). The frequencies of sites with plaque (32% vs. 19%, P = 0.013), calculus (11% vs. 5%, 5 = 0.034), bleeding on probing (26% vs. 14%, P < 0.01), and probing depth 2 mm (32% vs. 2%, P < 0.001) were higher among JIA patients. No sites with attachment loss or reduced marginal bone level were observed. Conclusions. These obtained results are probably because of joint pain, making it difficult to perform oral hygiene as well as the use of medication and general disease activity. [source] Failure to distinguish systemic-onset juvenile idiopathic arthritis from incomplete Kawasaki disease in an infantJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 10 2007Haruki Komatsu Abstract: In an infant, an initial diagnosis of incomplete Kawasaki disease was made according to the American Heart Association guidelines. However, the diagnosis of systemic-onset juvenile idiopathic arthritis was established later. Physicians need to recognize that systemic-onset juvenile idiopathic arthritis can be mistaken for incomplete Kawasaki disease, even when the guidelines are used. [source] A survey of foot problems in juvenile idiopathic arthritisMUSCULOSKELETAL CARE, Issue 4 2008G. Hendry BSc(Hons) Abstract Background:,Evidence suggests that foot problems are common in juvenile idiopathic arthritis (JIA), with prevalence estimates over 90%. The aim of this survey was to describe foot-related impairment and disability associated with JIA and foot-care provision in patients managed under modern treatment paradigms, including disease-modifying anti-rheumatic drugs (DMARDs) and biologic therapies. Methods:,The Juvenile Arthritis Foot Disability Index (JAFI), Child Health Assessment Questionnaire (CHAQ), and pain visual analogue scale (VAS) were recorded in 30 consecutive established JIA patients attending routine outpatient clinics. Foot deformity score, active/limited joint counts, walking speed, double-support time (s) (DS) and step length symmetry index % (SI) were also measured. Foot-care provision in the preceding 12 months was determined from medical records. Results:,Sixty-three per cent of children reported some foot impairment, with a median (range) JAFI subscale score of 1 (0,3); 53% reported foot-related activity limitation, with a JAFI subscale score of 1 (0,4); and 60% reported participation restriction, with a JAFI subscale score of 1 (0,3). Other reported variables were CHAQ 0.38 (0,2), VAS pain 22 (0,79), foot deformity 6 (0,20), active joints 0 (0,7), limited joints 0 (0,31), walking speed 1.09,m/s (0.84,1.38,m/s), DS 0.22,s (0.08,0.26,s) and SI ±4.0% (±0.2,±31.0%). A total of 23/30 medical records were reviewed and 15/23 children had received DMARDS, 8/23 biologic agents and 20/23 multiple intra-articular corticosteroid injections. Ten children received specialist podiatry care comprising footwear advice, orthotic therapy and silicone digital splints together with intrinsic muscle strengthening exercises. Conclusion:,Despite frequent use of DMARD/biologic therapy and specialist podiatry-led foot care, foot-related impairment and disability persists in some children with JIA. Copyright © 2008 John Wiley & Sons, Ltd. [source] Pseudoporphyria and Nonsteroidal Antiinflammatory Agents in Children with Juvenile Idiopathic ArthritisPEDIATRIC DERMATOLOGY, Issue 6 2000Bernadette De Silva M.R.C.P. Nonsteroidal antiinflammatory drugs (NSAIDs) are implicated in the etiology of this condition. In a 1-year prospective study of children attending the pediatric rheumatology clinic in Edinburgh we found a prevalence of pseudoporphyria of 10.9% in children taking NSAIDs for juvenile idiopathic arthritis. Naproxen was the most commonly implicated NSAID, independent of dosage. Blue/gray eye color was an independent risk factor for the development of pseudoporphyria. We would advise caution in prescribing naproxen in these children to prevent disfiguring facial scarring. [source] Autoantibody to heterogeneous nuclear ribonucleoprotein-A2 (RA33) in juvenile idiopathic arthritis: Clinical significancePEDIATRICS INTERNATIONAL, Issue 2 2009Hoda Y. Tomoum Abstract Background:, Objective biomarkers are needed for early diagnosis of juvenile idiopathic arthritis (JIA). Anti-A33 antibodies are considered good markers for adult rheumatoid arthritis (RA), but little information is available on their occurrence in JIA. The aim of the present study was therefore to investigate the value of anti-RA33 for diagnosis of JIA (both early and established disease), and its relation to markers of disease activity, and bone resorption. Subjects:, This case,control study was conducted on 34 children with JIA. Ten patients with arthritis of short duration (<6 weeks) were included, as undifferentiated arthritis. Forty-four age- and sex- matched healthy children served as controls. Beside evaluation and assessment of disease activity, urinary calcium, serum parathyroid hormone and serum anti-RA33 were measured in included subjects. Joints were examined radiologically and modified Larsen index (LI) was estimated. Results:, During follow up, eight of the patients with undifferentiated arthritis were diagnosed as having early JIA. Patients with JIA (early and established cases) had higher anti-RA33 levels than the control group (z = 6.04, 3.95, respectively). A total of 66.7% of the patients were positive for anti-RA33, results were comparable in early and established cases. Anti-RA33 values were correlated to disease activity (clinical and laboratory), to laboratory markers (urinary calcium, parathyroid hormone levels) and radiological evidence (LI) of bone resorption (r = 0.95, 0.63, 0.94, respectively). Conclusion:, Anti-RA33 is detected in two-thirds of JIA patients and occurs with comparable frequency early in the disease. Its levels are correlated to disease activity and markers of bone resorption and it seems to convey diagnostic and prognostic insights for appropriate management. [source] Mizoribine oral pulse therapy for a patient with polyarticular juvenile idiopathic arthritisPEDIATRICS INTERNATIONAL, Issue 6 2006ATSUSHI HAYASHI No abstract is available for this article. [source] Current management of juvenile idiopathic arthritisPRESCRIBER, Issue 5 2009FRCP(Glasg), FRCPCH, Paul Galea MD Our series Prescribing in children gives practical advice for successful management of childhood problems in general practice. Here, the author describes the available treatment options for juvenile idiopathic arthritis and their place in management. Copyright © 2009 Wiley Interface Ltd [source] Impairment and coping in children and adolescents with chronic fatigue syndrome: a comparative study with other paediatric disordersTHE JOURNAL OF CHILD PSYCHOLOGY AND PSYCHIATRY AND ALLIED DISCIPLINES, Issue 3 2004M. Elena Garralda Background:, Functional impairment is a key feature of chronic fatigue syndrome (CFS) of childhood. Aim:, To compare impairment, illness attitudes and coping mechanisms in childhood CFS and in other paediatric disorders. Method:, Participants were 28 children and adolescents with CFS, 30 with juvenile idiopathic arthritis (JIA) and 27 with emotional disorders (ED). The measures used were interviews with children and parents, with detailed enquiry on impairment, including the Functional Disability Inventory (FDI), Illness Attitudes Scales (IAS), and Kidcope to measure coping styles in relation to common problems, illness and disability. Results:, Children with CFS reported significantly more illness impairment, especially in school attendance, than those with JIA and ED. They had higher ,worry about illness' scores on the IAS. On the Kidcope they named school issues (work, expectations, attendance) as illness- or disability-related problems more than the other two groups. Fewer CFS participants reported using problem solving as a strategy to cope with illness and disability than with other problems in their lives. More in the CFS than in the JIA group used emotional regulation to cope with illness and disability. Fewer in the CFS than in the ED groups used social withdrawal to cope with illness and self-criticism for disability, but more used resignation to cope with disability. Conclusion:, Severe illness-related impairment, particularly through school non-attendance, and high levels of illness-related school concerns appear specific to CFS. CFS may also have characteristically high levels of generalised illness worry and particular styles of coping with illness and disability. [source] Juvenile psoriatic arthritis carrying familial Mediterranean fever gene mutations in a 14-year-old Turkish girlTHE JOURNAL OF DERMATOLOGY, Issue 5 2007Betul Sozeri YENIAY ABSTRACT Juvenile psoriatic arthritis (JPsA) is characterized by asymmetric arthritis of big and small joints, enthesitis, dactylitis, psoriatic skin lesions and nail pitting. Investigators agree that JPsA is a relatively common chronic arthropathy of childhood that differs clinically, serologically, and genetically from both juvenile idiopathic arthritis and juvenile ankylosing spondylitis. Familial Mediterranean fever (FMF) is a multisystemic autosomal recessive disease occasionally accompanied by sacroiliitis. This is characterized by recurrent self-limited attacks of fever and accompanying abdominal, chest and arthricular pain. We present a 14-year-old Turkish girl with JPsA and carrying FMF gene mutations. In this patient, JPsA was diagnosed according to her physical, laboratory and skin biopsy findings and a treatment with methotrexate and sulfasalazine was started. As an inadequate clinical and laboratory response was obtained after the first month of therapy, the patient was investigated for FMF, and was diagnosed by molecular analyses of related gene (E148Q heterozygous/V726A homozygous mutation) besides clinical findings. After 2 weeks of the colchicine treatment, symptoms of the patient regressed and acute phase reactants decreased. To our knowledge, this is the first case presenting with psoriatic arthritis and FMF gene mutations together and responds to colchicine, methotrexate and sulfasalazine dramatically in clinical and laboratory findings. This case has been presented to remind that cases with psoriatic arthritis may also carry mutations in the MEFV gene. [source] Heterogeneity in juvenile idiopathic arthritis: Impact of molecular profiling based on DNA polymorphism and gene expression patternsARTHRITIS & RHEUMATISM, Issue 9 2010Susan D. Thompson First page of article [source] Should the food and drug administration warning of malignancy in children receiving tumor necrosis factor , blockers change the way we treat children with juvenile idiopathic arthritis?ARTHRITIS & RHEUMATISM, Issue 8 2010Thomas J. A. Lehman No abstract is available for this article. [source] Quantification of the familial contribution to juvenile idiopathic arthritisARTHRITIS & RHEUMATISM, Issue 8 2010Sampath Prahalad Objective We previously demonstrated that there is familial aggregation of juvenile idiopathic arthritis (JIA). Using a large JIA cohort, we sought to identify additional clusters of JIA cases and to calculate robust estimates of the relative risk (RR) of JIA in the siblings and cousins of JIA probands. We also estimated the population attributable risk (PAR) of familial factors in JIA. Methods A probabilistic record-linking analysis was performed by matching the records of 862 patients with JIA with the records of ,7 million individuals in the Utah Population Database (UPDB), a computerized genealogic database. For each patient, 5 control subjects matched for birth year and sex were selected from the UPDB. Specialized software was used to test for familial aggregation of disease, to estimate the magnitude of familial risks, and to identify families at high risk of disease. Results We identified 22 founders who had significantly more descendants with JIA than expected (5,13 descendants; P values ranged from <0.0001 to <0.008). The PAR of familial factors for JIA was ,13%. The RR of JIA in the siblings of patients was significantly increased (11.6, 95% confidence interval [95% CI] 4.9,27.5, P < 2.59 × 10,8). The RR of JIA in first cousins was also increased (5.82, 95% CI 2.5,13.8, P < 6.07 × 10,5). Conclusion We have identified the largest sets of JIA pedigrees described to date. Approximately 13% of cases of JIA can be attributed to familial factors. Siblings and first cousins of probands with JIA have an increased risk of JIA. The observed decline in the magnitude of risk between siblings and cousins suggests that JIA is influenced by shared genetic factors. [source] Juvenile idiopathic arthritis and HLA Class I and Class II interactions and age-at-onset effectsARTHRITIS & RHEUMATISM, Issue 6 2010Jill A. Hollenbach Objective The aim of this study was to quantitate risk and to examine heterogeneity for HLA at high resolution in patients with the most common subtypes of juvenile idiopathic arthritis (JIA), IgM rheumatoid factor,negative polyarticular JIA and oligoarticular JIA. Use of 4-digit comprehensive HLA typing enabled great precision, and a large cohort allowed for consideration of both age at disease onset and disease subtype. Methods Polymerase chain reaction,based high-resolution HLA typing for class I and class II loci was accomplished for 820 patients with JIA and 273 control subjects. Specific HLA epitopes, potential interactions of alleles at specific loci and between loci (accounting for linkage disequilibrium and haplotypic associations), and an assessment of the current International League of Associations for Rheumatology classification criteria were considered. Results An HLA,DRB1/DQB1 effect was shown to be exclusively attributable to DRB1 and was similar between patients with oligoarticular JIA and a younger subgroup of patients with polyarticular JIA. Furthermore, patients with polyarticular JIA showed age-specific related effects, with disease susceptibility in the group older than age 6 years limited to an effect of the HLA,DRB1*08 haplotype, which is markedly different from the additional susceptibility haplotypes, HLA,DRB1*1103/1104, found in the group with oligoarticular JIA and the group of younger patients with polyarticular JIA. Also in contrast to findings for oligoarticular JIA, patients with polyarticular arthritis had no evidence of an HLA class I effect. Markers associated with a reduced risk of disease included DRB1*1501, DRB1*0401, and DRB1*0701. DRB1*1501 was shown to reduce risk across the whole cohort, whereas DRB1*0401 and DRB1*0701 were protective for selected JIA subtypes. Surprisingly, the disease predisposition mediated by DPB1*0201 in individuals without any disease-predisposing DRB1 alleles was great enough to overcome even the very strong protective effect observed for DRB1*1501. Conclusion Inherited HLA factors in JIA show similarities overall as well as differences between JIA subtypes. [source] Long-term safety and efficacy of abatacept in children with juvenile idiopathic arthritis,ARTHRITIS & RHEUMATISM, Issue 6 2010Nicolino Ruperto Objective We previously documented that abatacept was effective and safe in patients with juvenile idiopathic arthritis (JIA) who had not previously achieved a satisfactory clinical response with disease-modifying antirheumatic drugs or tumor necrosis factor blockade. Here, we report results from the long-term extension (LTE) phase of that study. Methods This report describes the long-term, open-label extension phase of a double-blind, randomized, controlled withdrawal trial in 190 patients with JIA ages 6,17 years. Children were treated with 10 mg/kg abatacept administered intravenously every 4 weeks, with or without methotrexate. Efficacy results were based on data derived from the 153 patients who entered the open-label LTE phase and reflect ,21 months (589 days) of treatment. Safety results include all available open-label data as of May 7, 2008. Results Of the 190 enrolled patients, 153 entered the LTE. By day 589, 90%, 88%, 75%, 57%, and 39% of patients treated with abatacept during the double-blind and LTE phases achieved responses according to the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30), Pedi 50, Pedi 70, Pedi 90, and Pedi 100 criteria for improvement, respectively. Similar response rates were observed by day 589 among patients previously treated with placebo. Among patients who had not achieved an ACR Pedi 30 response at the end of the open-label lead-in phase and who proceeded directly into the LTE, 73%, 64%, 46%, 18%, and 5% achieved ACR Pedi 30, Pedi 50, Pedi 70, Pedi 90, and Pedi 100 responses, respectively, by day 589 of the LTE. No cases of tuberculosis and no malignancies were reported during the LTE. Pneumonia developed in 3 patients, and multiple sclerosis developed in 1 patient. Conclusion Abatacept provided clinically significant and durable efficacy in patients with JIA, including those who did not initially achieve an ACR Pedi 30 response during the initial 4-month open-label lead-in phase. [source] Analysis of intracellular methotrexate polyglutamates in patients with juvenile idiopathic arthritis: Effect of route of administration on variability in intracellular methotrexate polyglutamate concentrationsARTHRITIS & RHEUMATISM, Issue 6 2010Mara L. Becker Objective Intracellular methotrexate (MTX) polyglutamates (MTXGlu) have been shown to be potentially useful biomarkers of clinical response in adult patients with rheumatoid arthritis. The present study was undertaken to measure intracellular MTXGlu concentrations in a cohort of patients with juvenile idiopathic arthritis (JIA) to determine the predictors of MTXGlu variability in these patients. Methods Blood samples were obtained from patients with JIA who were being treated with a stable dose of MTX for ,3 months. Clinical data were collected by chart review. Concentrations of MTXGlu1,7 in red blood cell lysates were quantitated using an innovative ion-pairing chromatography procedure, with detection by mass spectrometry. Results Patients with JIA from a single center (n = 99; mean ± SD age 117.8 ± 56.5 months, 69 female) were included in the analysis. The mean ± SD dose of MTX was 0.51 ± 0.25 mg/kg per week, with a median treatment duration of 18 months (interquartile range 3,156 months). MTX was administered subcutaneously in 66 patients (67%). Fifty-six patients (57%) had active arthritis at the time of the clinic visit. Total intracellular MTXGlu (MTXGluTOT) concentrations varied 40-fold, with a mean ± SD total concentration of 85.8 ± 48.4 nmoles/liter. Concentrations of each MTXGlu subtype (MTXGlu1,7) were measured individually and as a percentage of MTXGluTOT in each patient. MTXGlu3 was the most prominent subtype identified, comprising 42% of MTXGluTOT, and the interindividual variability in the concentration of MTXGlu3 was the most highly correlated with that of MTXGluTOT (r = 0.96). The route of MTX administration was significantly associated with MTXGlu1,5 subtypes; higher concentrations of MTXGlu1 + 2 were observed in patients receiving oral doses of MTX, whereas higher concentrations of MTXGlu3,5 were observed in patients receiving subcutaneous doses of MTX (P < 0.0001). Conclusion In this cohort of patients with JIA, the MTXGluTOT concentration varied 40-fold. Individual MTXGlu metabolites (MTXGlu1,7), which have, until now, not been previously reported in patients with JIA, were detected. The route of MTX administration contributed to the variability in concentrations of MTXGlu1,5. [source] Synovial fluid proteins differentiate between the subtypes of juvenile idiopathic arthritisARTHRITIS & RHEUMATISM, Issue 6 2010Margalit E. Rosenkranz Objective Juvenile idiopathic arthritis (JIA) is a heterogeneous group of inflammatory diseases, and no clinically useful prognostic markers to predict disease outcome in children with JIA are currently available. Synovial fluid likely reflects the proteins present in the inflamed synovium. The purpose of this study was to delineate the synovial fluid proteome and determine whether protein expression differs in the different subtypes of JIA. Methods Synovial fluid samples obtained from children with oligoarticular JIA, polyarticular JIA, or systemic JIA were compared. Two-dimensional gel electrophoresis for protein separation and matrix-assisted laser desorption ionization,time-of-flight mass spectrometry and quadripole time-of-flight mass spectrometry for protein identification were used for this study. Synovial fluid cells were analyzed by polymerase chain reaction (PCR) for the presence of haptoglobin messenger RNA (mRNA). Results The synovial fluid proteome of the samples was delineated. The majority of proteins showed overexpression in JIA synovial fluid as compared with noninflammatory control samples. There were 24 statistically significantly differentially expressed spots (>2-fold change; P < 0.05) between the subtypes of JIA. PCR analysis revealed haptoglobin mRNA, suggesting that haptoglobin is locally produced in an inflamed joint in JIA. Conclusion Despite the similar histologic appearance of inflamed joints in patients with different subtypes of JIA, there are differences in protein expression according to the subtype of JIA. Haptoglobin is differentially expressed between the subtypes of JIA and is locally produced in an inflamed joint in JIA. Haptoglobin and other differentially expressed proteins may be potential biomarkers in JIA. [source] Risk factors for development of uveitis differ between girls and boys with juvenile idiopathic arthritisARTHRITIS & RHEUMATISM, Issue 6 2010R. K. Saurenmann Objective Uveitis is the most common extraarticular manifestation of juvenile idiopathic arthritis (JIA) and is associated with considerable morbidity. The aim of this study was to examine the risk factors associated with uveitis in JIA. Methods We conducted a chart review of 1,047 patients with JIA from a single tertiary care pediatric rheumatology center for factors associated with the development of uveitis. Special emphasis was put on the following known risk factors: oligoarthritis, antinuclear antibody (ANA) status, sex, and age at the time of onset of JIA. Results The risk of uveitis developing was age dependent in girls but not in boys. Among girls, the risk was maximal (47%) in those who were ANA positive and were ages 1,2 years at the time of the onset of JIA; this risk decreased to <10% in those in whom the age at onset was >7 years. Only girls had an age-dependent and ANA-associated increased risk of uveitis. The time interval from the diagnosis of JIA to the diagnosis of uveitis was statistically significantly longer in patients in whom the onset of JIA occurred at a younger age (P = 0.04). This effect was even more pronounced in ANA-positive patients (P = 0.004). The JIA subtype did not influence a patient's risk of the development of uveitis. Conclusion An age-associated risk of uveitis was observed only in girls who were younger than 7 years of age at the time of the onset of JIA. The duration of time between the diagnosis of JIA and the onset of uveitis was longer in patients in whom JIA was diagnosed at a younger age, especially in those who were ANA positive. We suggest that our findings have implications for uveitis screening in patients with JIA. [source] Biologic predictors of extension of oligoarticular juvenile idiopathic arthritis as determined from synovial fluid cellular composition and gene expressionARTHRITIS & RHEUMATISM, Issue 3 2010Patricia J. Hunter Objective To identify biomarkers in the first synovial fluid (SF) aspirate obtained from children with oligoarticular juvenile idiopathic arthritis (JIA), which could be used to identify children whose disease is likely to extend to a more severe phenotype. Methods Patients with recent-onset oligoarticular JIA were identified and grouped according to those whose mild disease persisted (persistent disease) or those whose disease would extend from a mild to more severe phenotype (extended-to-be disease) at 1 year after diagnosis. Flow cytometry was used to delineate differences in the mononuclear cell populations between the first blood sample and first SF aspirate from the same patient and between outcome (persistent versus extended-to-be) groups. Proportions of lymphocytes in the joint were modeled on chemotaxis of lymphocytes to CCL5, using Transwell migration assays. Levels of CCL5 in the SF were quantified by enzyme-linked immunosorbent assay. RNA profiles of SF mononuclear cells were compared between groups using the Affymetrix GeneChip hybridization protocol and hierarchical clustering analyses. Results Compared with peripheral blood mononuclear cells, SF mononuclear cells displayed an expansion of CD8+ T cells, reduced proportion of B cells, and expansion of CD16, natural killer cells. The lower CD4:CD8 ratio in the SF was recapitulated in vitro by the observed migration of blood T cells in response to CCL5. Synovial CCL5 levels were higher in children whose disease extended to a more severe phenotype. The CD4:CD8 ratio in the SF was significantly lower in patients with extended-to-be oligoarticular JIA (0.57 compared with 0.90 in the persistent disease group, difference 0.33, 95% confidence interval 0.04,0.62; P = 0.009). Gene expression profiling revealed that 344 genes were >1.5-fold differentially expressed between outcome groups (P < 0.05), and these included genes associated with inflammation and macrophage differentiation, which showed increased levels in patients with extended disease at 1 year, and genes associated with immune regulation, which showed increased levels in patients with persistent disease at 1 year. Conclusion Analyses of the proportions of synovial lymphocytes, levels of CCL5, and differential gene expression yielded potential biomarkers with which to predict the likelihood of extension of oligoarticular JIA to a more severe disease phenotype. [source] Associations between the American College of Rheumatology pediatric response measures and the continuous measures of disease activity used in adult rheumatoid arthritis: A secondary analysis of clinical trial data from children with Polyarticular-Course Juvenile Idiopathic ArthritisARTHRITIS & RHEUMATISM, Issue 12 2009Sarah Ringold Objective To measure associations between the American College of Rheumatology (ACR) pediatric criteria for improvement and the continuous measures of disease activity used for rheumatoid arthritis in adult patients with polyarticular-course juvenile idiopathic arthritis (JIA). Methods In this retrospective analysis of 2 etanercept trials, disease activity was calculated at baseline, 3 months, and 6 months using the Disease Activity Score (DAS), the DAS based on 28 joints (DAS28), the Simplified Disease Activity Index (SDAI), and the Clinical Disease Activity Index (CDAI). The ACR pediatric response and the European League Against Rheumatism (EULAR) response were also determined for the 3-month and 6-month evaluations. Data were analyzed in 94 patients with JIA independent of the treatment arm. Correlation coefficients between measures were calculated for each visit. The areas under the receiver operating characteristic curve (AUC of ROC) were calculated to assess the discriminative properties of the scores for the ACR pediatric response measures. Results The mean DAS, DAS28, CDAI score, and SDAI score were 3.7, 4.7, 30.8, and 36.4, respectively, at baseline, corresponding to high levels of disease activity (CDAI/SDAI) or moderate levels of disease activity (DAS/DAS28). At 3 months, the mean scores corresponded to low (DAS/DAS28) or moderate (CDAI/SDAI) disease activity. At 6 months, the mean scores corresponded to low disease activity (DAS/DAS28/CDAI) or moderate disease activity (SDAI). Most children met the criteria for a good or moderate EULAR response at 3 months and 6 months. The correlation between continuous outcome measures and each pediatric core set component was moderate to very good. The AUC of ROC values for each measure were high (range 0.76,0.98). Conclusion Good correlation and discriminative abilities were seen between the DAS, DAS28, CDAI, and SDAI for the ACR pediatric criteria for improvement. These disease activity measures may be useful for research and clinical care in polyarticular-course JIA. [source] | |||||||||||||||||||||||||||||||