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Sample records for juvenile arthritis disease

  1. Juvenile idiopathic arthritis

    Science.gov (United States)

    Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... The cause of juvenile idiopathic arthritis (JIA) is not known. It ... illness . This means the body attacks and destroys healthy body ...

  2. Juvenile Arthritis

    Science.gov (United States)

    Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

  3. Juvenile chronic arthritis and coeliac disease in the Netherlands

    NARCIS (Netherlands)

    George, EK; HertzbergertenCate, R; vanSuijlekomSmit, LWA; vonBlomberg, BME; Stapel, SO; vanElburg, RM; Mearin, ML

    1996-01-01

    Objective. It has been suggested that juvenile chronic arthritis (JCA) is associated with coeliac disease in a frequency of 0.4-2%. In order to investigate the frequency of coeliac disease in cases of JCA and the possibility of underdiagnosis in our area, we screened 62 children with JCA (mean age

  4. Juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Prakken, Berent; Albani, Salvatore; Martini, Alberto

    2011-01-01

    Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years. Pivotal studies in the past 5 years have led to substantial progress in various areas, ranging from disease classification to new treatments. Gene expres

  5. Serum melatonin in juvenile rheumatoid arthritis: correlation with disease activity.

    Science.gov (United States)

    El-Awady, Hanaa Mahmoud; El-Wakkad, Amany Salah El-Dien; Saleh, Maysa Tawheed; Muhammad, Saadia Ibraheem; Ghaniema, Eiman Mahmoud

    2007-05-01

    The study was conducted to investigate the abnormalities in early morning serum melatonin among patients with Juvenile Rheumatoid Arthritis (JRA) and to outline its relation to disease activity and severity. Twenty one patients with JRA and twenty healthy age and sex matched controls were enrolled in the study. Fifteen patients had polyarticular JRA, 3 had oligoarticular and 3 had systemic onset JRA. Evaluation was carried out clinically, functionally and radiologically by using disease activity score, Juvenile Arthritis Functional Assessment Report for Children (JAFAR-C score) and modified Larsen score, respectively. Laboratory investigations included Complete Blood Picture (CBC), The Erythrocyte Sedimentation Rate (ESR), C-Reactive Protein (CRP), classic IgM Rheumatoid Factor (RF), Anti-nuclear Antibodies (ANA) and melatonin estimation in serum. The serum levels of melatonin were significantly increased in JRA patients (mean +/- SD = 13.9 +/- 8 pg mL(-1)) as compared to healthy controls (mean +/- SD = 8.1 +/- 2.7 pg mL(-1), p 0.05). Hence the study conclude that the elevated melatonin levels among JRA patients with active synovitis and its close relation to disease activity rather than disease severity suggests that melatonin might play a promoting role in rheumatoid arthritis. Hence, inhibition of its synthesis and/or action by specific antagonists may be of therapeutic value.

  6. Assessment of disease activity in juvenile idiopathic arthritis. The number and the size of joints matter

    DEFF Research Database (Denmark)

    Berntson, Lillemor; Wernroth, Lisa; Fasth, Anders

    2007-01-01

    Variables for assessment of disease activity of juvenile idiopathic arthritis (JIA) were studied, in order to develop a disease activity score for children with JIA.......Variables for assessment of disease activity of juvenile idiopathic arthritis (JIA) were studied, in order to develop a disease activity score for children with JIA....

  7. What Is Juvenile Arthritis?

    Science.gov (United States)

    ... Analgesics for Osteoarthritis (Report from AHRQ) Joint Replacement Surgery: Health Information Basics for You and Your Family NIH Pediatric Rheumatology Clinic Health Information Juvenile Arthritis Find a Clinical Trial Journal Articles Juvenile Arthritis PDF Version Size: 123 KB ...

  8. Juvenil idiopatisk arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2002-01-01

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis...

  9. Mechanisms of disease and therapy in severe juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Vastert, S.J.

    2013-01-01

    This thesis describes results of translational research (both bench to bedside as reverse translation from bedside back to the bench) in severe Juvenile idiopathic Arthritis (JIA). It focuses on understanding critical immunological features of both systemic and polyarticular JIA and relates this to

  10. Juvenile chronic arthritis.

    Science.gov (United States)

    Southwood, T R; Woo, P

    1995-05-01

    The nomenclature and classification criteria for arthritis in children should be dealt with initially as separate issues, although they are undoubtedly intertwined. The classification criteria should aim to delineate homogeneous patient populations, yet should be flexible enough to incorporate advances in disease knowledge. It should be recognized that arriving at an international consensus for classification criteria will merely provide a set of operational definitions to facilitate research, and not a set of diagnostic criteria. Indeed the only point to obtaining consensus is to begin a process of systematic ongoing review of the criteria. The labels attached to any of these diseases should facilitate accurate communication. In view of the heterogeneous nature of childhood arthritis, consideration should be given to using a broad umbrella term such as juvenile or childhood arthritis only for communicating with the lay public. Medical nomenclature should be formulated to reflect accurately homogeneous subgroups of arthritis, and should not artificially proscribe a relationship between paediatric and adult disease.

  11. Validity of a three-variable Juvenile Arthritis Disease Activity Score in children with new-onset juvenile idiopathic arthritis

    Science.gov (United States)

    Mcerlane, Flora; Beresford, Michael W; Baildam, Eileen M; Chieng, S E Alice; Davidson, Joyce E; Foster, Helen E; Gardner-Medwin, Janet; Lunt, Mark; Wedderburn, Lucy R; Thomson, Wendy; Hyrich, Kimme L

    2013-01-01

    Objectives To investigate the validity and feasibility of the Juvenile Arthritis Disease Activity Score (JADAS) in the routine clinical setting for all juvenile idiopathic arthritis (JIA) disease categories and explore whether exclusion of the erythrocyte sedimentation rate (ESR) from JADAS (the ‘JADAS3’) influences correlation with single markers of disease activity. Methods JADAS-71, JADAS-27 and JADAS-10 were determined at baseline for an inception cohort of children with JIA in the Childhood Arthritis Prospective Study. JADAS3-71, JADAS3-27 and JADAS3-10 were determined using an identical formula but with exclusion of ESR. Correlation of JADAS with JADAS3 and single measures of disease activity/severity were determined by category. Results Of 956 eligible children, sufficient data were available to calculate JADAS-71, JADAS-27 and JADAS-10 at baseline in 352 (37%) and JADAS3 in 551 (58%). The median (IQR) JADAS-71, JADAS-27 and JADAS-10 for all 352 children was 11 (5.9–18), 10.4 (5.7–17) and 11 (5.9–17.3), respectively. Median JADAS and JADAS3 varied significantly with the category (Kruskal–Wallis p=0.0001), with the highest values in children with polyarticular disease patterns. Correlation of JADAS and JADAS3 across all categories was excellent. Correlation of JADAS71 with single markers of disease activity/severity was good to moderate, with some variation across the categories. With the exception of ESR, correlation of JADAS3-71 was similar to correlation of JADAS-71 with the same indices. Conclusions This study is the first to apply JADAS to all categories of JIA in a routine clinical setting in the UK, adding further information about the feasibility and construct validity of JADAS. For the majority of categories, clinical applicability would be improved by exclusion of the ESR. PMID:23256951

  12. Juvenile Arthritis

    Science.gov (United States)

    ... increased risk of developing an inflammatory eye problem (iritis or uveitis). Eye inflammation may persist independently of the arthritis. Because iritis usually does not cause symptoms, regular exams by ...

  13. [Juvenile idiopathic arthritis].

    Science.gov (United States)

    Herlin, Troels

    2002-08-19

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis. In addition to the clinical characteristics, genetic and biochemical differences suggest that JIA could be regarded as a general term covering various diseases. Complications described are uveitis, temporomandibular joint affection and growth disturbances. The therapeutic strategy should be planned individually according to age, subtype and disease activity and carried out as teamwork with several specialties. Drugs showing significant effectiveness in controlled studies are primarily methotrexate and sulphasalazine. An immunomodulating agent, etanercept, a soluble TNF alpha-receptor fusion protein, has shown a promising effect in severe polyarticular JIA refractory to methotrexate treatment.

  14. Discrimination of acute lymphoblastic leukemia from systemic-onset juvenile idiopathic arthritis at disease onset

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    Mirian S. Tamashiro

    2011-01-01

    Full Text Available OBJECTIVE: To assess clinical and laboratory features that differentiate acute lymphoblastic leukemia from systemic juvenile idiopathic arthritis at disease onset. METHODS: Fifty-seven leukemia patients with musculoskeletal involvement, without blasts on peripheral blood and without glucocorticoid therapy at disease onset and 102 systemic juvenile idiopathic arthritis patients (International League of Associations for Rheumatology criteria were retrospectively evaluated. The following features were examined: fever, rheumatoid rash, arthritis, limb pain, hepatomegaly, splenomegaly, pericarditis, myocarditis, pleuritis, weight loss, bleeding, anemia, leukopenia, neutropenia, thrombocytopenia, erythrocyte sedimentation rate, and lactic dehydrogenase levels. RESULTS: The median age at disease onset was significantly higher in leukemia patients than in those with systemic-onset juvenile idiopathic arthritis (5.8 vs. 3.8 years. In addition, the frequencies of limb pain, hepatomegaly, weight loss and hemorrhagic manifestations were significantly higher in leukemia patients than in systemic-onset juvenile idiopathic arthritis patients (70% vs. 1%, 54% vs. 32%, 30% vs. 8%, and 9% vs. 0%, respectively. Likewise, the frequencies of anemia, leukopenia, neutropenia, thrombocytopenia and high lactic dehydrogenase levels were statistically higher in leukemia patients than in patients with systemic-onset juvenile idiopathic arthritis (88% vs. 57%, 39% vs. 1%, 60% vs. 1%, 77% vs. 1%, and 56% vs. 14%, respectively. Remarkably, multivariate analysis revealed that limb pain (OR = 553; 95% CI =46.48-6580.42 and thrombocytopenia (OR = 754.13; 95% CI =64.57-8806.72 were significant independent variables that differentiated leukemia from systemic-onset juvenile idiopathic arthritis. The R2 of the Nagelkerke test was 0.91, and the Kaplan-Meier survival curves were similar for acute lymphoblastic leukemia patients with and without limb pain. CONCLUSION: Our study

  15. Overlap of disease susceptibility loci for rheumatoid arthritis and juvenile idiopathic arthritis

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    Hinks, Anne; Eyre, Steve; Ke, Xiayi; Barton, Anne; Martin, Paul; Flynn, Edward; Packham, Jon; Worthington, Jane; Thomson, Wendy

    2010-01-01

    Background Genome-wide association studies (GWAS) have been extremely successful in the search for susceptibility risk factors for complex genetic autoimmune diseases. As more studies are published, evidence is emerging of considerable overlap of loci between these diseases. In juvenile idiopathic arthritis (JIA), another complex genetic autoimmune disease, the strategy of using information from autoimmune disease GWAS or candidate gene studies to help in the search for novel JIA susceptibility loci has been successful, with confirmed association with two genes, PTPN22 and IL2RA. Rheumatoid arthritis (RA) is an autoimmune disease that shares similar clinical and pathological features with JIA and, therefore, recently identified confirmed RA susceptibility loci are also excellent JIA candidate loci. Objective To determine the overlap of disease susceptibility loci for RA and JIA. Methods Fifteen single nucleotide polymorphisms (SNPs) at nine RA-associated loci were genotyped in Caucasian patients with JIA (n=1054) and controls (n=3531) and tested for association with JIA. Allele and genotype frequencies were compared between cases and controls using the genetic analysis software, PLINK. Results Two JIA susceptibility loci were identified, one of which was a novel JIA association (STAT4) and the second confirmed previously published associations of the TRAF1/C5 locus with JIA. Weak evidence of association of JIA with three additional loci (Chr6q23, KIF5A and PRKCQ) was also obtained, which warrants further investigation. Conclusion All these loci are good candidates in view of the known pathogenesis of JIA, as genes within these regions (TRAF1, STAT4, TNFAIP3, PRKCQ) are known to be involved in T-cell receptor signalling or activation pathways. PMID:19674979

  16. 25-hydroxyvitamin D levels and juvenile idiopathic arthritis: is there an association with disease activity?

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    To examine the association between serum levels of 25-hydroxyvitamin D [25(OH)D] and disease activity in juvenile idiopathic arthritis (JIA), to determine the prevalence of vitamin D (VD) deficiency [25(OH)D=19 ng/ml] and insufficiency [25(OH)D 20-29 ng/ml], and to determine factors associated with ...

  17. Juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Krupa H Bhatt

    2014-01-01

    Full Text Available Juvenile Idiopathic Arthritis (JIA is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential.

  18. Genetics in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Albers, Heleen Marion

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is a non-common disease in children that can persist into adulthood. JIA is considered to be an auto-immune disease. Genetic factors play a role in the pathogenesis. In a new cohort of JIA patients from North-West European descent genetic candidate gene associatio

  19. Ankle arthritis predicts polyarticular disease course and unfavourable outcome in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Esbjörnsson, Anna-Clara; Aalto, Kristiina; Broström, Eva W

    2015-01-01

    on remission was available for 427 of these children. Occurrence of clinically assessed ankle arthritis was analysed in relation to JIA category, clinical characteristics and remission data eight years after disease onset. RESULTS: In 440 children with JIA, 251 (57%) experienced ankle arthritis during...... the first eight years of disease. Ankle arthritis was least common in the persistent oligoarticular category (25%) and most common in children with extended oligoarticular (83%) and polyarticular RF-negative (85%) JIA. Children who developed ankle arthritis during the first year of disease were younger...... at disease onset (median age 4.9 (IQR 2.1-8.8) vs. 6.6 (IQR 2.8-10.1) years, pdisease onset, if the ankle joint...

  20. JUVENILE RHEUMATOID ARTHRITIS

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    I N Sartika

    2012-11-01

    Full Text Available Juvenile rheumatoid arthritis (JRA is the most common rheumatic condition in children. JRA is defined as persistent arthritis in 1 or more joints for at least 6 weeks, with the onset before age 16 years. The etiology of JRA is unknown. Antigen activated CD4+ T cell stimulate monocytes, macrophages, and synovial fibroblasts to produce the cytokines Interleukin-1 (IL-1, IL-6, and tumor necrosis factor ? (TNF-? and to secrete matrix metalloproteinases, which lead to chronic inflammation due to infiltration of inflammatory cell, angiogenesis, destruction of cartilage and bone with pannus formation. The 3 major subtypes of JRA are based on the symptoms at disease onset and are designated systemic onset, pauciarticular onset, and polyarticular onset. For all patients, the goals of therapy are to decrease chronic joint pain and suppress the inflammatory process. Poor prognostic have been observed in patients with polyarticular onset, rheumatoid factor, persistent morning stiffness, tenosynovitis, involvement of the small joints, rapid appearance of erosions, active late onset childhood, subcutaneous nodules, or antinuclear antibody.

  1. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... Home Health Conditions juvenile idiopathic arthritis juvenile idiopathic arthritis Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  2. Juvenile arthritis and uveitis.

    Science.gov (United States)

    Kanski, J J

    1990-01-01

    The association between juvenile arthritis and uveitis is reviewed. Some children with the HLA-B27 related spondyloarthropathies develop anterior uveitis. About 20% of patients with juvenile rheumatoid arthritis (JRA) who are negative for IgM rheumatoid factor develop a frequently bilateral, nongranulomatous chronic anterior uveitis. Risk factors for uveitis in JRA patients are: female gender, pauciarticular onset of arthritis, presence of circulating antinuclear antibodies, and the antigens HLA-DW5 and HLA-DPw2. Uveitis is rare after seven years or more have elapsed from the onset of arthritis. The visual prognosis in patients with uveitis is good in 25% and fair in 50%. The remaining 25% develop visual impairment from complicated cataract and/or secondary inflammatory glaucoma. The potential benefit of cytotoxic agents in the treatment of intractable uveitis is outweighed by the risk of serious side effects. The management of secondary inflammatory glaucoma is unsatisfactory, but the results of treatment of complicated cataracts by lensectomy-vitrectomy are good.

  3. Imaging of juvenile idiopathic arthritis

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    Johnson, Karl [Birmingham Children' s Hospital, Radiology Department, Birmingham (United Kingdom)

    2006-08-15

    Over the past decade there have been considerable changes in the classification and imaging of juvenile idiopathic arthritis (JIA). Radiology now has a considerable role in the management of JIA, the differential diagnosis, monitoring disease progression and detecting complications. The different imaging modalities available, their role and limitations are discussed in this article and the various disease features that the radiologist should be aware of are described. An approach to the imaging of the child with joint disease and in the monitoring of disease complications are also discussed. (orig.)

  4. Characteristics and relationship of periodontal disease with juvenile idiopathic and rheumatoid arthritis.

    Science.gov (United States)

    Vahabi, Surena; Rostamian, Abdolrahman; Baniebrahimi, Ghazaleh

    2015-01-01

    Rheumatoid arthritis (RA) is the most prevalent chronic inflammatory disease of the joints. It is correlated with periodontal disease due to similar factors that exist in both diseases. The present study assessed the relationship of periodontal disease with RA and juvenile idiopathic arthritis (JIA). In this case-control study, 30 RA and 30 JIA patients along with similar number of matched controls were selected among patients referred to Imam Khomeini Hospital, Tehran, Iran. Periodontal parameters including pocket depth (PD), clinical attachment level (CAL), O'Leary and Bay plaque index (PI) and bleeding on probing (BOP) were determined in cases and controls. Erythrocyte sedimentation rate, number of painful and inflamed joints and severity of disease were evaluated in RA and JIA patients. Mann-Whitney U-test nonparametric, Spearman and Pearson's correlation coefficients, and Chi-square tests were used as statistical analysis (α = 0.05). PD (4.17 vs. 3.6 mm; P 4 mm (58.83% vs. 44.33%; P 3 mm (74.13% vs. 64.4%; P disease severity and number of painful and inflamed joints with periodontal factors. In JIA patients, no significant relationships were found between JIA findings and periodontal parameters. Considering the limitations of this study, there was a relationship between RA and periodontal disease. Severity of periodontal disease increases in patients with RA, while no increased risk of periodontal disease or its severity was observed among JIA patients.

  5. A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis?

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    Prendiville, Julie S; Tucker, Lori B; Cabral, David A; Crawford, Richard I

    2004-01-01

    The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic

  6. Different familial association patterns of autoimmune diseases between juvenile-onset systemic lupus erythematosus and juvenile rheumatoid arthritis.

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    Huang, Chun-Mei; Yang, Yao-Hsu; Chiang, Bor-Luen

    2004-04-01

    The aim of this study was to determine if the prevalence of autoimmune disorders in the relatives of patients with systemic lupus erythematosus (SLE) is greater than that of relatives of patients with juvenile rheumatoid arthritis (JRA). Interviews were used to obtain histories of the following autoimmune disorders among living or deceased first-, second-, and third-degree relatives of 91 SLE and 110 JRA families: ankylosing spondylitis, SLE, rheumatoid arthritis (RA), JRA, multiple sclerosis, juvenile dermatomyositis, Sjögren's syndrome, myasthenia gravis, psoriasis, and thyroid diseases. There were statistically significant differences between the SLE and JRA probands in mean age and gender ratio (19.1 +/- 4.8 vs 14.0 +/- 5.5 years; M (male)/F (female): 17/74 vs 62/48, pJRA families (11.8%), but not statistically significantly so. The mean age (18.0 +/- 5.3 vs 14.0 +/- 4.3 years), mean age at diagnosis (13.4 +/- 4.3 vs 7.9 +/- 3.9 years) and gender ratio (F/M, 16/3 vs 5/8) of the patients with affected relatives between these 2 groups all had statistically significant differences. A higher prevalence of SLE in relatives was found in SLE families than in JRA cases. Furthermore, this study revealed a higher incidence of autoimmune disorders among second- and third-degree relatives of SLE or JRA probands versus first-degree ones, especially sisters (including 1 pair of twins) and the maternal aunt in SLE families. These data demonstrate that the prevalence of autoimmune disorders in the relatives of patients with SLE is greater than those of relatives of patients with JRA. This suggests that clinically different autoimmune phenotypes may share common susceptibility genes, which may act as risk factors for autoimmunity.

  7. Atherosclerosis in Juvenile Idiopathic Arthritis

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    Ewa Jednacz

    2012-01-01

    Full Text Available Atherosclerosis is a chronic inflammatory disease of the arteries. Clinical consequences of the atherosclerotic process occur in the adult population, however atherosclerotic process begins in childhood. The classic risk factors for atherosclerosis include obesity, dyslipidaemia, age, gender or family history. In recent years, attention has been drawn to the similarity between atherosclerotic inflammatory processes and inflammatory changes in the course of systemic connective tissue disease, in particular systemic lupus etythematosus (SLE or rheumatoid arthritis (RA. There is also observed the similarity of the pathogenetic background of development of atherosclerosis and juvenile idiopathic arthritis (JIA. Elevated levels of pro-inflammatory cytokines are observed in the course of juvenile idiopathic arthritis. Also homocysteine concentrations, which may play a significant role in the development of atherosclerotic lesions, are observed higher in patients with JIA. Some studies revealed higher carotid intima-media thickness (IMT index values in children with JIA. In view of the fact that atherosclerotic process begins as early as in childhood, the introduction of appropriate preventive measures in children is a matter of utmost importance.

  8. Glucocorticoids in juvenile idiopathic arthritis.

    Science.gov (United States)

    Malattia, Clara; Martini, Alberto

    2014-05-01

    Although the use of corticosteroids in juvenile idiopathic arthritis (JIA) is now much more limited owing to the availability of methotrexate and biological agents, there are clinical scenarios where it is still indicated. For example, corticosteroids may be indicated for intraarticular injections to prevent joint deformities, as a "bridge" drug to relieve symptoms in polyarticular disease while waiting for methotrexate and biologics to exert their full therapeutic effects, and in the treatment of chronic iridocyclitis, macrophage activation syndrome, and systemic JIA, although the advent of interleukin (IL)-1 and IL-6 blockers has greatly reduced the latter indication.

  9. Characteristics and relationship of periodontal disease with juvenile idiopathic and rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Surena Vahabi

    2015-01-01

    Full Text Available Background: Rheumatoid arthritis (RA is the most prevalent chronic inflammatory disease of the joints. It is correlated with periodontal disease due to similar factors that exist in both diseases. The present study assessed the relationship of periodontal disease with RA and juvenile idiopathic arthritis (JIA. Materials and Methods: In this case-control study, 30 RA and 30 JIA patients along with similar number of matched controls were selected among patients referred to Imam Khomeini Hospital, Tehran, Iran. Periodontal parameters including pocket depth (PD, clinical attachment level (CAL, O′Leary and Bay plaque index (PI and bleeding on probing (BOP were determined in cases and controls. Erythrocyte sedimentation rate, number of painful and inflamed joints and severity of disease were evaluated in RA and JIA patients. Mann-Whitney U-test nonparametric, Spearman and Pearson′s correlation coefficients, and Chi-square tests were used as statistical analysis (α = 0.05. Results: PD (4.17 vs. 3.6 mm; P 4 mm (58.83% vs. 44.33%; P 3 mm (74.13% vs. 64.4%; P < 0.001, percentage of sites with BOP (9.67% vs. 6.87%; P < 0.0001 and PI index (85.73% vs. 80.63%; P < 0.0001 were significantly higher in RA patients than controls. In this group, direct and significant correlations were found between serologic findings, disease severity and number of painful and inflamed joints with periodontal factors. In JIA patients, no significant relationships were found between JIA findings and periodontal parameters. Conclusion: Considering the limitations of this study, there was a relationship between RA and periodontal disease. Severity of periodontal disease increases in patients with RA, while no increased risk of periodontal disease or its severity was observed among JIA patients.

  10. Aerobic capacity and disease activity in children, adolescents and young adults with juvenile idiopathic arthritis (JIA

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    van Pelt Philomien A

    2012-08-01

    Full Text Available Abstract Background As patients with juvenile idiopathic arthritis (JIA progress into adulthood, long-term outcome is determined by disease activity, physical and psychosocial development. Decreased aerobic capacity may play a critical role in health-related outcomes in JIA, since it has been linked with cardiovascular morbidity and mortality in late adulthood. The objectives of the current study are to examine the aerobic capacity and its relation to parameters of disease activity in children, adolescents and young adults with JIA. Methods Sixty-three patients with JIA (aged 10–27 years were cross sectional studied regarding their aerobic capacity and correlations were made to demographic, disease-related variables, and medication utilization. in a cross-sectional study group of 63 patients of all subtypes. Patients were divided in three age groups, 10–13 years; 14–17 years and 18–27 years. Results Reduced aerobic capacity is found in clinical remission as well as active disease in all subtypes and all age groups. Aerobic capacity is more impaired in active disease shown by DAS 28, JADAS 27, ESR and serum thrombocyte counts. Lower haemoglobin has a negative impact. Long-term used medication including methotrexate and corticosteroids didn’t influence outcome. There is no association with current sports participation. Conclusion Reduced aerobic capacity is present in children and adolescents with JIA, both in active disease and in patients with remission. Measures of aerobic capacity may serve as important outcome measure in JIA.

  11. Erythrocyte-methotrexate and disease activity in children treated with oral methotrexate for juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Kristensen, K; Nielsen, S; Karup Pedersen, F;

    2000-01-01

    The concentration of methotrexate (MTX) in erythrocytes (E-MTX) was measured twice with three months interval in 21 children suffering from juvenile chronic arthritis (JCA). At the same time joint score, visual analogue scale (VAS), and laboratory parameters (CRP, WBC, PMNs, and ALAT) were obtained...

  12. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  13. Retrocalcaneal bursitis in juvenile chronic arthritis.

    OpenAIRE

    Goldenstein-Schainberg, C; Homsi, C; Rodrigues Pereira, R M; W. Cossermelli

    1992-01-01

    Retrocalcaneal bursitis has been described in various adult rheumatic diseases and septic bursitis unrelated to previous bursal disease has been reported in children. The case is reported here of a girl with juvenile chronic arthritis who developed non-septic retrocalcaneal bursitis; the diagnosis was suggested by a combination of clinical and radiographic studies and was confirmed by ultrasonography.

  14. Retrocalcaneal bursitis in juvenile chronic arthritis.

    Science.gov (United States)

    Goldenstein-Schainberg, C; Homsi, C; Rodrigues Pereira, R M; Cossermelli, W

    1992-01-01

    Retrocalcaneal bursitis has been described in various adult rheumatic diseases and septic bursitis unrelated to previous bursal disease has been reported in children. The case is reported here of a girl with juvenile chronic arthritis who developed non-septic retrocalcaneal bursitis; the diagnosis was suggested by a combination of clinical and radiographic studies and was confirmed by ultrasonography. Images PMID:1444631

  15. Validity and predictive ability of the juvenile arthritis disease activity score based on CRP versus ESR in a Nordic population-based setting

    DEFF Research Database (Denmark)

    Nordal, E B; Zak, Marek Stanislaw; Aalto, K

    2012-01-01

    To compare the juvenile arthritis disease activity score (JADAS) based on C reactive protein (CRP) (JADAS-CRP) with JADAS based on erythrocyte sedimentation rate (ESR) (JADAS-ESR) and to validate JADAS in a population-based setting.......To compare the juvenile arthritis disease activity score (JADAS) based on C reactive protein (CRP) (JADAS-CRP) with JADAS based on erythrocyte sedimentation rate (ESR) (JADAS-ESR) and to validate JADAS in a population-based setting....

  16. Investigation of type 1 diabetes and coeliac disease susceptibility loci for association with juvenile idiopathic arthritis

    Science.gov (United States)

    Hinks, Anne; Martin, Paul; Flynn, Edward; Eyre, Steve; Packham, Jon; Barton, Anne; Worthington, Jane; Thomson, Wendy

    2010-01-01

    Background There is strong evidence suggesting that juvenile idiopathic arthritis (JIA) shares many susceptibility loci with other autoimmune diseases. Objective To investigate variants robustly associated with type 1 diabetes (T1D) or coeliac disease (CD) for association with JIA. Methods Sixteen single-nucleotide polymorphisms (SNPs) already identified as susceptibility loci for T1D/CD were selected for genotyping in patients with JIA (n=1054) and healthy controls (n=3129). Genotype and allele frequencies were compared using the Cochrane–Armitage trend test implemented in PLINK. Results One SNP in the LPP gene, rs1464510, showed significant association with JIA (ptrend=0.002, OR=1.18, 95% CI 1.06 to 1.30). A second SNP, rs653178 in ATXN2, also showed nominal evidence for association with JIA (ptrend=0.02, OR=1.13, 95% CI 1.02 to 1.25). The SNP, rs17810546, in IL12A showed subtype-specific association with enthesitis-related arthritis (ERA) subtype (ptrend=0.005, OR=1.88, 95% CI 1.2 to 2.94). Conclusions Evidence for a novel JIA susceptibility locus, LPP, is presented. Association at the SH2B3/ATXN2 locus, previously reported to be associated with JIA in a US series, also supports this region as contributing to JIA susceptibility. In addition, a subtype-specific association of IL12A with ERA is identified. All findings will require validation in independent JIA cohorts. PMID:20647273

  17. [HLA antigens in juvenile rheumatoid arthritis].

    Science.gov (United States)

    Rumba, I V; Sochnev, A M; Kukaĭne, E M; Burshteĭn, A M; Benevolenskaia, L I

    1990-01-01

    Antigens of I class HLA system (locus A and B) were investigated in 67 patients of Latvian nationality suffering from juvenile rheumatoid arthritis (JRA). Associations of HLA antigens with juvenile rheumatoid arthritis partially coincided with the ones revealed earlier. Typing established an increased incidence of antigen B27 (p less than 0.01) and gaplotype A2, B40 (p less than 0.01). Antigen B15 possessed a protective action with respect to JRA. Interlocus combinations demonstrated a closer association with the disease than a single antigen. The authors also revealed markers of various clinico-anatomical variants of JRA.

  18. Heart Disease, Hypertension, Gestational Diabetes Mellitus, and Preeclampsia/Eclampsia in Mothers With Juvenile Arthritis: A Nested Case-Control Study.

    Science.gov (United States)

    Feldman, Debbie E; Vinet, Évelyne; Bérard, Anick; Duffy, Ciarán; Hazel, Beth; Meshefedjian, Garbis; Sylvestre, Marie-Pierre; Bernatsky, Sasha

    2017-02-01

    To determine whether women with a history of juvenile arthritis are at higher risk for heart disease and hypertension and for developing adverse maternal outcomes: gestational diabetes mellitus, maternal hypertension, and preeclampsia/eclampsia. We designed a nested case-control study from a cohort of first-time mothers with prior physician billing codes suggesting juvenile arthritis, and a matched comparison group without juvenile arthritis. For the nested case-control design, we selected 3 controls for each case for the outcomes of heart disease (n = 403), prepregnancy hypertension (n = 66), gestational diabetes mellitus (n = 285), maternal hypertension (n = 561), and preeclampsia/eclampsia (n = 236). We used conditional logistic regression, adjusting for maternal age and education. Having juvenile arthritis was associated with heart disease (odds ratio [OR] 2.44 [95% confidence interval (95% CI) 1.15-5.15]) but not with gestational hypertension, diabetes mellitus, or preeclampsia/eclampsia. All 66 cases of prepregnancy hypertension had juvenile arthritis. Having prepregnancy hypertension was strongly associated with preeclampsia/eclampsia (OR 8.05 [95% CI 2.69-24.07]). Women with a history of juvenile arthritis had a higher risk of heart disease. This risk signals the potential importance of cardiac prevention strategies in juvenile arthritis. As this was a retrospective study, it was not possible to correct for some relevant potential confounders. Further studies should assess the impact of medications, disease severity, and other factors (e.g., obesity) on cardiac outcomes in juvenile arthritis. © 2016, American College of Rheumatology.

  19. Antineutrophil cytoplasmic antibodies in juvenile chronic arthritis

    NARCIS (Netherlands)

    Mulder, L; Horst, G; Limburg, P; deGraeffMeeder, ER; Kuis, W; Kallenberg, C

    1997-01-01

    Objective, To evaluate the diagnostic significance of antineutrophil cytoplasmic antibodies (ANCA) by assessing the prevalence of ANCA in juvenile chronic arthritis (JCA) (n = 93) of either oligoarticular, polyarticular, or systemic onset. To investigate the prevalence of ANCA in other diseases of c

  20. The human microbiome and juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Verwoerd, Anouk; ter Haar, Nienke M.; de Roock, Sytze; Vastert, Sebastiaan J.; Bogaert, Debby

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The

  1. The human microbiome and juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Verwoerd, Anouk; ter Haar, Nienke M.; de Roock, Sytze; Vastert, Sebastiaan J.; Bogaert, Debby

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The

  2. Aerobic capacity and disease activity in children, adolescents and young adults with juvenile idiopathic arthritis (JIA

    Directory of Open Access Journals (Sweden)

    van Pelt Philomine A

    2012-08-01

    Full Text Available Abstract Background As patients with juvenile idiopathic arthritis (JIA progress into adulthood, long-term outcome is determined by disease activity, physical and psychosocial development. Decreased aerobic capacity may play a critical role in health-related outcomes in JIA, since it has been linked with cardiovascular morbidity and mortality in late adulthood. The objectives of the current study are to examine the aerobic capacity and its relation to parameters of disease activity in children, adolescents and young adults with JIA. Methods Sixty-three patients with JIA (aged 10–27 years were cross sectional studied regarding their aerobic capacity and correlations were made to demographic, disease-related variables, and medication utilization. in a cross-sectional study group of 63 patients of all subtypes. Patients were divided in three age groups, 10–13 years; 14–17 years and 18–27 years. Results Reduced aerobic capacity is found in clinical remission as well as active disease in all subtypes and all age groups. Aerobic capacity is more impaired in active disease shown by DAS 28, JADAS 27, ESR and serum thrombocyte counts. Lower haemoglobin has a negative impact. Long-term used medication including methotrexate and corticosteroids didn’t influence outcome. There is no association with current sports participation. Conclusion Reduced aerobic capacity is present in adolescents and young adults with JIA, both in active disease and in patients with remission. Measures of aerobic capacity may serve as important outcome measure in JIA.

  3. Uveitis in juvenile chronic arthritis.

    Science.gov (United States)

    Kanski, J J

    1990-01-01

    About 20% of patients with juvenile chronic arthritis develop uveitis which is frequently bilateral. Risk factors for uveitis are: female gender, pauciarticular onset of arthritis, presence of circulating antinuclear antibodies, and the antigens HLA-DW5 and HLA-DPw2. The visual prognosis in patients with uveitis is good in 25% and fair in 50%. The remaining 25% develop cataract and/or glaucoma. The management of glaucoma is unsatisfactory, but the results of cataract surgery by lensectomy are good.

  4. [Unusual presentation of juvenile idiopathic arthritis and autoimmune hepatitis].

    Science.gov (United States)

    Moreno Prieto, M; Carbonero Celis, M J; Cuadrado Caballero, M C

    2015-01-01

    The coexistence of autoimmune hepatitis and juvenile idiopathic arthritis is very rare. This is the case of an 18 month old female patient whose first sign of disease was torticollis due to an underlying atlanto-axial subluxation. Three months later, bilateral knee arthritis developed and she was diagnosed with Juvenile Idiopathic Arthritis. Throughout the disease a persistent elevation of liver enzymes was noted, combined with positive antinuclear antibodies and hypergammaglobulinemia, reaching the diagnosis of concomitant autoimmune hepatitis.

  5. Systematic review of disease-modifying antirheumatic drugs for juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Kemper Alex R

    2012-03-01

    Full Text Available Abstract Background Treatment of juvenile idiopathic arthritis (JIA with disease-modifying antirheumatic drugs (DMARDs may improve outcomes compared to conventional therapy (e.g., non-steroidal anti-inflammatory drugs, intra-articular corticosteroids. The purpose of this systematic review was to evaluate the comparative effectiveness and safety of DMARDs versus conventional therapy and versus other DMARDs. Results A systematic evidence review of 156 reports identified in MEDLINE®, EMBASE®, and by hand searches. There is some evidence that methotrexate is superior to conventional therapy. Among children who have responded to a biologic DMARD, randomized discontinuation trials suggest that continued treatment decreases the risk of having a flare. However, these studies evaluated DMARDs with different mechanisms of action (abatacept, adalimumab, anakinra, etanercept, intravenous immunoglobulin, tocilizumab and used varying comparators and follow-up periods. Rates of serious adverse events are similar between DMARDs and placebo in published trials. This review identified 11 incident cases of cancer among several thousand children treated with one or more DMARD. Conclusions Few data are available to evaluate the comparative effectiveness of either specific DMARDs or general classes of DMARDs. However, based on the overall number, quality, and consistency of studies, there is moderate strength of evidence to support that DMARDs improve JIA-associated symptoms. Limited data suggest that short-term risk of cancer is low.

  6. Juvenile idiopathic arthritis in adulthood: fulfilment of classification criteria for adult rheumatic diseases, long-term outcomes and predictors of inactive disease, functional status and damage

    Science.gov (United States)

    Oliveira-Ramos, Filipa; Eusébio, Mónica; M Martins, Fernando; Mourão, Ana Filipa; Furtado, Carolina; Campanilho-Marques, Raquel; Cordeiro, Inês; Ferreira, Joana; Cerqueira, Marcos; Figueira, Ricardo; Brito, Iva; Santos, Maria José; Melo-Gomes, José A; Fonseca, João Eurico

    2016-01-01

    Objectives To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. Methods Patients with JIA registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index—articular (JADI-A) and Juvenile Arthritis Damage Index—extra-articular (JADI-E) damage index and disease activity were analysed. Results 426 patients were included. Most of patients with systemic JIA fulfilled criteria for Adult Still's disease. 95.6% of the patients with rheumatoid factor (RF)-positive polyarthritis and 57.1% of the patients with RF-negative polyarthritis matched criteria for rheumatoid arthritis (RA). 38.9% of the patients with extended oligoarthritis were classified as RA while 34.8% of the patients with persistent oligoarthritis were classified as spondyloarthritis. Patients with enthesitis-related arthritis fulfilled criteria for spondyloarthritis in 94.7%. Patients with psoriatic arthritis maintained this classification. Patients with inactive disease had lower disease duration, lower diagnosis delay and corticosteroids exposure. Longer disease duration was associated with higher HAQ, JADI-A and JADI-E. Higher JADI-A was also associated with biological treatment and retirement due to JIA disability and higher JADI-E with corticosteroids exposure. Younger age at disease onset was predictive of higher HAQ, JADI-A and JADI-E and decreased the chance of inactive disease. Conclusions Most of the included patients fulfilled classification criteria for adult rheumatic diseases, maintain active disease and have functional impairment. Younger age at disease onset was predictive

  7. Cartilage oligomeric matrix protein in patients with juvenile idiopathic arthritis: relation to growth and disease activity

    DEFF Research Database (Denmark)

    Bjørnhart, Birgitte; Juul, Anders; Nielsen, Susan

    2009-01-01

    OBJECTIVE: Cartilage oligomeric matrix protein (COMP) has been identified as a prognostic marker of progressive joint destruction in rheumatoid arthritis. In this population based study we evaluated associations between plasma concentrations of COMP, disease activity, and growth velocity in patie......OBJECTIVE: Cartilage oligomeric matrix protein (COMP) has been identified as a prognostic marker of progressive joint destruction in rheumatoid arthritis. In this population based study we evaluated associations between plasma concentrations of COMP, disease activity, and growth velocity...

  8. Juvenile Idiopathic Arthritis

    Science.gov (United States)

    ... Physical Therapy Regular Exercise en español Artritis idiopática juvenil It may begin with a swollen knuckle, a ... may suddenly appear and disappear, developing in one area and then another. High fevers that tend to ...

  9. The significance of elevated serologic markers of celiac disease in children with juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Al-Mayouf Sulaiman

    2003-01-01

    Full Text Available Aim: The aim of this study is to determine the frequency of celiac disease (CD in a group of children with juvenile rheumatoid arthritis (JRA and determine the correlation between the presence of the serologic markers and the histological diagnosis of CD. Patients and Methods : Forty-two children (24 females with JRA, aged between 5-15 years underwent study of serologic markers for CD (gliadin-IgA, gliadin-IgG, reticulin and endomysium-IgA antibodies. Endoscopic intestinal biopsy was performed in patients who had positive serologic markers for CD. The diagnosis of CD was based on the classic finding of villous atrophy and crypt hypertrophy. Results: Eighteen patients (42.8% had serologic markers for CD; ten of them with a systemic form, five with a polyarticular form and three with a pauciarticular form of JRA. Levels of AGA -IgG were high in 14 patients (77.8%, four patients (22.2% had high levels of AGA-IgA and seven patients (38.9% had anti-endomysium antibodies (AEA. One patient had anti-reticulin antibodies (ARA 5.5%. Sixteen patients underwent intestinal biopsy; in only one patient with AEA antibodies (2.38%, biopsy revealed typical finding of CD. The patient with CD showed improvement in both growth parameter as well as articular symptoms after starting gluten-free diet Conclusion: Our study shows that the screening for silent CD among children with JRA may be useful. Those patients with AEA need further follow up since these antibodies are quite sensitive and specific for CD

  10. Alteration of Fecal Microbiota Profiles in Juvenile Idiopathic Arthritis. Associations with HLA-B27 Allele and Disease Status

    Science.gov (United States)

    Di Paola, Monica; Cavalieri, Duccio; Albanese, Davide; Sordo, Maddalena; Pindo, Massimo; Donati, Claudio; Pagnini, Ilaria; Giani, Teresa; Simonini, Gabriele; Paladini, Alessia; Lionetti, Paolo; De Filippo, Carlotta; Cimaz, Rolando

    2016-01-01

    Alteration of gut microbiota is involved in several chronic inflammatory and autoimmune diseases, including rheumatoid arthritis, and gut microbial “pro-arthritogenic” profiles have been hypothesized. Intestinal inflammation may be involved in spondyloarthropathies and in a subset of patients affected by Juvenile Idiopathic Arthritis (JIA), the most common chronic rheumatic disease of childhood. We compared the fecal microbiota composition of JIA patients with healthy subjects (HS), evaluating differences in microbial profiles between sub-categories of JIA, such as enthesitis-related arthritis (JIA-ERA), in which inflammation of entheses occurs, and polyarticular JIA, non-enthesitis related arthritis (JIA-nERA). Through taxon-level analysis, we discovered alteration of fecal microbiota components that could be involved in subclinical gut inflammation, and promotion of joint inflammation. We observed abundance in Ruminococcaceae in both JIA categories, reduction in Clostridiaceae and Peptostreptococcaceae in JIA-ERA, and increase in Veillonellaceae in JIA-nERA, respectively, compared with HS. Among the more relevant genera, we found an increase in Clostridium cluster XIVb, involved in colitis and arthritis, in JIA-ERA patients compared with HS, and a trend of decrease in Faecalibacterium, known for anti-inflammatory properties, in JIA-nERA compared with JIA-ERA and HS. Differential abundant taxa identified JIA patients for the HLA-B27 allele, including Bilophila, Clostridium cluster XIVb, Oscillibacter, and Parvimonas. Prediction analysis of metabolic functions showed that JIA-ERA metagenome was differentially enriched in bacterial functions related to cell motility and chemotaxis, suggesting selection of potential virulence traits. We also discovered differential microbial profiles and intra-group variability among active disease and remission, suggesting instability of microbial ecosystem in autoimmune diseases with respect to healthy status. Similarly to

  11. Alteration of fecal microbiota profiles in juvenile idiopathic arthritis. Associations with HLA-B27 allele and disease status.

    Directory of Open Access Journals (Sweden)

    Monica Di Paola

    2016-10-01

    Full Text Available Alteration of gut microbiota is involved in several chronic inflammatory and autoimmune diseases, including rheumatoid arthritis, and gut microbial pro-arthritogenic profiles have been hypothesized. Intestinal inflammation may be involved in spondyloarthropathies and in a subset of patients affected by Juvenile Idiopathic Arthritis (JIA, the most common chronic rheumatic disease of childhood. We compared the fecal microbiota composition of JIA patients with healthy subjects (HS, evaluating differences in microbial profiles between sub-categories of JIA, such as enthesitis-related arthritis (JIA-ERA, in which inflammation of entheses occurs, and polyarticular JIA, non-enthesitis related arthritis (JIA-nERA. Through taxon-level analysis, we discovered alteration of fecal microbiota components that could be involved in subclinical gut inflammation, and promotion of joint inflammation. We observed abundance in Ruminococcaceae in both JIA categories, reduction in Clostridiaceae and Peptostreptococcaceae in JIA-ERA, and increase in Veillonellaceae in JIA-nERA, respectively compared with HS. Among the more relevant genera, we found an increase in Clostridium cluster XIVb, involved in colitis and arthritis, in JIA-ERA patients compared with HS, and a trend of decrease in Faecalibacterium, known for anti-inflammatory properties, in JIA-nERA compared with JIA-ERA and HS. Differential abundant taxa identified JIA patients for the HLA-B27 allele, including Bilophila, Clostridium cluster XIVb, Oscillibacter and Parvimonas. Prediction analysis of metabolic functions showed that JIA-ERA metagenome was differentially enriched in bacterial functions related to cell motility and chemotaxis, suggesting selection of potential virulence traits. We also discovered differential microbial profiles and intra-group variability among active disease and remission, suggesting instability of microbial ecosystem in autoimmune diseases with respect to healthy status. Similarly

  12. Cytokine profiles in peripheral blood and whole blood cell cultures associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Sørensen, Lars Korsbaek; Stoltze, Kaj

    2005-01-01

    Cytokines play a key role in the pathogenesis of inflammatory diseases. An obvious question is whether patients with aggressive periodontitis, juvenile idiopathic arthritis, or rheumatoid arthritis share blood cytokine profiles distinguishing them from individuals free of disease....

  13. Cytokine profiles in peripheral blood and whole blood cell cultures associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Sørensen, Lars Korsbaek; Stoltze, Kaj

    2005-01-01

    Cytokines play a key role in the pathogenesis of inflammatory diseases. An obvious question is whether patients with aggressive periodontitis, juvenile idiopathic arthritis, or rheumatoid arthritis share blood cytokine profiles distinguishing them from individuals free of disease....

  14. Ongoing disease activity and changing categories in a long-term nordic cohort study of juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Nordal, Ellen; Zak, Marek; Aalto, Kristiina

    2011-01-01

    OBJECTIVE: The aim of the study was to describe disease characteristics, long-term course and outcome of juvenile idiopathic arthritis (JIA) in a population-based setting. METHODS: Consecutive cases of JIA from defined geographical areas of Denmark, Finland, Sweden and Norway with disease onset...... repeated visits with last visit more than seven years after disease onset (median 98 months, range 84-147). Changes in ILAR category occurred in 10.8% of the children, in addition to extended oligoarthritis developing in 34.8% of the oligoarticular group. Disease modifying anti-rheumatic drugs (DMARD......), including biologic medications, were used in 58.0% of the children during the observation period. Ongoing disease activity was mostly mild, but 22.9% developed some JIA-related damage. At the last follow-up, remission off medication was found in 42.4% of the children, 8.9% were in remission on medication...

  15. Report - Recurrent hip arthritis diagnosed as juvenile idiopathic arthritis: A case report.

    Science.gov (United States)

    Chang, Tung-Ming; Yang, Kuender D; Yong, Su-Boon

    2016-05-01

    Juvenile idiopathic arthritis is the most common rheumatic disease in childhood. It is a chronic inflammatory disease associated with arthritis of unknown etiology that begins before the age of 16 and persists for longer than 6 weeks. In this report, the case of a child who suffered recurrent alternative hip arthritis with bilateral hip arthritis is examined, in which he was finally diagnosed as suffering from Juvenile idiopathic arthritis. A 14-year-old boy of Taiwanese origin presented with a normal birth and developmental history. At the age of 10, right-side hip joint pain was experienced, which later migrated to the left side. On further inspection, synovium hypertrophy, cartilage erosion and hip turbid fluid accumulation were found and aseptic arthritis was presumed to be the primary cause. However, after re-examining both his clinical history and presentation, Juvenile idiopathic arthritis was the final diagnosis. Any child presenting with repeat joint swelling are at risk of Juvenile idiopathic arthritis. This is still to be the case if symptoms recede or heal and no initial diagnosis is made. Therefore, a better understanding of the risk of recurrent arthritis is needed. It cannot be emphasized strongly enough that Juvenile idiopathic arthritis should be suspected at all times when a child suffers from recurrent aseptic arthritis of the hip joint.

  16. Juvenile idiopathic arthritis overview and involvement of the temporomandibular joint: prevalence, systemic therapy.

    Science.gov (United States)

    Carrasco, Ruy

    2015-02-01

    The temporomandibular joint (TMJ) is one of the many joints involved in the inflammatory arthritides. As imaging of joints has developed, so have the data regarding extent and prevalence of TMJ involvement in these diseases. TMJ disease is especially prevalent in juvenile arthritis. The adult and pediatric inflammatory arthritides share common pathophysiology but are still markedly different. The preponderance of TMJ arthritis research exists in juvenile arthritis. This article discusses classification, treatment, and TMJ involvement in juvenile idiopathic arthritis.

  17. Systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Cimaz, Rolando

    2016-09-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a systemic inflammatory disease which has up to now been classified as a category of juvenile idiopathic arthritis. However, in this context, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it may rather be part of the spectrum of autoinflammatory disorders. The disease is in fact unique with regard to the other JIA categories, in terms of clinical manifestations, prognosis, and response to conventional immunosuppressant therapies. It is characterized clinically by fever, lymphadenopathy, arthritis, rash, and serositis. IL-1 and IL-6 play a major role in the pathogenesis of SoJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SoJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation continue to be a major issue in patients' care. Recent advances on the pathogenesis and treatment have revolutionized the care and prognosis of this potentially life-threatening pediatric condition.

  18. Juvenile rheumatoid arthritis: therapeutic perspectives.

    Science.gov (United States)

    Chikanza, Ian C

    2002-01-01

    Juvenile rheumatoid arthritis (JRA) is the most common childhood chronic systemic autoimmune inflammatory disease. The therapeutic approach to JRA has, to date, been casual and based on extensions of clinical experiences gained in the management of adult rheumatoid arthritis (RA). The physiology of inflammation has been systemically studied and this has led to the identification of specific therapeutic targets and the development of novel approaches to the management of JRA. The classical treatments of the disease such as methotrexate, sodium aurothiomalate and sulfasalazine, are not always effective in controlling RA and JRA. This has necessitated the development of novel agents for treating RA, most of which are biological in nature and are targeted at specific sites of the inflammatory cascades. These biological therapeutic strategies in RA have proved successful and are being applied in the management of JRA. These developments have been facilitated by the advances in molecular biology which have heralded the advent of biodrugs (recombinant proteins) and gene therapy, in which specific genes can be introduced locally to enhance in vivo gene expression or suppress gene(s) of interest with a view to down-regulating inflammation. Some of these biodrugs, such as anti-tumor necrosis factor alpha (anti-TNFalpha), monoclonal antibodies (infliximab, adalimumab), TNF soluble receptor constructs (etanercept) and interleukin-1 receptor antagonist (IL-1Ra) have been tested and shown to be effective in RA. Etanercept has now been licensed for JRA. Clinical trials of infliximab in JRA are planned. Studies show that the clinical effects are transient, necessitating repeated treatments and the risk of vaccination effects. Anti-inflammatory cytokines such as IL-4, IL-10, transforming growth factor-beta and interferon-beta (IFN-beta) are undergoing clinical trials. Many of these agents have to be administered parenterally and production costs are very high; thus, there is a need

  19. Bite force and temporomandibular disorder in juvenile chronic arthritis.

    Science.gov (United States)

    Wenneberg, B; Kjellberg, H; Kiliaridis, S

    1995-08-01

    The aim of this study was to investigate the functional condition of the stomatognathic system in children suffering from juvenile chronic arthritis, with respect to bite force and temporomandibular disorder in relation to radiographic abnormalities of the mandibular condyle, occlusal factors and systemic disease parameters. Thirty-five children with juvenile chronic arthritis were compared to 89 healthy children with an Angle Class I occlusion and 62 children with an Angle Class II malocclusion. Subjective symptoms and clinical signs of temporomandibular disorder and radiographic mandibular condylar changes were more common in children with juvenile chronic arthritis than in the two comparison groups. Maximal molar and incisal bite forces and maximal molar bite force endurance times were also significantly reduced in children with juvenile chronic arthritis. It is concluded that the differences between the groups are caused mainly by the systemic inflammatory disease itself, but a functional influence of weakened masticatory muscles cannot be excluded.

  20. Extremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases

    Directory of Open Access Journals (Sweden)

    Y. Xia

    Full Text Available The aim of this research was to explore whether IL-18 can be a serological marker for the diagnosis of systemic-onset juvenile idiopathic arthritis (sJIA. A total of 23 sJIA patients (13 males, median age 8.2, 20 acute lymphoblastic leukemia (ALL patients, 18 patients with severe infections (SIF, 26 Kawasaki disease (KD patients, 18 juvenile idiopathic arthritis (JIA patients, and 25 healthy control patients were selected for this study. Enzyme-linked immunosorbent assays (ELISAs were used to determine the serum concentrations of the S100A8, S100A9, and IL-6 proteins. The serum IL-18 levels were detected by a cytometric bead array (CBA. The serum IL-6 concentrations in various disease groups were significantly higher than that in the healthy control group. The IL-6 concentrations exhibited no significant difference between disease groups. The S100A8 level in the sJIA group was significantly higher than those of the ALL, JIA, and healthy control groups but showed no significant difference compared to the SIF and KD groups. The S100A9 serum concentration in the sJIA group was significantly higher than those in the ALL and healthy control groups and exhibited no significant difference from the SIF, KD, and JIA groups. The IL-18 level of the sJIA group was significantly higher than that of the other febrile disease groups. The IL-18 serum concentration may be used as a biological serum marker to distinguish sJIA from other febrile diseases.

  1. Extremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases

    Science.gov (United States)

    Xia, Y.; Cui, P.; Li, Q.; Liang, F.; Li, C.; Yang, J.

    2017-01-01

    The aim of this research was to explore whether IL-18 can be a serological marker for the diagnosis of systemic-onset juvenile idiopathic arthritis (sJIA). A total of 23 sJIA patients (13 males, median age 8.2), 20 acute lymphoblastic leukemia (ALL) patients, 18 patients with severe infections (SIF), 26 Kawasaki disease (KD) patients, 18 juvenile idiopathic arthritis (JIA) patients, and 25 healthy control patients were selected for this study. Enzyme-linked immunosorbent assays (ELISAs) were used to determine the serum concentrations of the S100A8, S100A9, and IL-6 proteins. The serum IL-18 levels were detected by a cytometric bead array (CBA). The serum IL-6 concentrations in various disease groups were significantly higher than that in the healthy control group. The IL-6 concentrations exhibited no significant difference between disease groups. The S100A8 level in the sJIA group was significantly higher than those of the ALL, JIA, and healthy control groups but showed no significant difference compared to the SIF and KD groups. The S100A9 serum concentration in the sJIA group was significantly higher than those in the ALL and healthy control groups and exhibited no significant difference from the SIF, KD, and JIA groups. The IL-18 level of the sJIA group was significantly higher than that of the other febrile disease groups. The IL-18 serum concentration may be used as a biological serum marker to distinguish sJIA from other febrile diseases. PMID:28225869

  2. Treatment in juvenile rheumatoid arthritis and new treatment options.

    Science.gov (United States)

    Kasapçopur, Özgür; Barut, Kenan

    2015-03-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of the childhood with the highest risk of disability. Active disease persists in the adulthood in a significant portion of children with juvenile rheumatoid arthritis despite many developments in the diagnosis and treatment. Therefore, initiation of efficient treatment in the early period of the disease may provide faster control of the inflammation and prevention of long-term harms. In recent years, treatment options have also increased in children with juvenile idiopathic arthritis owing to biological medications. All biological medications used in children have been produced to target the etiopathogenesis leading to disease including anti-tumor necrosis factor, anti-interleukin 1 and anti-interleukin 6 drugs. In this review, scientific data about biological medications used in the treatment of rheumatoid arthritis and new treatment options will be discussed.

  3. Treatment in juvenile rheumatoid arthritis and new treatment options

    Science.gov (United States)

    Kasapçopur, Özgür; Barut, Kenan

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of the childhood with the highest risk of disability. Active disease persists in the adulthood in a significant portion of children with juvenile rheumatoid arthritis despite many developments in the diagnosis and treatment. Therefore, initiation of efficient treatment in the early period of the disease may provide faster control of the inflammation and prevention of long-term harms. In recent years, treatment options have also increased in children with juvenile idiopathic arthritis owing to biological medications. All biological medications used in children have been produced to target the etiopathogenesis leading to disease including anti-tumor necrosis factor, anti-interleukin 1 and anti-interleukin 6 drugs. In this review, scientific data about biological medications used in the treatment of rheumatoid arthritis and new treatment options will be discussed. PMID:26078691

  4. Effects of the live attenuated measles-mumps-rubella booster vaccination on disease activity in patients with juvenile idiopathic arthritis : a randomized trial

    NARCIS (Netherlands)

    Heijstek, Marloes W; Kamphuis, Sylvia; Armbrust, Wineke; Swart, Joost; Gorter, Simone; de Vries, Lara D; Smits, Gaby P; van Gageldonk, Pieter G; Berbers, Guy A M; Wulffraat, Nico M

    2013-01-01

    IMPORTANCE: The immunogenicity and the effects of live attenuated measles-mumps-rubella (MMR) vaccination on disease activity in patients with juvenile idiopathic arthritis (JIA) are matters of concern, especially in patients treated with immunocompromising therapies. OBJECTIVES: To assess whether M

  5. GANGGUAN PERTUMBUHAN MANDIBULA PADA JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    Ria Puspitawati

    2015-08-01

    Full Text Available Juvenile Rheumatoid Arthritis (JRA is a systemic disease on childhood, which has chronic arthritis as its most prominent manifestation. One very common complication of JRA is growth disturbance. JRA involving temporomandibular joint usually result in mandibular growth retardation which eventually can lead to micrognathia, retrognathia, malocclusion and other mandibulofacial developmental aberrations. Factors considered to be the cause of these growth and developmental disturbances are: congenital, the disease's direct effect on the condyle, functional deficiency of the temporomandibular joint, duration, age of disease onset and type of the JRA and corticosteroid therapy. However, the mechanism for the mandibulofacial growth and developmental aberrations due to JRA are not fully understood. This literature review will discuss the hypotheses concerning mechanisms of those growth and developmental disturbances, especially based on clinical and radiographic studies on JRA cases.

  6. Juvenile Idiopathic Arthritis

    Science.gov (United States)

    ... of the inside of the eye/s is called iritis or anterior uveitis. While uveitis usually causes symptoms ... treating diseases of the eye) to check for iritis in any patient with JIA What are the ...

  7. Juvenil idiopatisk arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2002-01-01

    . In addition to the clinical characteristics, genetic and biochemical differences suggest that JIA could be regarded as a general term covering various diseases. Complications described are uveitis, temporomandibular joint affection and growth disturbances. The therapeutic strategy should be planned...

  8. Juvenile idiopathic arthritis: the paediatric perspective

    Energy Technology Data Exchange (ETDEWEB)

    Jordan, Alison [Birmingham Children' s Hospital, Department of Adolescent Rheumatology, Birmingham (United Kingdom); McDonagh, Janet E. [Birmingham Children' s Hospital, Institute of Child Health, Birmingham (United Kingdom)

    2006-08-15

    Paediatric rheumatology is a relatively new specialty that has developed rapidly over the last 30 years. There have been major advances, which have included improvements in the classification and management of juvenile idiopathic arthritis (JIA). The former has led to enhanced international collaboration with disease registries, multicentre research and the development of new therapeutic agents. This has resulted in improved disease control and remission induction in many. There is, however, still significant morbidity associated with JIA during childhood, adolescence and adulthood, and challenges for the future include early identification of those with a poorer prognosis, appropriate administration of safe therapies and optimizing outcomes as young people move through adolescence into adulthood. (orig.)

  9. Quantitative MR characterization of disease activity in the knee in children with juvenile idiopathic arthritis: a longitudinal pilot study

    Energy Technology Data Exchange (ETDEWEB)

    Workie, Dagnachew W. [University of Cincinnati, Department of Physics, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Imaging Research Center, Cincinnati, OH (United States); Graham, T.B. [Cincinnati Children' s Hospital Medical Center, Division of Rheumatology, Cincinnati, OH (United States); Laor, Tal; Racadio, Judy M. [Cincinnati Children' s Hospital Medical Center, Department of Pediatrics, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Rajagopal, Akila; O' Brien, Kendall J.; Bommer, Wendy A. [Cincinnati Children' s Hospital Medical Center, Imaging Research Center, Cincinnati, OH (United States); Shire, Norah J. [University of Cincinnati, Division of Epidemiology and Biostatistics, Cincinnati, OH (United States); University of Cincinnati, Division of Digestive Diseases, Cincinnati, OH (United States); Dardzinski, Bernard J. [Cincinnati Children' s Hospital Medical Center, Imaging Research Center, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Pediatrics, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States)

    2007-06-15

    The development of a quantifiable and noninvasive method of monitoring disease activity and response to therapy is vital for arthritis management. The purpose of this study was to investigate the utility of quantitative dynamic contrast-enhanced MRI (DCE-MRI) based on pharmacokinetic (PK) modeling to evaluate disease activity in the knee and correlate the results with the clinical assessment in children with juvenile idiopathic arthritis (JIA). A group of 17 children with JIA underwent longitudinal clinical and laboratory assessment and DCE-MRI of the knee at enrollment, 3 months, and 12 months. A PK model was employed using MRI signal enhancement data to give three parameters, K{sup trans} ' (min{sup -1}), k{sub ep} (min{sup -1}), and V{sub p} ' and to calculate synovial volume. The PK parameters, synovial volumes, and clinical and laboratory assessments in most children were significantly decreased (P < 0.05) at 12 months when compared to the enrollment values. There was excellent correlation between the PK and synovial volume and the clinical and laboratory assessments. Differences in MR and clinical parameter values in individual subjects illustrate persistent synovitis when in clinical remission. A decrease in PK parameter values obtained from DCE-MRI in children with JIA likely reflects diminution of disease activity. This technique may be used as an objective follow-up measure of therapeutic efficacy in patients with JIA. MR imaging can detect persistent synovitis in patients considered to be in clinical remission. (orig.)

  10. Cytokines in juvenile rheumatoid arthritis (JRA).

    Science.gov (United States)

    Mangge, H; Schauenstein, K

    1998-06-01

    Juvenile rheumatoid arthritis (JRA), unlike rheumatoid arthritis of adulthood (RA), is a heterogenous disease comprising at least five subtypes that differ in clinical course and prognosis, and require different therapeutical approaches. As compared to RA, the production of local and systemic cytokines in JRA have not yet been as extensively investigated. In this article we review the available literature on cytokine expression in serum and synovial fluid in all five different subtypes of JRA. Even though the data are still fragmentary, the evidence so far suggests that the determination of serum cytokines yields relevant information as to clinical subtype and inflammatory activity of the disease. Furthermore, the cytokine data suggest that the pathogenesis of JRA may even by more heterogenous than defined by the clinical subtypes. Finally, future directions of research in this area are proposed, and-based on the latest results-arguments for (anti)cytokine therapies in JRA are critically discussed.

  11. Tocilizumab in the treatment of systemic juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Murakami M

    2012-07-01

    Full Text Available Miho Murakami,1 Minako Tomiita,2,3 Norihiro Nishimoto11Laboratory of Immune Regulation, Wakayama Medical University, Wakayama, 2Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, 3Department of Allergy and Rheumatology, Chiba Children's Hospital, Chiba, JapanAbstract: Systemic juvenile idiopathic arthritis is one of the common rheumatic diseases in childhood and characterized by spiking fever, evanescent skin rash, lymphadenopathy, hepatosplenomegaly, and serositis, in addition to arthritis. Children with systemic juvenile idiopathic arthritis often show growth retardation and developmental abnormality, as well as macrophage activation syndrome, a life-threatening complication. Overproduction of interleukin-6 is pathologically responsible for the systemic inflammatory manifestations and abnormal laboratory results with systemic juvenile idiopathic arthritis. Thus, tocilizumab, a humanized antihuman interleukin-6 receptor antibody, has been developed as a therapeutic agent for the disease. A series of clinical studies have demonstrated the excellent efficacy and safety of tocilizumab for patients with active disease. Tocilizumab was approved for systemic juvenile idiopathic arthritis in Japan in 2008 and in the European Union and the United States in 2011.Keywords: systemic juvenile idiopathic arthritis, tocilizumab, antihuman interleukin-6 receptor antibody, biologics

  12. Diagnosis and classification of juvenile idiopathic arthritis.

    Science.gov (United States)

    Eisenstein, Eli M; Berkun, Yackov

    2014-01-01

    In recent years, it has become increasingly clear that the term Juvenile Idiopathic Arthritis (JIA) comprises not one disease but several. Moreover, recent studies strongly suggest that some of these clinico-pathophysiologic entities appear to cross current diagnostic categories. The ultimate goal of the JIA classification is to facilitate development of better, more specific therapy for different forms of disease though improved understanding of pathophysiology. The past two decades have witnessed significant advances in treatment and improved outcomes for many children with chronic arthritis. However, understanding of the basic biologic processes underlying these diseases remains far from complete. As a result, even the best biologic agents of today represent "halfway technologies". Because they do not treat fundamental biologic processes, they are inherently expensive, need to be given for a long time in order to ameliorate the adverse effects of chronic inflammation, and do not cure the disease. Pediatric rheumatology is now entering an era in which diagnostic categories may need to change to keep up with discovery. A more precise, biologically based classification is likely to contribute to development of more specific and improved treatments for the various forms of childhood arthritis. In this review, we discuss how genetic, gene expression, and immunologic findings have begun to influence how these diseases are understood and classified.

  13. Juvenile idiopathic arthritis and the temporomandibular joint ...

    African Journals Online (AJOL)

    ... resonance imaging findings of temporomandibular joint inflammation among juvenile ... The mean total MRI score was significantly higher in patients with active ... Clinical signs of TMJ arthritis can be used as filter for MRI examination TMJ is ...

  14. Managing juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Hawkins, Madeleine J; Dick, Andrew D; Lee, Richard J W; Ramanan, Athimalaipet V; Carreño, Ester; Guly, Catherine M; Ross, Adam H

    2016-01-01

    Bilateral chronic anterior uveitis is an extra-articular feature of juvenile idiopathic arthritis. Although figures vary, uveitis occurs in approximately 11%-13% of patients with this disease and is most commonly associated with the female gender, oligoarthritis, and presence of antinuclear antibodies. The disease has an insidious onset and is often asymptomatic. Managing patients with juvenile idiopathic arthritis-associated uveitis remains challenging as the disease may prove to be refractory to traditional treatment regimens. Stepwise immunomodulatory therapy is indicated, with new biologic drugs being used last in cases of refractory uveitis. Small scale studies and practice have provided the evidence to undertake randomized control trials to evaluate the efficacy, safety, and cost-effectiveness of anti-tumor necrosis factor-α therapies, such as infliximab and adalimumab. These have demonstrated promising results, with further data awaited from ongoing trials for adalimumab (as SYCAMORE and ADJUVITE trials). Lower grade evidence is supporting the use of newer biologics such as rituximab, daclizumab, tocilizumab, and abatacept in those cases refractory to anti-tumor necrosis factor-α therapy.

  15. Exercise testing and fitness training in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Singh-Grewal, D.

    2010-01-01

    Juvenile Idiopathic Arthritis is the commonest rheumatic disease of childhood affecting 1:1000 children under the age of 16 years. Children with JIA have long been sidelined from physical activity due to active disease or irrational concerns that activity may in some way worsen disease. Children

  16. Exercise testing and fitness training in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Singh-Grewal, D.

    2010-01-01

    Juvenile Idiopathic Arthritis is the commonest rheumatic disease of childhood affecting 1:1000 children under the age of 16 years. Children with JIA have long been sidelined from physical activity due to active disease or irrational concerns that activity may in some way worsen disease. Children wit

  17. HLA-B27 predicts a more extended disease with increasing age at onset in boys with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Berntson, Lillemor; Damgård, Michael; Andersson-Gäre, Boel

    2008-01-01

    OBJECTIVE: Juvenile idiopathic arthritis (JIA) is a heterogeneous condition with very few clinical and laboratory signs that can help predict the course and severity of the disease in the individual patient. The cell-surface antigen HLA-B27 is well known to be associated with spondyloarthropathies...... to a population-based study as possible. METHODS: We studied an incidence-based cohort of 305 patients collected prospectively in 3 Nordic countries (Sweden, Norway, Denmark). Clinical and serological data of the first 3 years of the disease were collected. RESULTS: HLA-B27 was found to be positive in 25.......5% of the patients, and we found a higher proportion of HLA-B27-positive boys with older age at disease onset (p=0.034). Regression analysis showed a correlation of 0.7 in the HLA-B27-positive boys, pointing to a higher risk of more joint involvement with older age at disease onset. By Fisher's exact test...

  18. Synovial and inflammatory diseases in childhood: role of new imaging modalities in the assessment of patients with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Damasio, Maria Beatrice [G. Gaslini Institute, Department of Diagnostic Imaging, Genoa (Italy); Malattia, Clara [G. Gaslini Institute, Department of Pediatrics 2, Genoa (Italy); Martini, Alberto [University of Genova, Department of Pediatrics, Genoa (Italy); Toma, Paolo [Bambin Gesu Pediatric Hospital, Rome (Italy)

    2010-06-15

    Juvenile idiopathic arthritis (JIA) represents a group of heterogeneous diseases characterized by a chronic inflammatory process primarily targeting the synovial membrane. A persistent synovitis is associated with an increased risk of osteocartilaginous damage. With the advent of effective structure-modifying treatment for JIA, it may be possible to significantly reduce or even completely prevent structural damage and associated functional disability. The trend towards early suppression of inflammation, in order to prevent erosive disease, shifts the emphasis away from conventional radiographic detectable structural damage to the slightest traces of early joint damage, and drives the need for alternative imaging techniques more sensitive in detecting early signs of disease activity and damage. In this regard MRI and US are playing an increasing role in the evaluation of arthritic joints. This article will review the key aspects of the current status and recent important advances of imaging techniques available to investigate the child with rheumatic disease, briefly discussing conventional radiography, and particularly focusing on MRI and US. In this era of advancing imaging technology, knowledge of the relative values of available imaging techniques is necessary to optimize the management of children with JIA. (orig.)

  19. Australian Paediatric Rheumatology Group standards of care for the management of juvenile idiopathic arthritis.

    Science.gov (United States)

    Munro, Jane; Murray, Kevin; Boros, Christina; Chaitow, Jeffrey; Allen, Roger C; Akikusa, Jonathan; Adib, Navid; Piper, Susan E; Singh-Grewal, Davinder

    2014-09-01

    This standards document outlines accepted standards of management for children, adolescents and young adults with juvenile idiopathic arthritis (JIA) in Australia. This document acknowledges that the chronic inflammatory arthritis conditions (JIA) in childhood are different diseases from inflammatory arthritis in adults and that specific expertise is required in the care of children with arthritis.

  20. Clinical outcome measures in juvenile idiopathic arthritis.

    Science.gov (United States)

    Consolaro, Alessandro; Giancane, Gabriella; Schiappapietra, Benedetta; Davì, Sergio; Calandra, Serena; Lanni, Stefano; Ravelli, Angelo

    2016-04-18

    Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with significant disease- and treatment-related morbidity, thus impacting children's quality of life. In order to optimize JIA management, the paediatric rheumatologist has begun to regularly use measurements of disease activity developed, validated and endorsed by international paediatric rheumatology professional societies in an effort to monitor the disease course over time and assess the efficacy of therapeutic interventions in JIA patients.A literature review was performed to describe the main outcome measures currently used in JIA patients to determine disease activity status.The Juvenile Disease Activity Score (JADAS), in its different versions (classic JADAS, JADAS-CRP and cJADAS) and the validated definitions of disease activity and response to treatment represent an important tool for the assessment of clinically relevant changes in disease activity, leading more and more to a treat-to-target strategy, based on a tight and thorough control of the patient condition. Moreover, in recent years, increasing attention on the incorporation of patient-reported or parent-reported outcomes (PRCOs), when measuring the health state of patients with paediatric rheumatic diseases has emerged.We think that the care of JIA patients cannot be possible without taking into account clinical outcome measures and, in this regard, further work is required.

  1. The conundrum of juvenile psoriatic arthritis.

    Science.gov (United States)

    Ravelli, Angelo; Consolaro, Alessandro; Schiappapietra, Benedetta; Martini, Alberto

    2015-01-01

    Juvenile psoriatic arthritis (JPsA) has provided paediatric rheumatologists with a controversial topic for many years. The principal area of contention centres on the discordance between its treatment as a single diagnostic category in current classification schemes and the demonstration of its heterogeneous nature. A further point of debate is the distinctiveness of JPsA as an entity. Owing to these uncertainties, the concept of JPsA has evolved over the years and there have been several changes in its definition and diagnostic criteria. Recently, strong evidence has been provided that the spectrum of JPsA include at least two distinct subgroups, one that has the same characteristics as early-onset ANA-positive JIA, and another that is part of the spectrum of spondyloarthropathies and resembles the forms of psoriatic arthritis in adults that belong to the same disease family. These findings call for a revision of the classification of childhood arthritis, that refutes the assumptions that children with JPsA constitute a single homogeneous population and that JPsA should be considered an individual disease entity.

  2. Contrast-enhanced MRI compared with the physical examination in the evaluation of disease activity in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hemke, Robert; Maas, Mario [Academic Medical Centre, University of Amsterdam, Department of Radiology, Amsterdam (Netherlands); Veenendaal, Mira van; Kuijpers, Taco W. [University of Amsterdam, Department of Paediatric Haematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Dolman, Koert M. [Department of Paediatric Rheumatology, Amsterdam (Netherlands); St. Lucas Andreas Hospital, Department of Paediatrics, Amsterdam (Netherlands); Rossum, Marion A.J. van; Berg, J.M. van den [University of Amsterdam, Department of Paediatric Haematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Department of Paediatric Rheumatology, Amsterdam (Netherlands)

    2014-02-15

    To assess the value of magnetic resonance imaging (MRI) in discriminating between active and inactive juvenile idiopathic arthritis (JIA) patients and to compare physical examination outcomes with MRI outcomes in the assessment of disease status in JIA patients. Consecutive JIA patients with knee involvement were prospectively studied using an open-bore MRI. Imaging findings from 146 JIA patients were analysed (59.6 % female; mean age, 12.9 years). Patients were classified as clinically active or inactive. MRI features were evaluated using the JAMRIS system, comprising validated scores for synovial hypertrophy, bone marrow oedema, cartilage lesions and bone erosions. Inter-reader reliability was good for all MRI features (intra-class correlation coefficient [ICC] = 0.87-0.94). No differences were found between the two groups regarding MRI scores of bone marrow oedema, cartilage lesions or bone erosions. Synovial hypertrophy scores differed significantly between groups (P = 0.016). Nonetheless, synovial hypertrophy was also present in 14 JIA patients (35.9 %) with clinically inactive disease. Of JIA patients considered clinically active, 48.6 % showed no signs of MRI-based synovitis. MRI can discriminate between clinically active and inactive JIA patients. However, physical examination is neither very sensitive nor specific in evaluating JIA disease activity compared with MRI. Subclinical synovitis was present in >35 % of presumed clinically inactive patients. (orig.)

  3. Serum ferritin levels as a useful diagnostic marker for the distinction of systemic juvenile idiopathic arthritis and Kawasaki disease.

    Science.gov (United States)

    Mizuta, Mao; Shimizu, Masaki; Inoue, Natsumi; Kasai, Kazuko; Nakagishi, Yasuo; Takahara, Tadamori; Hamahira, Kiyoshi; Yachie, Akihiro

    2016-11-01

    The clinical features and laboratory parameters of patients with Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (s-JIA) tend to overlap. Furthermore, there have been no definitive biomarkers for these diseases, making clinical diagnosis difficult. The purpose of this study was to investigate the diagnostic value of serum ferritin levels for differentiating KD from s-JIA and predicting the disease severity of KD. We analyzed 228 patients with KD and 81 patients with s-JIA. Serum ferritin levels were compared between patients with s-JIA and KD. Furthermore, serum ferritin levels in patients with KD were compared with respect to clinical features such as responsiveness to intravenous immunoglobulin (IVIG) therapy. Serum ferritin levels in KD patients with no response to IVIG therapy were significantly higher than those in KD patients with a good response to IVIG therapy. Serum ferritin levels in patients with KD needing plasma exchange (PE) were significantly higher than those in patients not needing PE. However, serum ferritin levels overlapped between severe KD patients with nonresponsiveness to IVIG therapy or needing PE and other patients with mild KD. Furthermore, patients with s-JIA showed a distinct elevation of serum ferritin levels compared with KD patients. The cutoff value of serum ferritin levels for differentiating KD from s-JIA was 369.6 ng/ml. Serum ferritin levels were significantly elevated in s-JIA patients compared with KD patients. Measurement of serum ferritin levels can be useful for differentiating s-JIA from KD.

  4. Temporomandibular Involvement and Craniofacial Development in Juvenile Idiopathic Arthritis

    NARCIS (Netherlands)

    M. Twilt (Marinka)

    2006-01-01

    textabstractJuvenile Idiopathic Arthritis (JIA) is a generalised autoimmune disease, which starts in childhood. JIA is one of the most frequent occurring autoimmune diseases in childhood, and concerns approximately 1 in a 1000 children. JIA is a heterogeneous group of conditions divided into seve

  5. Genetic Screening of Mutations Associated with Fabry Disease in a Nationwide Cohort of Juvenile Idiopathic Arthritis Patients

    Science.gov (United States)

    Gonçalves, Maria J.; Mourão, Ana F.; Martinho, António; Simões, Olívia; Melo-Gomes, José; Salgado, Manuel; Estanqueiro, Paula; Ribeiro, Célia; Brito, Iva; Fonseca, João E.; Canhão, Helena

    2017-01-01

    Fabry’s disease (FD) is a lysosomal storage disorder associated with an alpha-galactosidase A deficiency. The prevalence of FD among juvenile idiopathic arthritis (JIA) patients with established diagnosis is unknown, but as musculoskeletal pain may be an important complaint at presentation, misdiagnosed cases are anticipated. With this study, we aim to calculate the frequency of FD-associated mutations in a cohort of JIA patients. Children with JIA from a national cohort were selected. Clinical and laboratorial information was recorded in the Portuguese rheumatic diseases register (http://Reuma.pt). Molecular genetic testing to detect GLA gene mutations was performed. After the multiplex polymerase chain reactions technique for DNA amplification, direct sequencing of the complete sequence of GLA gene was completed. From a cohort of 292 patients with JIA (188 females, 104 males), mutations were identified in 5 patients (all female). Four patients had the mutation D313Y, a rare GLA variant, which is associated with low enzymatic levels in plasma, but normal lysosomal levels. One patient presented the missense mutation R118C, which was previously described in Mediterranean patients with FD. This is the first screening of FD mutations in a cohort of JIA patients. No “classic” pathogenic FD mutations were reported. The late-onset FD-associated mutation, R118C, was found in a frequency of 0.34% (1/292). PMID:28299312

  6. Ocular complications and visual outcome in juvenile chronic arthritis: a 25-year follow-up study

    DEFF Research Database (Denmark)

    Zak, Marek; Fledelius, Hans; Pedersen, Freddy Karup

    2003-01-01

    Assessment of longterm ophthalmic outcome in juvenile chronic arthritis (JCA) with emphasis on visual acuity and identification of disease-related parameters associated with rheumatic eye affection.......Assessment of longterm ophthalmic outcome in juvenile chronic arthritis (JCA) with emphasis on visual acuity and identification of disease-related parameters associated with rheumatic eye affection....

  7. Assessing the likelihood of new-onset inflammatory bowel disease following tumor necrosis factor-alpha inhibitor therapy for rheumatoid arthritis and juvenile rheumatoid arthritis.

    Science.gov (United States)

    Krishnan, Asha; Stobaugh, Derrick J; Deepak, Parakkal

    2015-04-01

    The association between inhibition of tumor necrosis factor-alpha (TNF-α) in patients with rheumatoid arthritis (RA) and juvenile rheumatoid arthritis (JRA) and the onset of inflammatory bowel disease (IBD) is unclear. We sought to evaluate this association by analyzing adverse events (AEs) reported to the Food and Drug Administration Adverse Event Reporting System (FAERS) with a standardized scoring tool for drug-induced AEs. A search of the FAERS for RA or JRA (January 2003-December 2011) reported with adalimumab, certolizumab pegol, etanercept, golimumab, or infliximab was performed. This dataset was then queried for cases indicating IBD. Full-length reports were accessed using the Freedom of Information Act and organized by age, sex, concomitant medications, co-morbidities, type of TNF-α inhibitor used, and diagnosis/treatment details. The Naranjo score was used to determine whether the drug-induced AEs were definite, probable, possible, or doubtful. There were 158 cases of IBD after TNF-α inhibitor exposure in RA or JRA patients. Use of the Naranjo score revealed that, in a majority of the cases (71.5 %), TNF-α inhibitor exposure was considered a 'possible' cause. A majority of the 'probable cases' in JRA were reported with etanercept (40 patients, 90.91 %). There were no 'definite' cases of anti-TNF-induced IBD. After applying the Naranjo scale, a weak association between new-onset IBD and TNF-α inhibitor therapy in RA patients and a moderately strong association especially with etanercept exposure in JRA patients was observed. However, causality cannot be determined due to limitations of the FAERS and the Naranjo score.

  8. AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Saha, Abhijeet; Chopra, Yogiraj; Theis, Jason D; Vrana, Julie A; Sethi, Sanjeev

    2013-10-01

    We report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive management of the systemic-onset juvenile idiopathic arthritis resulted in improvement in clinical and laboratory parameters. The case represents an unusual cause of nephrotic syndrome in children. Early diagnosis of renal amyloidosis and management of systemic-onset juvenile idiopathic arthritis is paramount to preventing progression of kidney disease.

  9. Measurement of biomarkers in juvenile idiopathic arthritis patients and their significant association with disease severity: a comparative study.

    Science.gov (United States)

    Gilliam, B E; Chauhan, A K; Low, J M; Moore, T L

    2008-01-01

    To evaluate in juvenile idiopathic arthritis (JIA) patients a biomarker panel of anti-cyclic citrullinated peptide (anti-CCP) antibodies, cartilage oligomeric matrix protein (COMP), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), IgM rheumatoid factor (RF), IgG RF, and IgA RF and compare to the presence of joint erosions (JE), joint space narrowing (JSN), and synovitis in order to evaluate aggressive disease. Sixty-eight JIA patients (19 RF positive polyarthritis, 23 RF negative polyarthritis, 17 persistent oligoarthritis, and 9 systemic-onset) were evaluated using the biomarker panel and compared to 18 healthy controls. All RF isotypes, anti-CCP antibodies, and COMP were measured by enzyme-linked immunosorbent assays (ELISA). Statistically significant differences and associations were assessed for each biomarker in relation to JE, JSN, and synovitis. Multiple regression analysis was used to find the variables associated with joint damage and synovitis. Patients with JE and JSN had significantly elevated levels of IgA RF, IgM RF, and anti-CCP antibodies. COMP levels were higher in early disease, but also later in disease in patients with no JE or JSN. ESR, CRP, and IgA RF were significantly elevated in patients with active synovitis. Regression analysis showed IgM RF and disease duration to be associated with JE and JSN. Anti-CCP antibodies and COMP were also associated with JSN. CRP and IgA RF were associated with synovitis. Our findings demonstrate the importance of measuring IgM RF and IgA RF by ELISA and anti-CCP antibodies by ELISA, in addition to COMP in the assessment of JIA patients to determine severity of disease.

  10. Juvenile idiopathic arthritis and oral health

    Directory of Open Access Journals (Sweden)

    Agnieszka Kobus

    2016-05-01

    Full Text Available Juvenile idiopathic arthritis (JIA is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child’s health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity.The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health.

  11. The human microbiome and juvenile idiopathic arthritis.

    Science.gov (United States)

    Verwoerd, Anouk; Ter Haar, Nienke M; de Roock, Sytze; Vastert, Sebastiaan J; Bogaert, Debby

    2016-09-20

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The microbiome has received increasing attention as a potential contributing factor to the development of a wide array of immune-mediated diseases, including inflammatory bowel disease, type 1 diabetes and rheumatoid arthritis. Also in JIA, there is accumulating evidence that the composition of the microbiome is different from healthy individuals. A growing body of evidence indeed suggests that, among others, the microbiome may influence the development of the immune system, the integrity of the intestinal mucosal barrier, and the differentiation of T cell subsets. In turn, this might lead to dysregulation of the immune system, thereby possibly playing a role in the development of JIA. The potential to manipulate the microbiome, for example by faecal microbial transplantation, might then offer perspectives for future therapeutic interventions. Before we can think of such interventions, we need to first obtain a deeper understanding of the cause and effect relationship between JIA and the microbiome. In this review, we discuss the existing evidence for the involvement of the microbiome in JIA pathogenesis and explore the potential mechanisms through which the microbiome may influence the development of autoimmunity in general and JIA specifically.

  12. [Juvenile idiopathic arthritis and oral health].

    Science.gov (United States)

    Kobus, Agnieszka; Kierklo, Anna; Sielicka, Danuta; Szajda, Sławomir Dariusz

    2016-05-04

    Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health.

  13. Fungal arthritis simulating juvenile rheumatoid arthritis.

    OpenAIRE

    Haapasaari, J; Essen, R V; Kahanpää, A; Kostiala, A A; Holmberg, K; Ahlqvist, J

    1982-01-01

    Petriellidium boydii is often isolated from maduromycosis but has recently been associated with arthritis. A previously healthy 6-year-old boy developed chronic purulent arthritis of the knee after a bicycle accident. Culture of aspirate grew no pathogens and antibiotic treatment had no effect. Culture of synovial fluid grew P boydii, which responded initially to amphotericin but reappeared after six months. Subsequent treatment with miconazole was stopped after development of haematuria. The...

  14. Analysis of the Juvenile Idiopathic Arthritis Immunization Schedule

    Directory of Open Access Journals (Sweden)

    L. S. Namazova-Baranova

    2016-01-01

    Full Text Available Background: The connection between vaccination and autoimmune diseases (and rheumatic pathology in particular is still a subject of discussions. When discussing the possibility of vaccinating rheumatic patients we should take into account the ultra high dangers that infectious diseases pose for such patients, including those that can be prevented by vaccination. We should also take into account the experience of using various vaccine types in rheumatic patients, which illustrates of their high safety profile.Objective: Our aim was to study the immunization schedule in children with juvenile idiopathic arthritis.Methods: The evaluation of vaccine history and other anamnestic data in juvenile idiopathic arthritis patients was based on individual medical records (individual child’s card/preventive vaccination certificate, as well as questionnaires filled by mothers.Results: It has been determined that a significant proportion of children with vaccination schedule deviations are juvenile idiopathic arthritis patients. Almost one in four children with a confirmed rheumatic diagnosis has not been immunized against the major vaccine-preventable diseases. In one non-vaccinated group, there was a case of juvenile arthritis onset after recovering from measles. A small number of patient mothers connects the manifestation of rheumatic diseases with vaccination.Conclusion: Violations of vaccination status in JIA patients require corrections according to the results of clinical studies and the recommendations of international experts.

  15. Imaging in juvenile idiopathic arthritis with a focus on ultrasonography

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Boesen, Mikael;

    2013-01-01

    Early therapeutic intervention and use of new highly efficacious treatments have improved the outcome in many patients with juvenile idiopathic arthritis (JIA), but have also led to the need for more precise methods to evaluate disease activity. In adult rheumatology, numerous studies have establ...

  16. Physical activity in adolescents with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Lelieveld, Otto; Armbrust, Wineke; van Leeuwen, M.A.; Duppen, N.; Geertzen, J.H.; Sauer, P.J.; van Weert, E.

    2008-01-01

    OBJECTIVE: To explore physical activity (PA) in adolescents with juvenile idiopathic arthritis (JIA) compared with a healthy population and to examine associations between PA and disease-related factors. METHODS: Total energy expenditure (TEE), activity-related energy expenditure (AEE), PA level, an

  17. Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

    NARCIS (Netherlands)

    Haasnoot, Anne-Mieke J. W.; Vernie, Lenneke A.; Rothova, Aniki; van der Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.

    2016-01-01

    Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze u

  18. Impact of juvenile idiopathic arthritis associated uveitis in early adulthood

    NARCIS (Netherlands)

    Haasnoot, A.-M.J.W. (Anne-Mieke J. W.); Vernie, L.A. (Lenneke A.); A. Rothova (Aniki); Doe, P.V.D. (Patricia V. D.); L.I. Los (Leonoor I.); N.E. Schalij-Delfos (Nicoline); J.H. de Boer (Joke)

    2016-01-01

    textabstractBackground: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was

  19. Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

    NARCIS (Netherlands)

    Haasnoot, AJW; Vernie, Lenneke A; Rothova, Aniki; V D Doe, Patricia; Los, Leonoor I; Schalij-Delfos, Nicoline E; de Boer, Joke H

    2016-01-01

    BACKGROUND: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

  20. Impact of juvenile idiopathic arthritis associated uveitis in early adulthood

    NARCIS (Netherlands)

    Haasnoot, A.-M.J.W. (Anne-Mieke J. W.); Vernie, L.A. (Lenneke A.); A. Rothová (Aniki); Doe, P.V.D. (Patricia V. D.); L.I. Los (Leonoor I.); N.E. Schalij-Delfos (Nicoline); J.H. de Boer (Joke)

    2016-01-01

    textabstractBackground: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was

  1. Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

    NARCIS (Netherlands)

    Haasnoot, AJW; Vernie, Lenneke A; Rothova, Aniki; V D Doe, Patricia; Los, Leonoor I; Schalij-Delfos, Nicoline E; de Boer, Joke H|info:eu-repo/dai/nl/140201890

    2016-01-01

    BACKGROUND: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

  2. Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

    NARCIS (Netherlands)

    Haasnoot, Anne-Mieke J. W.; Vernie, Lenneke A.; Rothova, Aniki; van der Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.

    2016-01-01

    Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze u

  3. "Immune Complexes in Juvenile Idiopathic Arthritis"

    Directory of Open Access Journals (Sweden)

    Terry Lynn Moore

    2016-05-01

    Full Text Available Abstract for invited review in Molecular Mechanisms of Immune Complex Pathophysiology thematic issue to be published in Frontiers in Immunology. Immune Complexes(ICin Juvenile Idiopathic Arthritis (JIA Terry L. Moore, MD, FAAP, FACR, MACR Professor of Internal Medicine,Pediatrics, and Molecular Biology and Immunology Director of Adult and Pediatric Rheumatology Saint Louis University School of Medicine Saint Louis, Missouri 631`04,USA Juvenile idiopathic arthritis (JIA reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs and associated complement activation by-products in their sera. ICs have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column,C1q solid phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these IC s have shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF, isotypes of anti-cyclic citrullinated (CCP peptide antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We

  4. [Current therapy of polyarticular forms of juvenile idiopathic arthritis].

    Science.gov (United States)

    Hospach, A; Rühlmann, J M; Weller-Heinemann, F

    2016-04-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in infancy and childhood. Approximately 20 % of patients with JIA suffer from the polyarticular form of the disease, which causes a substantial disease burden and long-term sequelae. Therapeutic approaches have used steroids and conventional disease modifying antirheumatic drugs (DMARD) but over the last decade new drugs have become available for the treatment of JIA, in particular biologic DMARD. This article summarizes the current therapy options for polyarticular JIA.

  5. X-linked agammaglobulinemia combined with juvenile idiopathic arthritis and invasive Klebsiella pneumoniae polyarticular septic arthritis.

    Science.gov (United States)

    Zhu, Zaihua; Kang, Yuli; Lin, Zhenlang; Huang, Yanjing; Lv, Huoyang; Li, Yasong

    2015-02-01

    X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the Bruton's tyrosine kinase (BTK) gene. XLA can also present in combination with juvenile idiopathic arthritis (JIA), the major chronic rheumatologic disease in children. We report herein the first known case of a juvenile patient diagnosed with XLA combined with JIA that later developed into invasive Klebsiella pneumoniae polyarticular septic polyarthritis. An additional comprehensive review of XLA combined with JIA and invasive K. pneumoniae septic arthritis is also presented. XLA was identified by the detection of BTK mutations while the diagnosis of JIA was established by clinical and laboratory assessments. Septic arthritis caused by invasive K. pneumoniae was confirmed by culturing of the synovia and gene detection of the isolates. Invasive K. pneumoniae infections can not only result in liver abscesses but also septic arthritis, although this is rare. XLA combined with JIA may contribute to invasive K. pneumoniae infection.

  6. Macrophages - silent enemies in juvenile idiopathic arthritis.

    Science.gov (United States)

    Świdrowska-Jaros, Joanna; Orczyk, Krzysztof; Smolewska, Elżbieta

    2016-07-06

    The inflammatory response by secretion of cytokines and other mediators is postulated as one of the most significant factors in the pathophysiology of juvenile idiopathic arthritis (JIA). The effect of macrophage action depends on the type of their activation. Classically activated macrophages (M1) are responsible for release of molecules crucial for joint inflammation. Alternatively activated macrophages (M2) may recognize self antigens by scavenger receptors and induce the immunological reaction leading to autoimmune diseases such as JIA. Molecules essential for JIA pathophysiology include: TNF-α, the production of which precedes synovial inflammation in rheumatoid arthritis; IL-1 as a key mediator of synovial damage; chemotactic factors for macrophages IL-8 and MCP-1; IL6, the level of which correlates with the radiological joint damage; MIF, promoting the secretion of TNF-α and IL-6; CCL20 and HIF, significant for the hypoxic synovial environment in JIA; GM-CSF, stimulating the production of macrophages; and IL-18, crucial for NK cell functions. Recognition of the role of macrophages creates the potential for a new therapeutic approach.

  7. [Optic neuritis in juvenile idiopathic arthritis patient].

    Science.gov (United States)

    Lourenço, Daniela M R; Buscatti, Izabel M; Lourenço, Benito; Monti, Fernanda C; Paz, José Albino; Silva, Clovis A

    2014-01-01

    Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5,793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required.

  8. Biological agents in polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

    2016-01-01

    BACKGROUND AND OBJECTIVE: Although various biological agents are in use for polyarticular juvenile idiopathic arthritis (pJIA), head-to-head trials comparing the efficacy and safety among them are lacking. We aimed to compare the efficacy and safety of biological agents in pJIA using all currently...

  9. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    OpenAIRE

    Nelson, Andrew D.; Fischer, Philip R.; Reed, Ann M.; Wylam, Mark E.

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis.

  10. JUVENILE RHEUMATOID ARTHRITIS (TERMINOLOGICALAND CLASSIFICATION ASPECTS

    Directory of Open Access Journals (Sweden)

    N N Kuzmina

    2000-01-01

    Full Text Available Basing on the data of home and foreign literature and on the long-term experience of pediatric rheumatologists, terminologic and classification aspects of Juvenile rheumatoid arthritis (JRA are presented. Approaches to developing of diagnostic and classification of JRA criteria in future are described.

  11. Orofacial pain, jaw function, and temporomandibular disorders in adult women with a history of juvenile chronic arthritis or persistent juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Bakke, M.; Zak, M.; Jensen, B.L.;

    2001-01-01

    Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis......Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis...

  12. Immune Complexes in Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Moore, Terry L

    2016-01-01

    Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients' sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA.

  13. Overview of the radiology of juvenile idiopathic arthritis (JIA)

    Energy Technology Data Exchange (ETDEWEB)

    Cohen, P.A.; Job-Deslandre, C.H.; Lalande, G.; Adamsbaum, C

    2000-02-01

    Plain films remain the basic tool for diagnosis and follow-up evaluation of juvenile idiopathic arthritis (JIA). In this paper, we review the new classification of JIA: systemic arthritis, oligoarthritis (persistent), oligoarthritis (extended), polyarticular arthritis (rheumatoid factor negative), polyarticular arthritis (rheumatoid factor positive), enthesitis related arthritis, psoriatic arthritis and unclassified arthritis. We will also review regional abnormalities of three stages: an early stage, an intermediate stage, a late stage, as well as the differential diagnosis.

  14. Psychosocial functioning in children and young adults with juvenile arthritis.

    Science.gov (United States)

    Ungerer, J A; Horgan, B; Chaitow, J; Champion, G D

    1988-02-01

    A questionnaire survey of 363 children and young adults with juvenile arthritis was conducted to assess the relations among disease severity, psychosocial functioning, and adjustment in three age groups--primary school, high school, and young adult. Parents were surveyed separately to determine which characteristics of the ill child at different ages most significantly impact the well-being of the family. Indices of psychologic functioning and disease severity were associated with adjustment in the primary school and high school groups, whereas measures of social relationships were strongly associated with adjustment only in the high school group. Relations among measures of psychologic functioning, social relationships, disease severity, and adjustment in young adults were minimal. Level of disease severity was associated with the presence of financial concerns, emotional problems, and physical strain in parents of high school children and young adults. The results emphasize the importance of using a developmental model for understanding the adjustment of individuals with chronic juvenile arthritis and their families.

  15. The impact of disease and antirheumatic therapy factors on growth retardation in children suffering from juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    T.M. Bzarova

    2006-01-01

    Full Text Available THE STUDY EVALUATED THE IMPACT OF REFRACTORY JUVENILE IDIOPATHIC ARTHRITIS (JIA AND INTERFERENCE OF ANTIRHEUMATIC THERAPY ON PHYSICAL STATURE AND GROWTH AND PHYSICAL DEVELOPMENT IN CHILDREN. WE OBSERVED 133 PATIENTS WITH JIA WITH AGE FROM 4.6 TO 18 YEARS. WE ASSESSED PATIENTS' PHYSICAL STATURE (DOCUMENTING HEIGHT AND WEIGHT AND FURTHER GROWTH AND DEVELOPMENT STARING FROM BIRTH, BEFORE THE ONSET OF JIA, AT THE FIRST YEAR FROM THE ONSET OF THE DISB EASE AND LATER ON. PHYSICAL RETARDATION WAS MARKED IN ALL CHILDREN IN THE FIRST YEAR FROM THE ONSET OF JIA. SYSTEMIC JIA, WHICH IS KNOWN FOR HIGH ACTIVITY AND POLYARTICULAR OR GENERALIZED JOINT LESION, WAS LINKED TO SIGNIFICANT PHYSICAL RETARDATION IN ALL CHILDREN WITH SYSBTEMIC JIA. LONGBTERM GLUCOCORTICOID ADMINISTRATION, EVEN AT LOW DOSES, ACCENTUATED THE NEGATIVE IMPACT OF JIA ON GROWTH RESULTING IN MARKED GROWTH DELAY OR ITS' TOTAL ARREST. WE HAVE ELABORATED RISK FACTORS FOR SHORT STATURE IN CHILDREN WITH JIA, WHICH SUGGEST THERAPY GUIDELINES, PRESENTED IN THIS ARTICLE.KEY WORDS: JUVENILE IDIOPATHIC ARTHRITIS, СHILDREN.

  16. Physiotherapy in pauciarticular juvenile idiopathic arthritis. Case study.

    Science.gov (United States)

    Zuk, Beata; Kaczor, Zofia; Zuk-Drążyk, Berenika; Księżopolska-Orłowska, Krystyna

    2014-01-01

    Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood and adolescence. This term encompasses a group of chronic systemic inflammatory diseases of the connective tissue which cause arthritis in patients under 16 years of age lasting at least 6 weeks. The authors presented the characteristic features of physiotherapy based on functional examination results on the basis of two cases of girls with pauciarticular JIA treated according to an established pharmacological regimen. Physiotherapy should be introduced at an early stage of the disease. Kinesiotherapy preceded by history-taking and a functional examination of the patient, has to focus on both primary and secondary joint lesions.

  17. Concurrence of Juvenile Idiopathic Arthritis and Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    Ben Abdelghani Kaouther

    2011-01-01

    Full Text Available We report a 21-year-old female patient known to have Juvenile idiopathic arthritis (JIA who later developed multiple sclerosis (MS. The disease was documented on the brain and cerebral magnetic resonance imaging (MRI and the visual evoked potential. Our case emphasizes the need to evaluate the symptoms and brain MRI carefully. The concurrence of MS and JIA is uncommon. The possible relationship between the 2 diseases was discussed.

  18. Juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Clarke, Sarah L N; Sen, Ethan S; Ramanan, Athimalaipet V

    2016-04-27

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.

  19. Nephrotic syndrome due to immunoglobulin M mesangial glomerulonephritis preceding juvenile idiopathic arthritis.

    Science.gov (United States)

    Voyer, Luis E; Alvarado, Caupolican; Cuttica, Rubén J; Balestracci, Alejandro; Zardini, Marta; Lago, Néstor

    2013-05-21

    The association between nephrotic syndrome and juvenile idiopathic arthritis have rarely been described in pediatric patients. We report a child with steroid-responsive nephrotic syndrome, with frequent relapses, who presented with a new relapse of nephrotic syndrome associated with arthritis and uveitis at 21 months in remission after treatment with chlorambucil. Juvenile idiopathic arthritis was diagnosed and kidney biopsy examination showed mesangial glomerulonephritis with immunoglobulin M deposits. To our knowledge, only 2 cases of nephrotic syndrome preceding juvenile idiopathic arthritis have been reported, one without histopathology assessment and the other with minimal change disease. Although mesangial glomerulonephritis with nephrotic syndrome and juvenile idiopathic arthritis could have been coincidental, the immune pathogenic mechanism accepted for both diseases suggests they could be related.

  20. Juvenile rheumatoid arthritis and lymphoedema: lymphangiographic aspects

    Energy Technology Data Exchange (ETDEWEB)

    Schmit, P.; Brunelle, F. [Service de Radiopediatrie, Groupe Hospitalier Necker-Enfants-Malades, Paris (France); Prieur, A.M. [Unite Fonctionnelle de Rhumatologie Infantile, Groupe Hospitalier Necker-Enfants-Malades, Paris (France)

    1999-05-01

    We report a 5{sup 1}/{sub 2}-year-old boy with juvenile rheumatoid arthritis (JRA) and lower-limb lymphoedema. US, MRI and lymphangiography were performed. Based on the lymphangiographic study, we propose a pathogenesis based on obstruction of normal superficial lymphatic vessels in the affected limb. This is discussed with other pathogenetic factors proposed in the 16 previously reported cases of lymphoedema complicating JRA. (orig.) With 3 figs., 5 refs.

  1. Juvenile idiopathic arthritis in the new world of biologics.

    Science.gov (United States)

    Ostring, Genevieve Tyra; Singh-Grewal, Davinder

    2013-09-01

    Juvenile idiopathic arthritis results in significant pain and disability in both children and adults. Advances in treatment resulting in improved long-term outcomes have occurred; however, an emphasis on early and aggressive diagnosis and management hopes to improve outcomes further. Juvenile idiopathic arthritis remains a clinical diagnosis of exclusion, but further research may delineate biological markers associated with the disease and its subtypes. Therapy for patients includes intra-articular steroid injections, disease modifying agents such as methotrexate and biological agents. Biological agents have provided exciting new therapeutic options in the last decade; however, long-term side effects of modulating the immune system are not yet fully understood. Systemic steroids may also be required but their long-term use is avoided. Uveitis needs to be screened for in all of those with the diagnosis. Multidisciplinary team care is required in managing these young people.

  2. Juvenile arthritis: current concepts in terminology, etiopathogenesis, diagnosis, and management.

    Science.gov (United States)

    Abramowicz, S; Kim, S; Prahalad, S; Chouinard, A F; Kaban, L B

    2016-07-01

    The latest change in terminology from juvenile rheumatoid arthritis (JRA) to juvenile idiopathic arthritis (JIA), established by the International League of Associations for Rheumatology (ILAR), has resulted in some confusion for OMFS and other treating clinicians. JIA comprises a group of systemic inflammatory diseases that result in the destruction of hard and soft tissues in a single or multiple joints. In a significant number of patients, one or both temporomandibular joints (TMJ) are also involved. TMJ disease may be accompanied by pain, swelling, and limitation of motion, as well as mandibular retrognathism, open bite, and asymmetry. The purpose of this article is to provide a review, for the oral and maxillofacial surgeon, of the terminology, etiopathogenesis, diagnosis, and management of children with JIA.

  3. JUVENILE IDIOPATHIC ARTHRITIS – A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Paresh H

    2012-11-01

    Full Text Available ABSTRACT: The prevalence of Juvenile idiopathic arthritis (JIA is 0.86 per 1000 children. Subcutaneous nodules have been reported in 5% to 10% of children with JIA. Approximately 90% of patients with RA and subc utaneous nodules test positive for rheumatoid factor (RF, and approximately 40% o f all RF-seropositive patients with RA have subcutaneous nodules, whereas only 6% in volvement is seen in seronegative cases. We hereby report a case of atypical Juvenile idiopathic arthritis (JIA in a 6 year old, female child with joint pain & myalgia along with subcutaneous nodules over the dorsum of feet, hands and elbows. Joint pain initial ly involving the left ankle, slowly progressed to involve the knee, shoulder, wrist, metacar pophalangeal and interphalangeal joints over a period of one year. Joint involvement was not symmetric. RF was Negative. Fundoscopy examination was normal. Histopathological examinat ion revealed a central zone of Fibrinoid necrosis surrounded by epithelioid h istiocytes and occasional lymphocytes. Differential diagnosis of Rheumatoid Nodule (R N or Subcutaneous Granuloma Annulare (SGA or Necrobiosis Lipoidica Diabeticorum was made. In light of clinicopathological findings, both SGA and NLD were ruled out a nd the diagnosis of Juvenile idiopathic arthritis presenting as RF-negative polyarthritis was made.

  4. Anterior uveitis in juvenile rheumatoid arthritis.

    Science.gov (United States)

    Kanski, J J

    1977-10-01

    The ocular and systemic characteristics of 160 patients with anterior uveitis and seronegative juvenile rheumatoid arthritis are reviewed. Chronic uveitis occurred in 131 patients, 76% of whom were girls. Both eyes were involved in 70% of the cases. Band keratopathy occurred in 41% of the eyes, cataract in 42%, and secondary glaucoma in 19%. Only 11 patients had uveitis before the onset of arthritis. Notable correlations included a pauciarticular onset of arthritis in 95% of the patients, and positive tests for antinuclear antibody in 82%. Of 29 patients with acute anterior uveitis, 27 were boys. The inflammation responded well to therapy, and serious complications did not occur. At follow-up 21 patients had typical ankylosing spondylitis, and five had sacroiliitis. The incidence of positive results of tests for HLA-B27 antigen was 94%.

  5. Screening for uveitis in juvenile chronic arthritis.

    Science.gov (United States)

    Kanski, J J

    1989-03-01

    Three hundred and fifteen patients with anterior uveitis associated with juvenile chronic arthritis (JCA) were studied in order to identify the various risk factors for uveitis. Girls were more susceptible to uveitis than boys by a ratio of 3:1. In 94% of cases the uveitis was diagnosed after the development of arthritis. The risk of uveitis was small after seven or more years had elapsed from the onset of arthritis. Patients with pauciarticular onset JCA had the highest risk of uveitis and systemic onset patients the least risk. The presence of circulating antinuclear antibody was also an important marker for an increased risk of uveitis. A regimen for routine screening of patients is suggested.

  6. Doença de Graves associada à artrite idiopática juvenil Graves' disease associated with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Vanessa de Matos Santos Mendonça Marques

    2011-04-01

    Full Text Available Os autores relatam o caso de uma menina de 10 anos de idade com diagnóstico de doença de Graves (DG, em tratamento com propiltiouracil, que desenvolveu uveíte e artrite poliarticular e cuja mãe também tem DG e lúpus discoide. São discutidos os diagnósticos diferenciais de artrite inflamatória que surge em uma criança com doença tireoidiana autoimune medicada com drogas antitireóideas.The authors report the case of a 10-year-old girl with Graves' disease (GD, treated with propylthiouracil, who developed uveitis and polyarticular arthritis, and whose mother also had GD and discoid lupus. The differential diagnosis of inflammatory arthritis that appears in a child with autoimmune thyroid disease managed with antithyroid drugs is discussed.

  7. Subpopulations Within Juvenile Psoriatic Arthritis: A Review of the Literature

    Directory of Open Access Journals (Sweden)

    Matthew L. Stoll

    2006-01-01

    Full Text Available The presentation of juvenile psoriatic arthritis (JPsA has long been recognized to be clinically heterogeneous. As the definition of JPsA expanded to accommodate atypical manifestations of psoriasis in young children, studies began to reflect an increasingly clear biphasic distribution of age of onset, with peaks in the first few years of life and again in early adolescence. These two subpopulations differ in gender ratio, pattern of joint involvement, laboratory findings and potentially response to therapy. Intriguingly, a similar distribution of age of onset has been observed in juvenile rheumatoid arthritis (JRA, and correlates with patterns of HLA association. While a secure classification of subpopulations within JPsA awaits improved pathophysiologic understanding, future research must consider the possibility that different disease mechanisms may be operative in distinct subsets of patients with this disorder.

  8. JUVENILE CHRONIC ARTHRITIS WITH EYE LESION

    Directory of Open Access Journals (Sweden)

    S O Salugina

    2002-01-01

    Full Text Available A bstract. Objective, to describe a series of pts with JRA/JCA and uveitis. Material and methods. The study included 81 pts with JRA and uveitis. There were 68 girls-84%, 13 boys-16%. We studied the clinical manifestations, the antinuclear antibodies (ANA using HEP-2 cells for the 33 pts with uveitis and 46 pts without uveitis, HLA status was determined for 36 pts. Results. 85,2% of the children had arthritis before uveitis. The mean age at onset of arthritis was 3,5 year (range: 1-10 yrs, the mean age at onset of uveitis was 6 year (range: 2-15 yrs. The mean interval between the onset of arthritis and uveitis was 3,02 years (range: 3,5 yrs before arthritis onset to 12,5 yrs after. In 68,1% pts the diagnosis of uveitis was made within 5 yrs after onset of arthritis. 93% of pts had mono-oligoarticular onset, but 50% had poliarticular course. 23,5% of pts had functional disability 3-4 classes. Ocular complications were developed in 53.1%: cataracts-38,3%, band keratopathy-11,1%, glaucoma-2,5%. 93,9% of 33 studied children with arthritis and uveitis were ANA positive, 9,1% were RF positive. 18,1 % had HLA-DR8 (p<0,001, 83,3% - HLA-A2 (p<0,00l, HLA-B27 - 30,6 % (p<0,00l. Conclusion. Clinical and laboratory data of our pts suggest that: lthe combination of arthritis and uveitis would be named JCA with uveitis; 2 according our opinion JCA with uveitis is separate nosological form among the juvenile arthritides.

  9. [Juvenil idiopathic arthritis. Part 1: diagnosis, pathogenesis and clinical manifestations].

    Science.gov (United States)

    Espada, Graciela

    2009-10-01

    Juvenile idiopathic arthritis is not a single disease and constitutes an heterogeneous group of illnesses or inflammatory disorders. This new nomenclature encompasses different disease categories, each of which has different presentation, clinical signs, symptoms, and outcome. The cause of the disease is still unknown but both environmental and genetic factors seem to be related to its pathogenesis. Is the most common chronic rheumatic disease in children and an important cause of short-term and long-term disability. In this article, clinical manifestation, new classification and approach to diagnosis are reviewed.

  10. Trends in paediatric rheumatology referral times and disease activity indices over a ten-year period among children and young people with Juvenile Idiopathic Arthritis: results from the childhood arthritis prospective Study.

    Science.gov (United States)

    McErlane, Flora; Foster, Helen E; Carrasco, Roberto; Baildam, Eileen M; Chieng, S E Alice; Davidson, Joyce E; Ioannou, Yiannis; Wedderburn, Lucy R; Thomson, Wendy; Hyrich, Kimme L

    2016-07-01

    The medical management of JIA has advanced significantly over the past 10 years. It is not known whether these changes have impacted on outcomes. The aim of this analysis was to identify and describe trends in referral times, treatment times and 1-year outcomes over a 10-year period among children with JIA enrolled in the Childhood Arthritis Prospective Study. The Childhood Arthritis Prospective Study is a prospective inception cohort of children with new-onset inflammatory arthritis. Analysis included all children recruited in 2001-11 with at least 1 year of follow-up, divided into four groups by year of diagnosis. Median referral time, baseline disease pattern (oligoarticular, polyarticular or systemic onset) and time to first definitive treatment were compared between groups. Where possible, clinical juvenile arthritis disease activity score (cJADAS) cut-offs were applied at 1 year. One thousand and sixty-six children were included in the analysis. The median time from symptom onset and referral to first paediatric rheumatology appointment (22.7-24.7 and 3.4-4.7 weeks, respectively) did not vary significantly (∼20% seen within 10 weeks of onset and ∼50% within 4 weeks of referral). For oligoarticular and polyarticular disease, 33.8-47 and 25.4-34.9%, respectively, achieved inactive disease by 1 year, with ∼30% in high disease activity at 1 year. A positive trend towards earlier definitive treatment reached significance in oligoarticular and polyarticular pattern disease. Children with new-onset JIA have a persistent delay in access to paediatric rheumatology care, with one-third in high disease activity at 1 year and no significant improvement over the past 10 years. Contributing factors may include service pressures and poor awareness. Further research is necessary to gain a better understanding and improve important clinical outcomes. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology.

  11. Advances in the treatment of polyarticular juvenile idiopathic arthritis

    Science.gov (United States)

    Webb, Kate; Wedderburn, Lucy R.

    2015-01-01

    Purpose of review To review recent advances in the management strategies of polyarticular course juvenile idiopathic arthritis (JIA) and identify unanswered questions and avenues for further research. Recent findings There is evidence for an early, aggressive, treat-to-target approach for polyarticular JIA. Clinical disease activity criteria have been recently defined and validated, including criteria for inactive disease and the juvenile arthritis disease activity score (JADAS). There is a need for evidence-based, defined disease targets and biomarkers for prediction of response, including targets for remission induction, and guidelines on drug withdrawal. Recent treatment consensus plans and guidelines are discussed and compared, including the 2015 NHS England clinical policy statement, the 2014 Childhood Arthritis and Rheumatology Research Alliance (CARRA) treatment plans and the 2011 American College of Rheumatology (ACR) guidelines. Evidence for new agents such as tocilizumab, rituximab, golimumab, ustekinumab, certolizumab and tofacitinib is promising: the recent clinical trials are summarized here. Stratification of individual patient treatment remains a goal, and predictive biomarkers have been shown to predict success in the withdrawal of methotrexate therapy. Summary There are promising advances in the treatment approaches, disease activity criteria, clinical guidelines, pharmaceutical choices and individually stratified therapy choices for polyarticular JIA. PMID:26147756

  12. CLINICAL CASE OF TOCILIZUMAB THERAPY IN A PATIENT WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E. I. Alexeeva

    2013-01-01

    Full Text Available The article presents a case of successful application of a monoclonal antibodies drug to interleukin 6 receptors (tocilizumab at severe systemic juvenile idiopathic arthritis with the development of secondary hemophagocytic syndrome. Tocilizumab treatment secured a decrease in clinical and laboratory parameters of the disease activity, life quality improvement, systemic juvenile idiopathic arthritis and hemophagocytic syndrome remission and allowed avoiding the per os prescription of glucocorticoids.

  13. Gold nephropathy in juvenile rheumatoid arthritis.

    Science.gov (United States)

    Husserl, F E; Shuler, S E

    1979-01-01

    A 2-year-old girl was treated with gold salts for juvenile rheumatoid arthritis. Treatment had to be discontinued when persistent proteinuria was detected. As this case report indicates, close monitoring of the urine is mandatory during treatment with gold salts to detect early signs of toxicity: hematuria followed by casts and then proteinuria as therapy is continued. Histologic examination with electron microscopy will help to differentiate the different forms of gold toxicity. When the findings are consistent with gold-induced renal involvement, therapy should be discontinued. The gold nephropathy usually resolves in time, with no permanent renal damage.

  14. Juvenile idiopathic arthritis: a clinical overview

    Energy Technology Data Exchange (ETDEWEB)

    Davidson, J

    2000-02-01

    The chronic arthritides in childhood remain a poorly understood group of conditions. Their classification has been a source of much confusion over the years with differences in terminology between Europe and North America. A significant step forward in paediatric rheumatology has been the recent development of an internationally agreed classification system which uses the overall term juvenile idiopathic arthritis (JIA). The various subtypes of JIA and their clinical features are described, together with an overview of their differential diagnosis, complications and outcomes. An outline of current management strategies is given and potential future developments highlighted.

  15. CYCLOSPORIN A IN THERAPY FOR JUVENILE ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E S Fedorov

    2010-01-01

    Full Text Available The paper describes approaches to using cyclosporin A (CsA in juvenile arthritis (JA. It shows the benefits of combination basic therapy with CsA and methotrexate included into a treatment regimen mainly for systemic JA and JA involving the eye (uveitis versus monotherapy with the above drugs. Attention is drawn to that the oral dose of glucocorticoids may be decreased when CsA is incorporated into the treatment regimen. CsA is shown to be of value as the drug of choice for the therapy of such a menacing complication of systemic JA as the macrophage activation syndrome

  16. EFFICACY OF ETANERCEPT IN TREATMENT OF VARIOUS TYPES OF JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    O. Yu. Konopel'ko

    2013-01-01

    Full Text Available Aim: to assess efficacy and safety of etanercept in treatment of various types of juvenile idiopathic arthritis in children under conditions of real clinical practice. Patients and methods: 52 children were included into the study, among them 16 were with systemic and 36 with juvenile idiopathic arthritis without extra-articular involvement. Results: etanercept treatment was the most efficient in patients with systemic juvenile idiopathic arthritis without extra-articular involvement. In 6 and 12 months of the treatment 50 and 70% improvement according to the ACRpedi criteria were established in 31/36 (86% and 28/36 (78% of the patients, respectively. In 24 months in 5 (29% of 17 children remained in the study remission stage of the diseases was confirmed. Conclusions: etanercept treatment was not associated with significant unfavorable effects, which allows to recommend this drug for treatment of juvenile idiopathic arthritis without extra-articular involvent and resistant to standard anti-rheumatic therapy.

  17. Etanercept therapy in children with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Hung, Jeng-Juh; Huang, Jing-Long

    2005-12-01

    Etanercept is an effective inhibitor of tumor necrosis factor that has shown a beneficial effect in patients with juvenile rheumatoid arthritis (JRA) that did not respond to other disease-modifying drugs. Here we report 3 patients with JRA who were refractory to traditional therapy; 1 with systemic JRA and 2 with polyarticular JRA. They received etanercept 0.4 mg/kg (maximum 25 mg) subcutaneously, twice a week for 3 months. The symptoms of arthritis improved significantly except that the patient with systemic JRA had disease flare-up during etanercept therapy. Two patients had upper respiratory tract infection during etanercept therapy and 1 suffered from seizure attack. The 2 patients with polyarticular JRA had disease flare-up within 2 months after etanercept was discontinued. This is the first report of etanercept treatment in JRA patients in Taiwan.

  18. Update on Genetic Susceptibility and Pathogenesis in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Morten Herlin

    2014-07-01

    Full Text Available Juvenile idiopathic arthritis (JIA is a multifactorial disease with a pathogenesis which remains inexplicable. However, genome-wide association studies brought forward within recent years have discovered several new susceptibility genes, and accumulating evidence supports genetic variability as playing a key role in JIA development. This review summarises the present knowledge of human leukocyte antigen (HLA and non-HLA polymorphisms conferring disease susceptibility, and discusses the areas in JIA genetics, which are still to be investigated in order to apply JIA genetics in a clinical setting.

  19. Assessment and Management of Pain in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Jennifer N Stinson

    2012-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA is a common chronic childhood illness. Pain is the most common and distressing symptom of JIA. Pain has been found to negatively impact all aspects of functioning, including physical, social, emotional and role functions. Children with arthritis continue to experience clinically significant pain despite adequate doses of disease-modifying antirheumatic drugs and anti-inflammatory agents. The present article reviews the prevalence and nature of pain in JIA, the biopsychosocial factors that contribute to the pain experience, current approaches to assessing pain in this population, and ways of managing both acute and persistent pain using pharmacological, physical and psychological therapies. Finally, new approaches to delivering disease self-management treatment for youth with JIA using the Internet will be outlined.

  20. Intraveous gammaglobulin for the treatment of juvenil idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Lòpez Ortíz Daniela Jazmin

    2014-07-01

    Full Text Available Recently there has been a growing interest in autoimmune and auto-inflammatory diseases, both entities involving a therapeutic challenge even though more sophisticated therapeutic options have been developed. According to this, juvenile idiopathic arthritis (JIA is an example of this challenge, with proven autoimmune mechanisms as in positive rheumatoid factor arthritis; and autoinflammatory mechanisms in systemic onset juvenile idiopathic arthritis. For both damage mechanisms, intravenous immunoglobulin (IVIG has been used as a successful immunomodulator. The treatment with IVIG for JIA and associated features as macrophage activation syndrome (MAS has shown to be beneficial. Nevertheless more studies are required to support its usefulness, as well as clinical trials to document the IVIG effectiveness in comparison with the rest of therapeutic agents used. Despite its cost, the IVIG is well tolerated and should be considered useful in combination with other drugs as part of the JIA treatment, especially in those patients with associated threatening-life systemic complications or with high risk of infection. The present review pretends to expose, according to previous references from various authors, that IVIG is an alternative therapeutic option in these cases.

  1. Methotrexate in juvenile idiopathic arthritis: towards tailor-made treatment.

    Science.gov (United States)

    Ćalasan, Maja Bulatović; Wulffraat, Nico M

    2014-07-01

    Methotrexate (MTX) is the key treatment in juvenile idiopathic arthritis (JIA). Nevertheless, MTX is not always sufficiently efficacious and can lead to adverse effects, which compromises complete disease control. In such cases, combination therapies with biologicals are given, even at MTX start, before knowing the patients' MTX response. Ideally, clinicians should be able to practice precision medicine by knowing before or early after MTX start, which patients will benefit from MTX only and which patients will not, thus requiring addition of biologicals. To make such tailor-made treatment decisions, clinicians require tools to optimize MTX treatment. In this review, we focus on tools for tailor-made MTX treatment in JIA.

  2. Complicated uveitis in late onset juvenile idiopathic psoriatic arthritis.

    Science.gov (United States)

    Bravo Ljubetic, L; Peralta Calvo, J; Larrañaga Fragoso, P

    2016-04-01

    A 6 year-old girl with juvenile psoriatic arthritis (JPsA) and bilateral complicated anterior uveitis developed several ocular complications that required 5 surgical procedures. Despite the aggressive course of ocular inflammation, her visual acuity remained good. Arthritis (main criterion for the diagnosis of JPsA) appeared years after ocular involvement. She showed a good anti-tumour necrosis factor initial response. The definitive diagnosis of JPsA was established years after the onset of symptoms. In addition, the patient maintained a good visual acuity, despite its complicated disease course. Finally, she showed a good clinical response to adalimumab. Copyright © 2015 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  3. Health Related Quality of Life before, during and after pregnancy in Norwegian women with Rheumatoid Arthritis and Juvenile Idiopathic Arthritis

    OpenAIRE

    Jakobsen, Bente

    2013-01-01

    Background: There is a known interaction between pregnancy and rheumatic disease. Women with rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) are concerned about the potential impact of a pregnancy. Therefore, it is important to get more knowledge on how pregnancy affects these womens health related quality of life (HRQL).Purpose: To study changes in HRQL in Norwegian women with RA and JIA before, during and after pregnancy.Methods: A total 35 patients with RA and 27 patients...

  4. Clinical Orofacial Examination in Juvenile Idiopathic Arthritis

    DEFF Research Database (Denmark)

    Stoustrup, Peter; Twilt, Marinka; Spiegel, Lynn

    2017-01-01

    review. The level of evidence for the 5 recommendations was derived primarily from descriptive studies, such as cross-sectional and case-control studies. CONCLUSION: Five recommendations are proposed for the orofacial examination of patients with JIA to improve the clinical practice and aid standardized......OBJECTIVE: To develop international consensus-based recommendations for the orofacial examination of patients with juvenile idiopathic arthritis (JIA), for use in clinical practice and research. METHODS: Using a sequential phased approach, a multidisciplinary task force developed and evaluated...... a set of recommendations for the orofacial examination of patients with JIA. Phase 1: A Delphi survey was conducted among 40 expert physicians and dentists with the aim of identifying and ranking the importance of items for inclusion. Phase 2: The task force developed consensus about the domains...

  5. Distinct synovial immunopathologic characteristics of juvenile-onset spondylarthritis and other forms of juvenile idiopathic arthritis

    NARCIS (Netherlands)

    E. Kruithof; V. van den Bossche; L. de Rycke; B. Vandooren; R. Joos; J.D. Canete; P.P. Tak; A.M.H. Boots; E.M. Veys; D. Baeten

    2006-01-01

    Objective. To characterize the synovial immunopathologic features of juvenile-onset spondylarthritis (SpA) in relation to adult SpA and other forms of juvenile idiopathic arthritis (JIA). Methods. Synovial biopsy samples were obtained from 10 patients with juvenile-onset SpA, 23 with adult SpA, 19 w

  6. EXPERIENCE OF RITUXIMAB APPLICATION ON A PATIENT, SUFFERING FROM JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E.I. Alexeeva

    2006-01-01

    Full Text Available The article describes the run of the severe systemic juvenile rheumatoid arthritis, which is resistant to the standard antirheumatic therapy. The disease was characterized by such systemic implications of the disease, as: fever, rash, pericarditis, lymphadenopathy, hepatomegaly accompanied by the generalized joint syndrome and high laboratory indices of activity. Introduction of rituximab into the treatment scheme allowed the researchers to decrease the general activity of the disease, arrest the systemic implications, improve functional status of the joints, and normalize the laboratory indices of activity. The effect duration was 5 months and 4 months after the first and second course of treatment by rituximab accordingly. The treatment results prove the perspective of rituximab application with in the complex therapy for the patients, suffering from juvenile rheumatoid arthritis. However, it is necessary to conduct further research to identify the location of antibodies to cd 20+ within the therapy scheme of this disease. Key words: children, treatment, rituximab, juvenile rheumatoid arthritis.

  7. Imaging of juvenile idiopathic arthritis. Part II: Ultrasonography and MRI

    Directory of Open Access Journals (Sweden)

    Iwona Sudoł-Szopińska

    2016-09-01

    Full Text Available Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals in the developmental age. Radiography, which was described in the first part of this publication, is the standard modality in the assessment of this condition. Ultrasound and magnetic resonance imaging enable early detection of the disease which affects soft tissues, as well as bones. Ultrasound assessment involves: joint cavities, tendon sheaths and bursae for the presence of synovitis, intraand extraarticular fat tissue to visualize signs of inflammation, hyaline cartilage, cartilaginous epiphysis and subchondral bone to detect cysts and erosions, and ligaments, tendons and their entheses for signs of enthesopathies and tendinopathies. Magnetic resonance imaging is indicated in children with juvenile idiopathic arthritis for assessment of inflammation in peripheral joints, tendon sheaths and bursae, bone marrow involvement and identification of inflammatory lesions in whole-body MRI, particularly when the clinical picture is unclear. Also, MRI of the spine and spinal cord is used in order to diagnose synovial joint inflammation, bone marrow edema and spondylodiscitis as well as to assess their activity, location, and complications (spinal canal stenosis, subluxation, e.g. in the atlantoaxial region. This article discusses typical pathological changes seen on ultrasound and magnetic resonance imaging. The role of these two methods for disease monitoring, its identification in the pre-clinical stage and establishing its remission are also highlighted.

  8. Imaging of juvenile idiopathic arthritis. Part II: Ultrasonography and MRI

    Science.gov (United States)

    Grochowska, Elżbieta; Gietka, Piotr; Płaza, Mateusz; Pracoń, Grzegorz; Saied, Fadhil; Walentowska-Janowicz, Marta

    2016-01-01

    Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals in the developmental age. Radiography, which was described in the first part of this publication, is the standard modality in the assessment of this condition. Ultrasound and magnetic resonance imaging enable early detection of the disease which affects soft tissues, as well as bones. Ultrasound assessment involves: joint cavities, tendon sheaths and bursae for the presence of synovitis, intraand extraarticular fat tissue to visualize signs of inflammation, hyaline cartilage, cartilaginous epiphysis and subchondral bone to detect cysts and erosions, and ligaments, tendons and their entheses for signs of enthesopathies and tendinopathies. Magnetic resonance imaging is indicated in children with juvenile idiopathic arthritis for assessment of inflammation in peripheral joints, tendon sheaths and bursae, bone marrow involvement and identification of inflammatory lesions in whole-body MRI, particularly when the clinical picture is unclear. Also, MRI of the spine and spinal cord is used in order to diagnose synovial joint inflammation, bone marrow edema and spondylodiscitis as well as to assess their activity, location, and complications (spinal canal stenosis, subluxation, e.g. in the atlantoaxial region). This article discusses typical pathological changes seen on ultrasound and magnetic resonance imaging. The role of these two methods for disease monitoring, its identification in the pre-clinical stage and establishing its remission are also highlighted. PMID:27679727

  9. Microcirculation of the juvenile knee in chronic arthritis

    DEFF Research Database (Denmark)

    Bünger, Cody; Bülow, J; Tøndevold, E

    1986-01-01

    In order to investigate pathogenetic factors in growth abnormalities of the knee in hemophilic arthropathy and juvenile rheumatoid arthritis, the hemodynamic changes of the knee following chronic synovial inflammation and elevated joint pressure were studied in puppies. Unilateral arthritis was i....... The growth plates formed borders for the extension of these changes. The increased permeability and surface area between blood and bone in arthritis may accelerate the resorption and subsequent destruction of subchondral bone in chronic arthropathies of the juvenile knee.......In order to investigate pathogenetic factors in growth abnormalities of the knee in hemophilic arthropathy and juvenile rheumatoid arthritis, the hemodynamic changes of the knee following chronic synovial inflammation and elevated joint pressure were studied in puppies. Unilateral arthritis...

  10. IL-1 inhibition in systemic juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Gabriella Giancane

    2016-12-01

    Full Text Available Systemic juvenile idiopathic arthritis (sJIA is the form of childhood arthritis whose treatment is most challenging. The demonstration of the prominent involvement of interleukin (IL-1 in disease pathogenesis has provided the rationale for the treatment with biologic medications that antagonize this cytokine. The three IL-1 blockers that have been tested so far (anakinra, canakinumab and rilonacept have all been proven effective and safe, although only canakinumab is currently approved for use in sJIA. The studies on IL-1 inhibition in sJIA published in the past few years suggest that children with fewer affected joints, higher neutrophil count, younger age at disease onset, shorter disease duration, or, possibly, higher ferritin level may respond better to anti-IL-1 treatment. In addition, it has been postulated that use of IL-1 blockade as first-line therapy may take advantage of a window of opportunity, in which disease pathophysiology can be altered to prevent the occurrence of chronic arthritis. In this review, we analyze the published literature on IL-1 inhibitors in sJIA and discuss the rationale underlying the use of these medications, the results of therapeutic studies, and the controversial issues.

  11. Consenso em reumatologia pediátrica: parte I - definição dos critérios de doença inativa e remissão em artrite idiopática juvenil/artrite reumatóide juvenil Consensus in pediatric rheumatology: part I - criteria definition of inactive disease and remission in juvenile idiopathic arthritis / juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Claudia Machado

    2005-02-01

    provided the basis for the development of a consensus conference using the nominal group technique (NGT to reach consensus on questions not solvable by the questionnaire format. One hundred and thirty PR from 34 countries responded the Delphi questionnaires and 20 PR from 9 countries attended a 2-day consensus conference. RESULTS: Consensus results were: criteria for inactive disease should include: 1 no active arthritis; 2 no fever, rash, serositis, splenomegaly, or generalised lymphadenopathy attributable to JIA/JRA; 3 no active uveitis; 4 normal ESR or CRP (if both are tested, both must be normal; 5 a physician's global assessment of disease activity rated at the best score possible and indicating no disease activity. CONCLUSIONS: According to consensus vote, 6 continuous months of inactive disease are necessary before classifying a patient as in remission on medication; 12 months off medication while maintaining inactive disease are necessary to classify a patient as in remission off medication. The criteria for in remission off medications should predict with 95% accuracy that a patient has a 20% probability of disease relapse within the next 5 years.

  12. Home Care Guide on Juvenile Rheumatoid Arthritis (For Parents).

    Science.gov (United States)

    Giesecke, Linda L.; And Others

    The booklet, written by the medical staff of a children's hospital, provides information for parents of children with juvenile rheumatoid arthritis (JRA). Arthritis is a swelling of the joint(s) in children and lasts for over 6 weeks (sometimes many years). Aspirin is the main medicine given for JRA, and it works not only to control pain but also,…

  13. Home Care Guide on Juvenile Rheumatoid Arthritis (For Parents).

    Science.gov (United States)

    Giesecke, Linda L.; And Others

    The booklet, written by the medical staff of a children's hospital, provides information for parents of children with juvenile rheumatoid arthritis (JRA). Arthritis is a swelling of the joint(s) in children and lasts for over 6 weeks (sometimes many years). Aspirin is the main medicine given for JRA, and it works not only to control pain but also,…

  14. Experience with conservative rehabilitation in patients with juvenile chronic arthritis

    Directory of Open Access Journals (Sweden)

    T. A. Shelepina

    2016-01-01

    Full Text Available Objective: to estimate a need for conservative rehabilitation treatment in patients with juvenile chronic arthritis (JCA.Material and methods. Data on the principles and procedures of rehabilitation treatment were analyzed in patients with JCA on the basis of 25- year experience. The need for these packages of measures in 1999, 2008, and 2014 was compared. Standard procedures for joints at different sites were described. According to the degree of joint functions, there were rehabilitation treatment packages: corrective, mobilization, and general health-improving.Results and discussion. All patients with juvenile arthritis need rehabilitation (physical, psychological, and social. Comparison of the total number of patients who had received rehabilitation treatment in 1999, 2008, and 2014 showed a small trend towards its reduction. This is due to the smaller number of patients with dysfunctions and to the larger number of those without movement disorders who had received adequate treatment in early periods of the disease. The high percentage of patients having limited joint functions needs a mobilization package. Analysis of the data available in the literature and the authors' experience may lead to the conclusion that all patients with JCA need exercise therapy. The latter is a major procedure for physical rehabilitation and should be included in the standards for adjuvant treatment during basic medical therapy. Emphasis is laid on the importance of the early initiation of treatment to prevent incapacitating deformity at early stages of the disease.

  15. Selected issues in diagnostic imaging of spondyloarthritides: psoriatic arthritis and juvenile spondyloarthritis.

    Science.gov (United States)

    Sudoł-Szopińska, Iwona; Płaza, Mateusz; Pracoń, Grzegorz

    2016-01-01

    Spondyloarthritides (also known as spondyloarthropathies) are a group of rheumatic diseases that consists of diversified entities, i.e. ankylosing spondylitis, reactive arthritis, psoriatic arthritis, arthritis in the course of Crohn's disease and ulcerative colitis, and juvenile spondyloarthropathies. In the diagnostics of spondyloarthritides, plain radiography has played a crucial role for years due to its undisputed ability to show distinctive bony changes. Yet as those diseases often manifest themselves by soft tissue pathology and bone marrow inflammation, ultrasonography and magnetic resonance imaging are currently a subject of numerous studies in the quest for setting up diagnostic criteria, especially at early stages of inflammatory processes. In our review, we present an up-to-date insight into classifications, etiopathogenesis and imaging of psoriatic arthritis and juvenile spondyloarthritis.

  16. Selected issues in diagnostic imaging of spondyloarthritides: psoriatic arthritis and juvenile spondyloarthritis

    Science.gov (United States)

    Płaza, Mateusz; Pracoń, Grzegorz

    2016-01-01

    Spondyloarthritides (also known as spondyloarthropathies) are a group of rheumatic diseases that consists of diversified entities, i.e. ankylosing spondylitis, reactive arthritis, psoriatic arthritis, arthritis in the course of Crohn’s disease and ulcerative colitis, and juvenile spondyloarthropathies. In the diagnostics of spondyloarthritides, plain radiography has played a crucial role for years due to its undisputed ability to show distinctive bony changes. Yet as those diseases often manifest themselves by soft tissue pathology and bone marrow inflammation, ultrasonography and magnetic resonance imaging are currently a subject of numerous studies in the quest for setting up diagnostic criteria, especially at early stages of inflammatory processes. In our review, we present an up-to-date insight into classifications, etiopathogenesis and imaging of psoriatic arthritis and juvenile spondyloarthritis. PMID:28115782

  17. 川崎病合并幼年特发性关节炎一例%Kawasaki disease combined with systemic juvenile idiopathic arthritis:a case report

    Institute of Scientific and Technical Information of China (English)

    汤昔康; 陈泽楷; 覃丽君

    2014-01-01

    In clinics,pediatric fever is commonly encountered characterized with a variety of causes, complex condition and rapid changes. This article reported the diagnosis and treatment of a child with typical kawasaki disease (KD)combined with systemic juvenile idiopathic arthritis (SJIA). After the failed therapy of administration of IVIG,the child then developed arthritis. The diagnosis was corrected to KD complicated with SJIA. The symptoms were alleviated after effective treatment. Albeit KD and SJIA share similar manifestations in clinical and laboratory tests,much attention should be paid to distinguish the differences and avoid treatment delay.%临床工作中,儿科发热患儿多见,病因多样,部分患儿病情复杂且变化快。该文报道了1例川崎病合并幼年特发性关节炎患儿的诊治过程。患儿经丙种球蛋白治疗无反应后,出现关节炎症状,修正诊断为川崎病合并全身型幼年特发性关节炎,调整治疗方案后得到缓解。该例提示,川崎病及全身型幼年特发性关节炎虽有许多类似的临床表现及实验室数据,但应根据其不同点进一步鉴别诊断,以防延误治疗。

  18. Imaging of juvenile idiopathic arthritis. Part I: Clinical classifications and radiographs.

    Science.gov (United States)

    Sudoł-Szopińska, Iwona; Matuszewska, Genowefa; Gietka, Piotr; Płaza, Mateusz; Walentowska-Janowicz, Marta

    2016-09-01

    Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals at the developmental age. Radiography is the primary modality employed in the diagnostic imaging in order to identify changes typical of this disease entity and rule out other bone-related pathologies, such as neoplasms, posttraumatic changes, developmental defects and other forms of arthritis. The standard procedure involves the performance of comparative joint radiographs in two planes. Radiographic changes in juvenile idiopathic arthritis are detected in later stages of the disease. Bone structures are assessed in the first place. Radiographs can also indirectly indicate the presence of soft tissue inflammation (i.e. in joint cavities, sheaths and bursae) based on swelling and increased density of the soft tissue as well as dislocation of fat folds. Signs of articular cartilage defects are also seen in radiographs indirectly - based on joint space width changes. The first part of the publication presents the classification of juvenile idiopathic arthritis and discusses its radiographic images. The authors list the affected joints as well as explain the spectrum and specificity of radiographic signs resulting from inflammatory changes overlapping with those caused by the maturation of the skeletal system. Moreover, certain dilemmas associated with the monitoring of the disease are reviewed. The second part of the publication will explain issues associated with ultrasonography and magnetic resonance imaging, which are more and more commonly applied in juvenile idiopathic arthritis for early detection of pathological features as well as the disease complications.

  19. Imaging of juvenile idiopathic arthritis. Part I: Clinical classifications and radiographs

    Directory of Open Access Journals (Sweden)

    Iwona Sudoł-Szopińska

    2016-09-01

    Full Text Available Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals at the developmental age. Radiography is the primary modality employed in the diagnostic imaging in order to identify changes typical of this disease entity and rule out other bone-related pathologies, such as neoplasms, posttraumatic changes, developmental defects and other forms of arthritis. The standard procedure involves the performance of comparative joint radiographs in two planes. Radiographic changes in juvenile idiopathic arthritis are detected in later stages of the disease. Bone structures are assessed in the first place. Radiographs can also indirectly indicate the presence of soft tissue inflammation (i.e. in joint cavities, sheaths and bursae based on swelling and increased density of the soft tissue as well as dislocation of fat folds. Signs of articular cartilage defects are also seen in radiographs indirectly – based on joint space width changes. The first part of the publication presents the classification of juvenile idiopathic arthritis and discusses its radiographic images. The authors list the affected joints as well as explain the spectrum and specificity of radiographic signs resulting from inflammatory changes overlapping with those caused by the maturation of the skeletal system. Moreover, certain dilemmas associated with the monitoring of the disease are reviewed. The second part of the publication will explain issues associated with ultrasonography and magnetic resonance imaging, which are more and more commonly applied in juvenile idiopathic arthritis for early detection of pathological features as well as the disease complications.

  20. Treatment of refractory juvenile idiopathic arthritis via pulse therapy using methylprednisolone and cyclophosphamide

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    Tania Caroline Monteiro de Castro

    Full Text Available CONTEXT: Patients with refractory juvenile idiopathic arthritis can benefit from aggressive therapy. CASE REPORT: We followed the clinical course of 4 patients (2 male, 2 female aged 9.1-17.8 years (mean of 14.5 years with polyarticular onset of juvenile rheumatoid arthritis and one 16-year-old boy with juvenile spondyloarthropathy associated with inflammatory bowel disease. All the juvenile rheumatoid arthritis patients fulfilled the diagnostic criteria established by the American College of Rheumatology. All patients had unremitting arthritis despite maximum therapy. All patients began receiving treatment using intravenous cyclophosphamide at 500-750 mg/m² and intravenous methylprednisolone at 30 mg/kg, for 3 days monthly (1 g maximum. The patients received between 3 and 11 monthly treatments, and/or 3-5 treatments every two months for 12 months, according to the severity of the disease and/or response to the therapy. All but one patient were evaluated retrospectively at the start (time 0 and 6 months (time 1, and 12 months (time 2 after the beginning of the treatment. A rapid and clinically significant suppression of systemic and articular manifestations was seen in all patients. Our results showed the favorable effect of this treatment on the clinical and some laboratory manifestations of juvenile idiopathic arthritis.

  1. Kre-Celazine(®) as a viable treatment for juvenile rheumatoid arthritis/juvenile idiopathic arthritis - a pilot study.

    Science.gov (United States)

    Golini, Jeff; Jones, Wendy Lou

    2014-09-01

    The purpose of this study was to ascertain whether an oral, non-prescription, nutritional supplement compound composed of a proprietary alkali-buffered creatine monohydrate and cetylated fatty acids mixture (Kre-Celazine(®)) was efficacious in reducing or eliminating refractory pain and inflammation, without untoward effects, in Juvenile Rheumatoid Arthritis (JRA), which is also called Juvenile Idiopathic Arthritis (JIA). JRA/JIA is a patho-physiologically complex, chronic childhood autoimmune inflammatory disease of unknown etiology. Numerous studies have unsuccessfully attempted to pinpoint a possible common initiation event. Officially considered an affliction of children below the age of 16 years, an initial diagnosis has been confirmed in infants less than 1 year old, to individuals older then 17 years. In this study, sixteen juveniles, ages 7 through 16 years, experiencing long-standing, unremitting pain and inflammation despite previous use of prescription anti-inflammatory drugs and NSAIDs, were enrolled in a 30-day, open-label clinical study and treated with Kre-Celazine. Efficacy of this nutritional supplement was determined by the juvenile's personal physician and based on observations of the following: (1) significant reduction or elimination of palpable signs of inflammation; (2) renormalization of range of motion; (3) reduction or absence of perceived pain as reported to the physician by the patient; (4) renormalization of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) values. In addition, the individual's previous steroid or non-steroidal anti-inflamatory medication(s) were reduced or eliminated in a stepwise progressive fashion during the study.

  2. The Etiology of Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Rigante, Donato; Bosco, Annalisa; Esposito, Susanna

    2015-10-01

    Over the years, the commonly used term to describe juvenile idiopathic arthritis (JIA) has changed. By definition, JIA includes all types of arthritis with no apparent cause, lasting more than 6 weeks, in patients aged less than 16 years at onset. JIA pathogenesis is still poorly understood: the interaction between environmental factors and multiple genes has been proposed as the most relevant working mechanism to the development of JIA. The concept that various microbes that colonize or infect not only the mucosal surfaces, like the oral cavity, but also the airways and gut might trigger autoimmune processes, resulting in chronic arthritides, and JIA was first drafted at the outset of last century. JIA development might be initiated and sustained by the exposure to environmental factors, including infectious agents which affect people at a young age, depending on the underlying genetic predisposition to synovial inflammation. Many data from patients with JIA suggest a scenario in which different external antigens incite multiple antigen-specific pathways, cytotoxic T cell responses, activation of classical complement cascade, and production of proinflammatory cytokines. In this review, emphasis is paid not only to the potential role of parvovirus B19 and Epstein-Barr virus in primis but also to the general involvement of different bacteria as Salmonella spp., Shigella spp., Campylobacter spp., Mycoplasma pneumoniae, Chlamydophila pneumoniae, Bartonella henselae, and Streptococcus pyogenes for the development of immune-mediated arthritides during childhood. No unequivocal evidence favoring or refuting these associations has been clearly proved, and today, the strict definition of JIA etiology remains unknown. The infection can represent a random event in a susceptible individual, or it can be a necessary factor in JIA development, always in combination with a peculiar genetic background. Further studies are needed in order to address the unsolved questions

  3. Juvenile Battens Disease.

    Science.gov (United States)

    Gayton, Romayne

    1987-01-01

    Ten children diagnosed with juvenile Battens disease were tested over a three-year period in general intelligence, memory, listening and speech, motor skills, and general learning. Results showed that the patients followed a predetermined pattern but that the time span for development of memory, communication, and behavior problems varied greatly.…

  4. Juvenile Battens Disease.

    Science.gov (United States)

    Gayton, Romayne

    1987-01-01

    Ten children diagnosed with juvenile Battens disease were tested over a three-year period in general intelligence, memory, listening and speech, motor skills, and general learning. Results showed that the patients followed a predetermined pattern but that the time span for development of memory, communication, and behavior problems varied greatly.…

  5. Secondary Osteoporosis in Patients with Juvenile Idiopathic Arthritis

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    Kristyna Brabnikova Maresova

    2011-01-01

    Full Text Available Bone disease in patients with juvenile idiopathic arthritis (JIA is associated with focal (joint erosion and juxtaarticular osteopenia and systemic bone loss (generalized osteopenia or reduction of bone mass density. Pathophysiology of bone loss is multifactorial and involves particularly proinflammatory cytokines and deleterious effects of glucocorticoid therapy. Clinical studies in patients with JIA indicate excessive activation of osteoclastogenesis and reduction of bone formation. Reduction of physical activity, muscle atrophy caused by high disease activity, and compulsory restriction in movements are also associated with bone loss. In patients with JIA, the disease can be complicated by growth cartilage involvement and systemic or local growth retardation. In the absence of preventive measures, fragility fractures can occur even at an early age.

  6. Effect of methotrexate on the temporomandibular joint and facial morphology in juvenile rheumatoid arthritis patients.

    Science.gov (United States)

    Ince, D O; Ince, A; Moore, T L

    2000-07-01

    Juvenile rheumatoid arthritis is a disease characterized by chronic inflammation in one or more joints; it affects children and adolescents up to 18 years of age. This disease may cause significant skeletal joint destruction, and the temporomandibular joint, like other joints, may become severely affected resulting in aberrant mandibular growth, abnormal dentofacial development, and/or altered orofacial muscle function. Methotrexate is the most common remittive agent used in juvenile rheumatoid arthritis to modify the course of inflammatory destruction of peripheral joints. The purpose of this study was: (1) to evaluate the effect of methotrexate therapy on the prevalence of temporomandibular joint lesions and aberration in craniofacial development in children afflicted with juvenile rheumatoid arthritis; (2) to further examine the relationship between the temporomandibular joint/cephalometric findings and rheumatologic data (ie, age at onset, duration of disease); and (3) to evaluate further pauciarticular- and polyarticular-onset disease in juvenile rheumatoid arthritis and the prevalence of temporomandibular joint lesions and facial dysmorphology. The following information was obtained from 45 patients with juvenile rheumatoid arthritis: (1) routine rheumatologic clinical examination data; (2) anamnestic temporomandibular joint evaluation data; (3) clinical temporomandibular joint examination data; (4) lateral cephalometric measurement data; (5) posteroanterior cephalometric measurement data; and (6) individually corrected axial tomographic data. The results demonstrated the following: (1) radiographic evidence of condylar degeneration was apparent in 63% of all patients with juvenile rheumatoid arthritis with pauciarticular patients showing less temporomandibular involvement than polyarticular patients; (2) polyarticular juvenile rheumatoid arthritis patients receiving methotrexate showed less severe temporomandibular joint involvement than the polyarticular

  7. Etanercept in the treatment of disease-modifying anti-rheumatic drug (DMARD)-refractory polyarticular course juvenile idiopathic arthritis: experience from Japanese clinical trials.

    Science.gov (United States)

    Mori, Masaaki; Takei, Syuji; Imagawa, Tomoyuki; Imanaka, Hiroyuki; Nerome, Yasuhito; Kurosawa, Rumiko; Kawano, Yoshifumi; Yokota, Shumpei; Sugiyama, Noriko; Yuasa, Hirotoshi; Fletcher, Tracey; Wajdula, Joseph S

    2011-12-01

    Efficacy, safety, and pharmacokinetics results from 4 studies-3 open-label (OL) and 1 randomized double-blind (DB)-have provided data for approval of etanercept for treatment of disease-modifying anti-rheumatic drug (DMARD)-refractory juvenile idiopathic arthritis (JIA) in Japan. Results from the 3 shorter-term (2 OL and 1 DB) studies are reported here. Subjects (4-17 years) enrolled in the OL studies had active JIA, i.e. ≥5 swollen joints and ≥3 joints with limitation of motion and pain or tenderness. Subjects enrolled in the primary OL study received etanercept 0.4 mg/kg subcutaneously twice weekly; in the lower-dose OL study subjects received etanercept 0.2 mg/kg. Subjects in the primary OL study who completed ≥48 weeks could continue into a 12-week DB dose-down extension study in which subjects received etanercept 0.4 or 0.2 mg/kg twice weekly. The primary endpoint in all 3 studies, i.e. 30% improvement in the American College of Rheumatology criteria for JIA (ACR Pedi 30) at 12 weeks, was achieved by ≥80% of subjects by week 2 and sustained to week 12. Common adverse events reported were injection site reactions, nasopharyngitis, and gastroenteritis. These results provide further evidence that etanercept is effective therapy for DMARD-refractory polyarticular JIA patients.

  8. Is this acute lymphoblastic leukaemia or juvenile rheumatoid arthritis.

    Science.gov (United States)

    Kirubakaran, Chellam; Scott, Julius Xavier; Ebenezer, Sam

    2011-08-01

    Arthritis could be a presenting feature of acute lymphoblastic leukaemia (ALL) and could be wrongly diagnosed as juvenile rheumatoid arthritis (JRA). Clinical and laboratory parameters might differentiate ALL and JRA in children who present with arthritis. Out of a total of 250 children of ALL, 10 were referred to the department of child health and paediatric haemato-oncology of Christian Medical College, Vellore during 1990-2002. They were compared with 10 age-matched children who had systematic onset of JRA. The age groups in ALL and JRA were 6.05 +/- 2.45 years and 5.47 +/- 4.4 years respectively. Severe pain as evidenced by inability to walk was found in children but one child with JRA was unable to walk (p JRA group. ESR was elevated in all cases in both the groups. One case in each group had antinuclear antibody positivity. It can be concluded that ALL can masquerade as systematic onset of JRA. So paediatricians should be careful enough while diagnosing the disease process.

  9. Uveíte na artrite idiopática juvenil Uveitis in juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Adriana M. Roberto

    2002-02-01

    üente na população de pacientes com AIJ associada com uveíte (60% do que naqueles sem uveíte (12% (pObjective: to evaluate the frequency of chronic anterior uveitis in patients with juvenile idiopathic arthritis and its association with the presence of antinuclear antibodies. Patients and methods: we retrospectively studied 72 patients with juvenile idiopathic arthritis. All of them were submitted to slit-lamp examination of the anterior chamber at diagnosis. Both antinuclear antibodies and rheumatoid factor were determined. Patients with positive results for antinuclear antibodies were evaluated every three months and those with negative results were assessed every six months.Results: forty patients were male (55.5% and 36 were Caucasoid (50%. The mean age at the onset of juvenile idiopathic arthritis was 6.4 years (range = 1 to 14 years and the mean age at the beginning of the study was 10.4 years (1 to 19 years. According to the type of disease at onset, 32 were pauciarticular (44.4% (17 boys and 15 girls, 30 were polyarticular (41.6% (17 boys and 13 girls and 10 were systemic (14% (6 boys and 4 girls. We observed chronic anterior uveitis in five patients (6.5% (mean age = 11.4 years. Among them, four (80% had pauciarticular juvenile idiopathic arthritis at disease onset (three girls with type I juvenile idiopathic arthritis and positive antinuclear antibodies and one boy with type I juvenile idiopathic arthritis and negative antinuclear antibodies and one girl with polyarticular juvenile idiopathic arthritis (negative antinuclear antibodies and rheumatoid factor. In this group, the mean age at the onset of juvenile idiopathic arthritis was 5.1 years and the mean age of uveitis onset was 9 years. Antinuclear antibodies were positive in 3/5 patients (60% with uveitis. Antinuclear antibodies were positive in 12% of the patients without uveitis (n = 67. Among the patients with uveitis, three had only one flare and the other two had four flares with cataract. The

  10. Altered signaling in systemic juvenile idiopathic arthritis monocytes.

    Science.gov (United States)

    Macaubas, Claudia; Wong, Elizabeth; Zhang, Yujuan; Nguyen, Khoa D; Lee, Justin; Milojevic, Diana; Shenoi, Susan; Stevens, Anne M; Ilowite, Norman; Saper, Vivian; Lee, Tzielan; Mellins, Elizabeth D

    2016-02-01

    Systemic juvenile idiopathic arthritis (sJIA) is characterized by systemic inflammation and arthritis. Monocytes are implicated in sJIA pathogenesis, but their role in disease is unclear. The response of sJIA monocytes to IFN may be dysregulated. We examined intracellular signaling in response to IFN type I (IFNα) and type II (IFNγ) in monocytes during sJIA activity and quiescence, in 2 patient groups. Independent of disease activity, monocytes from Group 1 (collected between 2002 and 2009) showed defective STAT1 phosphorylation downstream of IFNs, and expressed higher transcript levels of SOCS1, an inhibitor of IFN signaling. In the Group 2 (collected between 2011 and 2014), monocytes of patients with recent disease onset were IFNγ hyporesponsive, but in treated, quiescent subjects, monocytes were hyperresponsive to IFNγ. Recent changes in medication in sJIA may alter the IFN hyporesponsiveness. Impaired IFN/pSTAT1 signaling is consistent with skewing of sJIA monocytes away from an M1 phenotype and may contribute to disease pathology.

  11. Fever of unknown origin in a patient of systemic onset juvenile idiopathic arthritis

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    Vinod Kolar Vishwanath

    2010-01-01

    Full Text Available Hemophagocytic lymphohistiocytosis is a potentially fatal condition characterized by pathologic immune activation, which can complicate infections, childhood systemic rheumatologic diseases and malignancies. Here we report a case of reactive hemophagocytic lymphohistiocytosis [macrophage activation syndrome] complicating systemic onset juvenile idiopathic arthritis, which was treated successfully with dexamethasone and cyclosporine. Reactive hemophagocytic lymphohistiocytosis or macrophage activation syndrome should be considered in patients of juvenile idiopathic arthritis with prolonged fever of unknown origin and cytopenias. Early diagnosis with high index of suspicion and prompt, aggressive treatment are needed for successful outcomes.

  12. Clinical Case of Tocilizumab Use in a Patient with Systemic Juvenile Idiopathic Arthritis

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    Y. M. Spivakovskiy

    2015-01-01

    Full Text Available The article presents a case of using genetically engineered biopharmaceutical tocilizumab in a child with systemic juvenile idiopathic arthritis. On the initial stage, the treatment was characterized by resistance to high doses of glucocorticoids and cytostatic drugs. Successful termination of visceral and articular manifestations of systemic juvenile idiopathic arthritis and normalization of laboratory indicators of disease activity in the setting of use of interleukin 6 receptor blocker were described. We observed stable improvement of the child’s condition during a year-long follow-up in the setting of the selected anti-inflammatory therapy pattern. 

  13. Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease

    OpenAIRE

    Kim, Susan; Kahn, Philip; Robinson, Angela B; Lang, Bianca; Shulman, Andrew; Oberle, Edward J.; Schikler, Kenneth; Curran, Megan Lea; Barillas-Arias, Lilliana; Spencer, Charles H; Rider, Lisa G; Huber, Adam M.

    2017-01-01

    Background Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript...

  14. Muscle involvement in juvenile idiopathic arthritis.

    Science.gov (United States)

    Lindehammar, H; Lindvall, B

    2004-12-01

    An observational study of changes in muscle structure and the relation to muscle strength in juvenile idiopathic arthritis (JIA). Fifteen children and teenagers (eight girls and seven boys) with JIA, aged 9-19 yr (mean age 16.1), were studied. Muscle biopsies were obtained from the anterior tibial muscle and were examined using histopathological and immunohistochemical methods. Muscle fibre types were classified and fibre areas measured. As markers of inflammation, the major histocompatibility complex (MHC) class I and class II and the membrane attack complex (MAC) were analysed. Results were compared with biopsies from the gastrocnemius muscle in 33 young (19-23 yr) healthy controls. Isometric and isokinetic muscle strengths were measured in ankle dorsiflexion. Strength was compared with reference values for healthy age-matched controls. Nerve conduction velocities were recorded in the peroneal and sural nerves. Four of the 15 muscle biopsies were morphologically normal. Eleven biopsies showed minor unspecific changes. Two of these also showed minor signs of inflammation. MHC class II expression was found in 4/15 patients, which was significantly more than in the healthy controls (P = 0.0143). The expression of MHC class I and MAC did not differ from that in the controls. The mean area of type I fibres was lower than that of type IIA fibres in 12/13 biopsies. Muscle strength was significantly reduced in the patient group. There was a significant positive correlation between muscle fibre area and muscle strength. Nerve conduction studies were normal in all cases. Changes in leg muscle biopsies appear to be common in children and teenagers with JIA. The presence of inflammatory cells in the muscle and expression of MHC class II on muscle fibres may be a sign of inflammatory myopathy. There are no findings of type II muscle fibre hypotrophy or neuropathy, as in adults with RA.

  15. Do You Have a Child with Juvenile Rheumatoid Arthritis in Your Class?

    Science.gov (United States)

    Ferris, Jean; Fujishige, Carole

    The booklet provides information to help teachers understand juvenile rheumatoid arthritis (JRA). JRA is a chronic disease involving one or more joint(s); its cause is unknown. The five types of JRA are monarticular, pauciarticular of young girls, pauciarticular of boys, polyarticular, and systemic. Aspirin is the main treatment medication and…

  16. Aerobic and anaerobic exercise capacity in children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    van Brussel, Marco; Lelieveld, O T H M; van der Net, J; Engelbert, R H H; Helders, P J M; Takken, T

    2007-01-01

    Objective. To compare the aerobic and anaerobic exercise capacity of children with juvenile idiopathic arthritis (JIA) with healthy controls, to determine if there were differences based on disease onset type, and to examine the relationship between aerobic and anaerobic exercise capacity in childre

  17. Aerobic and anaerobic exercise capacity in adolescents with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Lelieveld, Otto; van Brussel, Marco; Takken, Tim; van Weert, Ellen; van Leeuwen, Miek A.; Armbrust, Wineke

    2007-01-01

    OBJECTIVE: To examine the aerobic and anaerobic exercise capacity in adolescents with juvenile idiopathic arthritis (JIA) compared with age- and sex-matched healthy individuals, and to assess associations between disease-related variables and aerobic and anaerobic exercise capacity. METHODS: Of 25 p

  18. CURRENT VIEW ON SYSTEMIC GLUCOCORTICOSTEROID THERAPY IN JUVENILE RHEUMATOID ARTHRITIS

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    N N Kuzmina

    2000-01-01

    Full Text Available Aim: To present modern approaches to the systemic therapy by glucocorticosteroids (GCS basing on own experience and literature data. Methods and material: Long-term observation of 350 patients with juvenile rheumatoid arthritis (JRA taking peroral GCS in different dosage. Results: Good therapeutical efficacy and sufficient tolerability of low starting doses (lower than 0.5 mg/ kg a day of GCS allow to inhibit inflammatory activity in the majority of patients. Alternative method (doses alternation is recommended in the period of long-term supporting GCS-therapv of JR.4. Conclusion: Basic strategy of treatment of systemic and polyarticular JRA j'orms is rational GCS application in combination with basic drugs which ensures control of pathologic process and modifies the disease.

  19. Updates on the risk markers and outcomes of severe juvenile idiopathic arthritis-associated uveitis

    Science.gov (United States)

    Angeles-Han, Sheila T; Yeh, Steven; Vogler, Larry B

    2013-01-01

    Uveitis is the most common extra-articular manifestation of juvenile idiopathic arthritis, which is the most common systemic cause of uveitis in children. Known risk factors for uveitis include antinuclear antibody seropositivity, young age of arthritis onset, specific juvenile idiopathic arthritis subtype and short duration of disease. Risk markers for severe ocular disease include gender, age and complications at initial visit. Due to the risk for vision-compromising sequelae such as cataracts, band keratopathy, glaucoma, vision loss and blindness, an understanding of the risk factors for uveitis development and severe ocular disease is crucial to help prevent serious visual disability and complications. This paper reviews the pathogenesis of uveitis, known risk factors for uveitis development and severe visual outcome, and addresses the need for additional biomarkers of uveitis risk, prognosis and remission. PMID:24187594

  20. Juvenile idiopathic arthritis outcome and prognosis according to catamnesis evaluation

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    O V Semenova

    2005-01-01

    Full Text Available Objective. To study outcome and prognosis in pts with juvenile idiopathic arthritis (JIA. Material and methods. 239 JIA pts with disease duration of 10 years and more were analyzed. 80 children and adolescents before 18 years old (66 girls and 14 boys were included in group 1. Arthritis clinical and laboratory activity, radiological stage and functional status according to Steinbroker and CHAQ questionnaire were assessed. 159 grown up pts (109 female and 50 male suffering from JIA from childhood were included in group 2. They were examined using Stanford Health Assessment Questionnaire (HAQ and a specially developed social status questionnaire. Results. Half pts of group 1 had recurrence of the disease during examination but activity in most cases did not exceed stage I or 2 (54% and 31% respectively. Joint destructive changes were revealed in 50% of pts. 80% of pts had radiological signs of secondary osteoarthritis. Amyloidosis was revealed in 2 from 27 pts with systemic type of JIA. 68% of pts had 1 or 2 functional class. 1/3 of pts did not have functional limitations (CHAQ=0. 13% of pts had maximal disability with CHAQ ranging from 2,1 to 3,0 (pts with polyarticular and systemic types of JIA. Most grown up pts (59% considered their health as good. Substantial part of them worked or learned. 10 pts did not worked because of the disease (1,5%. 61% of pts did not have functional limitations (HAQ=0. 32% of pts were disabled. Most of them had 3 or 2 disability degree and had possibility to work. Conclusion. Substantial part of pts with longstanding JIA have stabilization of the disease or remission. Recurrent course of the disease was characterized by decrease of activity. Most children and grown ups with longstanding disease have relatively benign functional outcome. Pts with polyarticular and systemic types of JIA require especial attention because they have maximal risk of joint destruction with severe disability.

  1. Imaging of the temporomandibular joint in juvenile idiopathic arthritis.

    Science.gov (United States)

    Vaid, Yoginder N; Dunnavant, F Daniel; Royal, Stuart A; Beukelman, Timothy; Stoll, Matthew L; Cron, Randy Q

    2014-01-01

    Temporomandibular joint (TMJ) arthritis in children with juvenile idiopathic arthritis (JIA) is extremely common but frequently asymptomatic. Magnetic resonance imaging (MRI) with contrast remains the gold standard for identifying TMJ arthritis in JIA. A reliable scoring system with published MRI examples of typical acute and chronic TMJ arthritis changes will be invaluable for future prospective treatment trials of TMJ arthritis in JIA. MRIs were collected from routine clinical studies assessing TMJ arthritis in JIA. Representative images were selected for publication to depict acute (synovial fluid, bone marrow edema, and synovial enhancement) and chronic (pannus, disc derangement, and condylar head flattening and erosions) TMJ arthritis findings. A preliminary MRI-based scoring system for assessing degrees of acute and chronic TMJ arthritis was developed and tested for inter- and intrareader reliability. TMJ MRIs representative of acute and chronic TMJ arthritis in JIA were selected from among thousands taken (>500 TMJ MRI studies annually at Children's of Alabama) since September 2007. Moreover, computed tomography scans depicting select bony changes (osteophyte formation, micrognathia) were chosen for publication. A description of the MRI protocol for assessing TMJ arthritis is included. A preliminary scoring system weighted for degree of acute and chronic TMJ arthritis MRI findings was found to have substantial inter- and intrareader reliability. A published set of MRIs depicting representative acute and chronic changes will help establish a standardized scoring system to assess TMJ arthritis in children with JIA. Future validation will aid in assessing improvement during treatment trials of TMJ arthritis. Copyright © 2014 by the American College of Rheumatology.

  2. MRI findings of juvenile psoriatic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Edward Y.; Kleinman, Paul K. [Harvard Medical School, Department of Radiology, Boston, MA (United States); Children' s Hospital Boston, MA (United States); Sundel, Robert P.; Kim, Susan [Harvard Medical School, Rheumatology Program, Division of Immunology and the Department of Pediatrics, Boston, MA (United States); Children' s Hospital Boston, MA (United States); Zurakowski, David [Harvard Medical School, Department of Radiology, Boston, MA (United States); Harvard Medical School, Department of Orthopaedic Surgery, Boston, MA (United States); Children' s Hospital Boston, MA (United States)

    2008-11-15

    The aim of this study was to describe the magnetic resonance imaging (MRI) features of juvenile psoriatic arthritis (JpsA) in children in order to facilitate early diagnosis and proper management. Two pediatric radiologists retrospectively reviewed in consensus a total of 37 abnormal MRI examinations from 31 pediatric patients (nine boys, 22 girls; age range 1-17 years; mean age 9.4 years) who had a definite diagnosis of JpsA and underwent MRI. Each MRI was evaluated for synovium abnormality (thickening and enhancement), joint effusion (small, moderate, and large), bone marrow abnormality (edema, enhancement, and location of abnormality), soft tissue abnormality (edema, enhancement, atrophy, and fatty infiltration), tendon abnormality (thickening, edema, tendon sheath fluid, and enhancement), and articular abnormality (joint space narrowing and erosion). The distribution of abnormal MRI findings among the six categories for the 37 MRI examinations was evaluated. The number of abnormal MRI findings for each MRI examination was assessed. Age at MRI examination and all six categories of abnormal MRI findings according to gender were evaluated. There were a total 96 abnormal MRI findings noted on 37 abnormal MRI examinations from 31 pediatric patients. The 37 abnormal MRI examinations included MRI of the hand (n=8), knee (n = 8), ankle (n = 5), pelvis (n = 5), temporomandibular joint (n = 4), wrist (n = 3), foot (n = 2), elbow (n = 1), and shoulder (n = 1). Twenty-eight diffuse synovial thickening and/or enhancement were the most common MRI abnormality (29.2%). Joint effusion comprised 22 abnormal MRI findings (22.9%). There were 16 abnormal MRI bone marrow edema and/or enhancement findings (16.7%), and in seven (7.3%) the edema involved non-articular sites. Soft tissue abnormality manifested as edema and/or enhancement constituted 14 abnormal MRI findings (14.5%). There were ten MRI abnormalities (10.4%) involving tendons. Articular abnormality seen as joint space

  3. EXPERIENCE WITH ABATACEPT IN TREATMENT OF JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    Margarita Fedorovna Dubko

    2012-01-01

    Full Text Available Persistent interest to Abatacept (ABA keeps growing due to continuous inflow of consistent efficacy and safety data from successful clinical trials. The objective of our retrospective trial was to evaluate ABA efficacy and safety in treatment of juvenile idiopathic arthritis (JIA in biologic-naive patients. 20 patients aged 3—17 y.o. were included into the study. All included cases had a long duration of the disease, high values of clinical and laboratory indicators of JIA activity corresponding to moderate and severe course of arthritis. 70% achieved improvement in ACR 30 after average 8 months treatment with ABA (duration range 3—20 mo. Best clinical responses were observed in patients with systemic JIA subtype (but without obvious clinical manifestations in all 3 cases out of 3 included, and polyarticular subtype — in 9 patients out of 11. 6 patients (30,0% discontinued treatment. Main reasons for discontinuing treatment were absence or lack of efficacy — in 4 cases, poor adherence — in 1 case, and adverse reactions — in 1 case. Hopefully these data will help practicing physicians with adequate choice of treatment.

  4. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis.

    Science.gov (United States)

    Havemose-Poulsen, Anne; Westergaard, Jytte; Stoltze, Kaj; Skjødt, Henrik; Danneskiold-Samsøe, Bente; Locht, Henning; Bendtzen, Klaus; Holmstrup, Palle

    2006-02-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile idiopathic arthritis (JIA), and rheumatoid arthritis (RA) share periodontal and hematological characteristics distinguishing them from individuals free of diseases. The study population consisted of white adults (rheumatoid factors (RFs), and antibodies to cyclic citrullinated peptides. RA patients had a higher percentage of sites with PD>or=4 mm, CAL>or=2 mm, and ABL>or=2 mm compared to controls. The percentage of sites with CAL>or=2 mm significantly correlated with the levels of IgM-RF and IgA-RF. Missing teeth in JIA and RA patients were not lost due to periodontitis. Patients with GAgP showed higher levels of leukocytes, including neutrophils, and CRP compared to controls. In part, JIA and RA patients showed similar results. Young adults with RA may develop periodontal destruction, and these patients require professional attention. Both differences and similarities in periodontal and hematological variables were seen in individuals with periodontitis, JIA, and RA.

  5. The adolescent experience in Juvenile Idiopathic Arthritis: A narrative approach

    NARCIS (Netherlands)

    Fuchs, C.E.|info:eu-repo/dai/nl/304820369

    2013-01-01

    This dissertation focused on the self-experience of adolescents with juvenile idiopathic arthritis (JIA) or chronic fatigue syndrome (CFS). Although the etiology and nosology of JIA and CFS are fundamentally different, some commonalities in the emotional experience of adolescents dealing with these

  6. Ultrasonography and color Doppler in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Nielsen, Susan;

    2012-01-01

    The wrist region is one of the most complex joints of the human body. It is prone to deformity and functional impairment in juvenile idiopathic arthritis (JIA), and is difficult to examine clinically. The aim of this study was to evaluate the role of ultrasonography (US) with Doppler in diagnosis...

  7. Randomized Trial of Tocilizumab in Systemic Juvenile Idiopathic Arthritis

    NARCIS (Netherlands)

    De Benedetti, Fabrizio; Brunner, Hermine I.; Ruperto, Nicolino; Kenwright, Andrew; Wright, Stephen; Calvo, Inmaculada; Cuttica, Ruben; Ravelli, Angelo; Schneider, Rayfel; Woo, Patricia; Wouters, Carine; Xavier, Ricardo; Zemel, Lawrence; Baildam, Eileen; Burgos-Vargas, Ruben; Dolezalova, Pavla; Garay, Stella M.; Merino, Rosa; Joos, Rik; Grom, Alexei; Wulffraat, Nico; Zuber, Zbigniew; Zulian, Francesco; Lovell, Daniel; Martini, Alberto

    2012-01-01

    BACKGROUND Systemic juvenile idiopathic arthritis (JIA) is the most severe subtype of JIA; treatment options are limited. Interleukin-6 plays a pathogenic role in systemic JIA. METHODS We randomly assigned 112 children, 2 to 17 years of age, with active systemic JIA (duration of >= 6 months and inad

  8. Causes of uveitis in children without juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Engelhard SB

    2015-06-01

    Full Text Available Stephanie B Engelhard, Asima Bajwa, Ashvini K ReddyDepartment of Ophthalmology, University of Virginia, Charlottesville, VA, USABackground: The purpose of this study was to report the demographics, disease characteristics, treatments, and visual outcomes of pediatric uveitis patients without juvenile idiopathic arthritis managed in a tertiary medical center.Methods: A retrospective, observational study was performed in pediatric uveitis patients without juvenile idiopathic arthritis and aged 0–18 years, who were seen at the University of Virginia from 1984 to 2014.Results: Thirty-nine pediatric uveitis patients (57 eyes were identified. The patient population was 51.28% female, 51.28% Caucasian, and 33.33% African American. The mean age at diagnosis was 11.9 years. The mean duration of follow-up was 3.11 years. The mean number of visits to the clinic was 10.41. Of 57 eyes, 31 (54.39% had anterior uveitis, 12 (21.05% had intermediate uveitis, nine (15.79% had posterior uveitis, and five (8.77% had panuveitis. The leading diagnoses were traumatic uveitis (25.64%, undifferentiated anterior uveitis (17.95%, undifferentiated intermediate uveitis (15.38%, HLA-B27-associated anterior uveitis (7.69%, and herpetic anterior uveitis (7.69%. Systemic associations included sarcoidosis, ulcerative colitis, and psoriatic arthritis (n=3. The most common treatment modalities included local steroids (66.67%, systemic steroids (23.08%, and antimetabolites (20.51%. Ocular hypertension was found in five (12.82% patients. Ocular surgery was performed in six (15.38% patients. Mean best-corrected visual acuity (BCVA at baseline across all anatomical locations was 0.458 logMAR, and was 0.411 logMAR at final follow-up. Mean BCVA improved during follow-up in all but the anterior uveitis group. The mean baseline intraocular pressure was 14.27 mmHg, and was 14.22 mmHg at final follow-up.Conclusion: Uveitis in childhood is a vision-threatening group of inflammatory

  9. Challenges in the management of juvenile idiopathic arthritis with etanercept

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    Clare E Pain

    2009-03-01

    Full Text Available Clare E Pain, Liza J McCannAlder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool, UKAbstract: Biologic agents have been designed with the help of immunological studies to target particular areas of the immune system which are thought to play a role in the pathogenesis of disease. Etanercept is a soluble anti-tumor necrosis factor alpha (TNF-α agent licensed for the treatment of active poly-articular juvenile idiopathic arthritis (JIA in children aged 4 to 17 years who have failed to respond to methotrexate alone, or who have been intolerant of methotrexate. The safety and efficacy of etanercept in this patient group has been established by one randomized controlled trial and several longitudinal studies. This, together with the fact that until recently etanercept was the only anti-TNF licensed in JIA, has made it the most common first choice biologic for many clinicians. However, there are still many unanswered questions about etanercept, including its efficacy and safety in different subtypes of JIA, in children under 4 years of age and in those with uveitis. There are still concerns about the long term safety of TNF antagonists in the pediatric age group and unanswered questions about increased risks of malignancy and infection. Although adult studies are useful to improve understanding of these risks, they are not a substitute for good quality pediatric research and follow-up studies. Adult trials often include greater numbers of patients. However, they evaluate a different population and drug behavior may vary in children due to differences in metabolism, growth and impact on a developing immune system. In addition, rheumatoid arthritis is a different disease than JIA. Clinicians need to carefully weigh up the risk benefit ratio of anti-TNF use in children with JIA and push for robust clinical trials to address the questions that remain unanswered. This article summarizes the evidence available for use of etanercept in children

  10. Reduced articular cartilage thickness in joints without a history of active arthritis in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Pradsgaard, Dan Østergaard; Spannow, Anne Helene; Heuck, Carsten;

    Background: The functional disability experienced in juvenile idiopathic arthritis is primarily caused by degeneration of the osteocartilaginous structures due to the inflammatory process in the synovium. It is therefore essential for evaluating the therapeutic efficacy to closely monitor...... the structural damage during the disease course. During the past decade musculoskeletal ultrasound (US) has become an established diagnostic method in adult rheumatology and within recent years an increased attention for utilizing US in pediatric rheumatology has emerged. Previously we have found differences...

  11. Juvenile rheumatoid arthritis. Aquatic exercise and lower-extremity function.

    Science.gov (United States)

    Bacon, M C; Nicholson, C; Binder, H; White, P H

    1991-06-01

    This pilot study investigates the effects of aquatic therapeutic exercise on lower-extremity range of motion, gait, balance, and functional mobility in children with juvenile arthritis. Eleven patients, aged 4-13, with lower-extremity joint involvement, diagnosed as functional class I-III, completed a 6-week program of aquatic exercise aimed at increasing lower-extremity range of motion and strength. Despite the small sample size and short duration of the study program, significant improvement was noted in external and internal hip rotation, bilaterally (p Aquatic exercises performed in a group setting can serve as an enjoyable and beneficial part of therapy for children with arthritis. Further investigation is recommended to determine fully the effects of aquatic therapeutic exercise on mobility and fitness in children with juvenile arthritis.

  12. Dynamic contrast-enhanced magnetic resonance imaging of the wrist in children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Nusman, Charlotte M. [Emma Children' s Hospital, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Academic Medical Center, Amsterdam (Netherlands); Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Lavini, Cristina; Hemke, Robert; Caan, Matthan W.A.; Maas, Mario [Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Schonenberg-Meinema, Dieneke; Berg, J.M. van den; Kuijpers, Taco W. [Emma Children' s Hospital, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Academic Medical Center, Amsterdam (Netherlands); Dolman, Koert M. [Sint Lucas Andreas Hospital, Department of Pediatrics, Amsterdam (Netherlands); Reade Institute location Jan van Breemen, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Rossum, Marion A.J. van [Reade Institute location Jan van Breemen, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Emma Children' s Hospital, Department of Pediatrics, Academic Medical Center, Amsterdam (Netherlands)

    2017-02-15

    Dynamic contrast-enhanced MRI provides information on the heterogeneity of the synovium, the primary target of disease in children with juvenile idiopathic arthritis (JIA). To evaluate the feasibility of dynamic contrast-enhanced MRI in the wrist of children with JIA using conventional descriptive measures and time-intensity-curve shape analysis. To explore the association between enhancement characteristics and clinical disease status. Thirty-two children with JIA and wrist involvement underwent dynamic contrast-enhanced MRI with movement-registration and were classified using validated criteria as clinically active (n = 27) or inactive (n = 5). Outcome measures included descriptive parameters and the classification into time-intensity-curve shapes, which represent the patterns of signal intensity change over time. Differences in dynamic contrast-enhanced MRI outcome measures between clinically active and clinically inactive disease were analyzed and correlation with the Juvenile Arthritis Disease Activity Score was determined. Comprehensive evaluation of disease status was technically feasible and the quality of the dynamic dataset was improved by movement registration. The conventional descriptive measure maximum enhancement differed significantly between clinically active and inactive disease (P = 0.019), whereas time-intensity-curve shape analysis showed no differences. Juvenile Arthritis Disease Activity Score correlated moderately with enhancing volume (P = 0.484). Dynamic contrast-enhanced MRI is a promising biomarker for evaluating disease status in children with JIA and wrist involvement. Conventional descriptive dynamic contrast-enhanced MRI measures are better associated with clinically active disease than time-intensity-curve shape analysis. (orig.)

  13. Nocardia brasiliensis infection mimicking juvenile idiopathic arthritis in a 4-year-old girl.

    Science.gov (United States)

    Kapur, Nitin; Adib, Navid; Grimwood, Keith

    2013-11-01

    Nocardia are ubiquitous environmental saprophytes that cause pneumonia and disseminated disease in immunocompromised patients. They can also cause localized cutaneous and soft tissue infections in healthy people after direct percutaneous inoculation. Nocardia arthritis is rare in both forms of the disease. Here we present the first published case of a child with septic arthritis caused by N brasiliensis. Importantly, this otherwise well 4-year-old girl had no known history of trauma but presented with transient cutaneous lesions and a 6-week history of arthritis involving the right fourth digit proximal interphalangeal joint without accompanying fever or raised systemic inflammatory markers. She received a diagnosis of juvenile idiopathic arthritis and underwent antiinflammatory and immunosuppressant therapy. After 2 months she developed frank septic arthritis, which necessitated a surgical joint washout, from which an intraoperative swab grew N brasiliensis. The patient received 6 months of high-dose trimethoprim-sulfamethoxazole and remains well more than 4 years after treatment. This unusual case highlights the importance of considering an indolent infection from slow-growing organisms, including Nocardia, when diagnosing the oligoarthritis subtype of juvenile idiopathic arthritis. This is especially relevant when a single joint is involved and response to antiinflammatory therapy is suboptimal because antiinflammatory agents may mask evolving signs of infection.

  14. Macrophage activation syndrome in a patient with systemic onset of the juvenile idiopathic arthritis.

    Science.gov (United States)

    Jain, Deepak; Aggarwal, Hari K; Rao, Avinash; Mittal, Anshul; Jain, Promil

    2016-01-01

    Systemic onset juvenile idiopathic arthritis (sJIA) is defined as arthritis affecting one or more joint usually in the juvenile age group (< 16 years of age) with or preceded by fever of at least 2 weeks duration that is documented to be daily ("quotidian") for at least 3 days which may be associated with evanescent (non-fixed) erythematous rash or generalized lymph node enlargement or hepatomegaly/splenomegaly/both or serositis. Macrophage activation syndrome (MAS) is a life-threatening complication of sJIA marked by sudden onset of non-remitting high fever, profound depression in all three blood cell lines (i.e. leukopenia, anemia, and thrombocytopenia), hepatosplenomegaly, lymphadenopathy, and elevated serum liver enzyme levels. In children with systemic juvenile idiopathic arthritis, the clinical picture may mimic sepsis or an exacerbation of the underlying disease. We report a case of a 16-year-old female patient presenting with high grade fever with joint pains and generalized weakness which proved to be systemic onset juvenile idiopathic arthritis with macrophage activation syndrome after ruling out all other differential diagnoses and responded well to intravenous steroids.

  15. Juvenile idiopathic arthritis and the temporomandibular joint

    African Journals Online (AJOL)

    Yasser Mohammed

    2012-02-01

    Feb 1, 2012 ... a Radiology, Ain Shams University hospitals, Faculty of Medicine, Ain Shams University, Cairo, .... pacemakers, metal implants or dental braces were excluded .... Patients with JIA are at high risk of developing arthritis of the.

  16. Complex genetic predisposition in adult and juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Oppermann Joachim

    2004-02-01

    Full Text Available Abstract Background Rheumatoid arthritis (RA and juvenile rheumatoid arthritis (JRA are complex multifactorial diseases caused by environmental influences and an unknown number of predisposing genes. The present study was undertaken in order to investigate association of polymorphisms in candidate genes with RA and JRA in German subjects. Results Up to 200 unrelated German RA and JRA patients each and 300–400 healthy controls have been genotyped for HLA-DRB1, TNFa, TNFA -238a/g, TNFA -308a/g, TNFA -857c/t, TNFR1 -609g/t, TNFR1 P12P, TNFR2 del 15bp, IKBL -332a/g, IKBL -132t/a, IKBL C224R, CTLA4 -318c/t, CTLA4 T17A, PTPRC P57P, MIF -173g/c, the MIF and IFNG microsatellites as well as for D17S795, D17S807, D17S1821 by polyacrylamide gel electrophoresis, single-strand conformation polymorphism analysis, restriction fragment length polymorphism analysis or allele specific hybridization. None of the investigated genetic markers is associated with both, RA and JRA, but there are some statistically significant differences between patients and controls that have to be discussed sensibly. Conclusions The difficulty in investigating the genetics of complex disorders like RA and JRA may arise from genetic heterogeneity in the clinically defined disease cohorts (and generally limited power of such studies. In addition, several to many genes appear to be involved in the genetic predisposition, each of which exerting only small effects. The number of investigated patients has to be increased to establish the possibility of subdivison of the patients according their clinical symptoms, severity of disease, HLA status and other genetic characteristics.

  17. Tumour necrosis factor (TNF)-blocking agents in juvenile psoriatic arthritis: are they effective?

    NARCIS (Netherlands)

    M.H. Otten; F.H.M. Prince; R. ten Cate; M.A.J. van Rossum; M. Twilt; E.P.A.H. Hoppenreijs; Y. Koopman-Keemink; A.P. Oranje; F.B. de Waard-van de Spek; S.L. Gorter; W. Armbrust; K.M. Dolman; N.M. Wulffraat; L.W.A. van Suijlekom-Smit

    2011-01-01

    Objectives To evaluate the effectiveness of tumour necrosis factor (TNF) blockers in juvenile psoriatic arthritis (JPsA). Methods The study was a prospective ongoing multicentre, observational study of all Dutch juvenile idiopathic arthritis (JIA) patients using biologicals. The response of arthriti

  18. POSSIBLE PARENTERAL METHOTREXATE APPLICATION IN THE TREATMENT OF JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    Yu. М. Spivakovsky

    2014-01-01

    Full Text Available The article describes a case of using methotrexate for subcutaneous injection in a rated dose of 12.5 mg / m2 of body surface per week when treating a child with juvenile idiopathic arthritis. When switching to the parenteral route of administration, methotrexate ensured the development of joint syndrome remission, normalization of laboratory indices of disease activity, and improvement of joint functions. After 6 months the inactive disease stage was detected, and after 12 months — remission.

  19. Assessment of joint pain in teenagers with juvenile arthritis

    Directory of Open Access Journals (Sweden)

    T A Shelepina

    2009-01-01

    Full Text Available Assessment of joint pain in teenagers with juvenile arthritisObjective. To assess mean total pain score in teenagers with juvenile arthritis (JA and its values in groups differing on sex, variant of course, activity measures, degree of functional disturbances. To assess advisability of application of these parameters in real clinical practice. Material and methods. 73 pts with JA (mean age 14,7±1,7 years, mean disease duration7,2±4,4 years were included. 18 from them had systemic, 42 – polyarticular and 13 – olygoarticular variant. 16 pts were examined repeatedly with interval exceeding one year. Total quantity of examinations was 94. 17 pts were examined at admission and at discharge. Pain was measured on visual analog scale (VAS and on McGill Pain Questionnaire (83 examinations including 3 scale: sensor (description of pain, affective (their influence on emotional state and evaluative (verbal assessment of pain intensity. General health assessment on VAS was performed in 59 pts (in 8 from them at admission and at discharge. Summary mean measures and their values in groups differing in sex, course variant, activity measure, functional class at pts admission to and at discharge from the Institute of Rheumatology of RAMS. Statistical treatment was performed with Biomed program(descriptive statistic, χ2, Student’s pared test. Results. Mean pain score on VAS in all teenagers with JA was 31,3±17,3. It was significantlyhigher in boys with systemic variant (in comparison with polyarticular, in pts with ESR elevation above 30, in pts with functional class 3 and higher at admission to the department. Pain score was significantly lower in pts with olygoarticular variant in comparison with mean value and with value in polyarthritis. Pain above 70 mm on VAS was found in pts with polyarticular damage in systemic and polyarticular variants in active phase of the disease and in severe functional disability. Summated (on sensor and affective scales

  20. [Juvenile rheumatoid artritis (a single or various diseases?].

    Science.gov (United States)

    Frati Munari, A C; Criollos Torres, O; Flores Suárez, R E

    1977-01-01

    Some characteristics of juvenile rheumatoid arthritis that appeared in recent literature have led us to think that it can be divided into the following four groups: I. Seronegative poliarthritis, with more or less systemic symptoms. With the same characteristics it may appear in adulthood. II. Seropositive poliarthritis, identical to the adult rheumatoid arthritis. III. B-27 negative oligoarthritis, complicated frequently with chronic uveitis and autolimited course. IV. B-27 positive oligoarthritis evolving to ankylosing spondylitis. These groups may represent different diseases.

  1. [Presence of riziform bodies in a patient with juvenile idiopathic arthritis: case report and literature review.

    Science.gov (United States)

    Campos, Leonardo Rodrigues; Sztajnbok, Fernanda Cardoso das Neves; Galvão, Stélio; Lessa, Marise de Araújo; Aymoré, Ierecê Lins; Sztajnbok, Flavio

    2014-10-23

    Riziform bodies are structures formed by fibrin and cells that can be found in the synovial fluid or attached to the synovium, and have this denomination due to its rice grain-like appearance. They have already been described in several diseases such as tuberculous arthritis, rheumatoid arthritis, and rarely in juvenile idiopathic arthritis (JIA). This is the case of a boy with a 4-month course of chronic monoarthritis of the left knee, with family history of sarcoidosis in which diagnostic investigation showed the presence of these riziform bodies in the synovial biopsy. Diagnostic investigation ruled out sarcoidosis, tuberculosis and malignancies, establishing the diagnosis of JIA. Our objective was to describe what we believe is the 9th case reported on the presence of riziform bodies in JIA, which are probably underdiagnosed, and should be considered mainly in cases of severe arthritis of difficult medical treatment.

  2. [Pain and coping strategies in juvenile idiopathic arthritis].

    Science.gov (United States)

    Herlin, Troels; Thastum, Mikael

    2008-02-18

    Pain is one of the primary symptoms of juvenile idiopathic arthritis (JIA). JIA patients have reduced pain tolerance and pain threshold compared to healthy controls. In children with JIA the greater use of coping strategies such as problem-solving, positive self-statements and distraction consistently have predicted less arthritis-related pain, even after controlling for relevant medical and demographic variables. Interventions specifically designed to modify maladaptive pain coping strategies and pain-related health beliefs may be effective in reducing pain in children with JIA.

  3. MRI and ultrasound in children with juvenile chronic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Lamer, S.; Sebag, G.H

    2000-02-01

    In this era of advancing imaging technology, a knowledge of the relative values of available imaging techniques is necessary to optimize the management of children with juvenile chronic arthritis (JCA). After clinical examination, plain films remain the initial investigation. The need for radiation protection must be a priority in children with JCA. Conventional radiographs allow grouping of the various arthritides (on the base of the distribution and pattern of joint space changes) and staging of disease progression. Ultrasound (US) is very sensitive in the detection of joint effusions, especially in the hip, and guides fluid aspiration. US and Doppler can be used for the evaluation of synovial hypertrophy and activity. Arthrography and to a certain extent nuclear studies have been replaced by magnetic resonance imaging (MRI). MRI can demonstrate articular cartilage, joint effusion, synovial hypertrophy, cortical and medullary bone, cartilage and bone perfusion, and fibrocartilaginous structures (menisci and ligaments). Contrast enhanced MRI is the most sensitive modality to determine whether an arthritic condition is present. However, it does not assist in establishing a specific diagnosis. MRI determines accurately the activity and the extent of the disease and is particularly useful in the early detection of articular damage. Finally, MRI is of major importance in the evaluation of response to local therapy (especially steroids) and the detection of complications.

  4. Anti-chromatin antibodies in juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    V. Gerloni

    2011-09-01

    Full Text Available Objective: to evaluate the prevalence and clinical significance of anti-chromatin antibodies (Abs in juvenile rheumatoid arthritis (JRA. Methods: IgG anti-chromatin Abs were detected by an enzyme-linked immunosorbent assay (ELISA, in sera of 94 children with JRA (10 children with systemic, 38 with polyarticular and 46 with oligoarticular disease onset. As control group, 33 age- and-sex-matched healthy children (HC were also examined. Results: Abs to chromatin were detected in 24/94 (25,5% of children suffering from JRA. Particularly, the higher prevalence of anti-chromatin Abs has been found in children with oligoarticular (30,4% and polyarticular (23,7% onset JRA. In these groups Abs titers were significantly higher compared to systemic JRA and HC (p=0.003. Anti-chromatin Abs were observed more frequently in patients with oligoarticular disease and chronic uveitis (21,7%. Furthermore, higher levels of anti-chromatin Abs has been found in all the patients treated with anti-TNFα therapy (p<0.0001. Conclusions: our results confirm previous data about the prevalence of anti-chromatin Abs in JRA. These Abs were significantly higher in the group of patients with oligoarticular onset with past or present hystory of ocular involvement and in the group with polyarticular JRA treated with biologic therapy. A long-term follow-up study could be useful to evaluate the potential utility of these autoantibodies.

  5. Incidence and prevalence of juvenile idiopathic arthritis in Catalonia (Spain).

    Science.gov (United States)

    Modesto, C; Antón, J; Rodriguez, B; Bou, R; Arnal, C; Ros, J; Tena, X; Rodrigo, C; Rotés, I; Hermosilla, E; Barceló, P

    2010-11-01

    To ascertain the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Catalonia (autonomous region in northeast Spain), examined according to the currently established disease subtypes. Before initiating the study, we conducted an educational programme on paediatric rheumatology, addressed to all general paediatricians in Catalonia. A 2-year (2004-2006), prospective, population-based study was then carried out to determine the incidence of JIA. Prospective and retrospective data retrieval was performed to calculate prevalence. The International League of Associations for Rheumatology (ILAR, Edmonton revision) classification criteria were applied. Over the study period, 145 new cases of JIA were diagnosed. The mean annual incidence was 6.9/10⁵ children aged less than 16 years (range 5.8-8.1 years; 9.0 years for girls and 4.8 years for boys). On separate analysis of patients ≤ 6 and > 6 years, the distribution in younger children was found to be similar for both girls and boys, whereas in older children, most girls belonged to the oligoarthritis and polyarthritis subgroups, and boys to the enthesitis-related arthritis and undifferentiated subgroups. The calculated prevalence of JIA (31 October 2006) was 39.7 (36.1-43.7)/10⁵ children younger than 16. The relative risk of girls having JIA was 2.1 [95% confidence interval (CI) 1.7-2.7, p population-based study on the epidemiology of JIA in Catalonia. Incidence and prevalence rates are lower than those reported for several areas in Nordic countries of Europe. Oligoarthritis was the most common subtype.

  6. Identification of dendritic cells in the blood and synovial fluid of children with Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Tuszkiewicz-Misztal

    2011-04-01

    Full Text Available Childhood chronic arthritis of unknown etiology is known collectively as juvenile idiopathic arthritis (JIA and consists of heterogeneous subtypes with unique clinical patterns of disease. JIA is the commonest rheumatic disease in children and may still result in significant disability, with joint deformity, growth impairment, and persistence of active arthritis into adulthood. Basic research is rather focused on rheumatoid arthritis, and this lead to small number of publications considering JIA. In this study we examine, by flow cytometry, the expression of dendritic cells (DCs in the peripheral blood and synovial fluid of children with active JIA in a group of 220 patients. We reveal a significant decrease in the percentage of immature DCs in the blood of patients compared to control children. Surprisingly, we found higher percentages of mature circulating dendritic cells. Both populations of DCs, immature and mature, were accumulated in patients’ synovial fluid. We also confirmed the presence of CD206+/CD209+ in JIA samples, which can represent a population of macrophages with dendritic cells morphology. Our results support the thesis that dendritic cells are crucial in the induction and maintenance of autoimmune response and local inflammation during juvenile idiopathic arthritis. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 1, pp. 188–199

  7. Bone mineral density in young females with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    V.V. Povoroznyuk

    2017-08-01

    Full Text Available Background. Study of bone mineral density (BMD in young adults with juvenile idiopathic arthritis (JIA is important because long-term administration of glucocorticoids and the presence of chronic systemic inflammation can lead to loss of bone tissue in young people with chronic inflammatory disease in anamnesis. Objective: the study of BMD in young female with juvenile onset of JIA. Materials and methods. Ninety-nine female patients aged 19 to 39 years were divided into two groups: І — 59 healthy young female, ІІ — 40 young female with JIA. The following parameters were evaluated: the age of the disease onset, the duration of delayed diagnosis, disease duration, the ILAR-variant in the disease onset, the BMD in different areas and their T and Z scores. Results. It was found that the onset of JIA was at the age of 11.16  ±  4.34 years, it took 23.52 ± 21.37 months from the beginning of the first clinical manifestations to the time of diagnosis, the disease duration was 11.9 ± 9.4 years, persistant oligoarthritis was detected in 25  % of patients, RF-negative polyarthritis in 22.5  %, extended oligoarthritis in 10  %, RF-positive polyarthritis in 10  %, systemic-onset JIA in 12.5  %, enthesitis-related JIA in 15  %, undifferentiated arthritis in 10  %, psoriatic arthritis in 5  % of patients. BMD (p < 0.000001, T (p = 0.00001 and Z scores in the lumbar spine were lower in female with JIA than in healthy people. Femoral neck BMD (p < 0.000001 and T score (p = 0.00002, total body BMD (p < 0.000001, T- (p = 0.00009 and Z-scores (p < 0.000001 were lower in patients with JIA than in healthy people. However, ultradistal forearm BMD in the female patients differed from healthy ones’ only in T and Z scores (p = 0.004. Z score < –2 SD in young adults was detected in 40  % of patients in the lumbar spine, in 24  % of patients in the femoral neck, in 35.5 % of patients in the total body and in 52.9  % of patients in ultra

  8. Chlorambucil induced chromosome damage in juvenile chronic arthritis.

    Science.gov (United States)

    Palmer, R G; Varonos, S; Doré, C J; Denman, A M; Ansell, B M

    1985-01-01

    Sister chromatid exchanges, a sensitive measure of chromosome damage, were counted in peripheral blood lymphocytes from 23 patients with juvenile chronic arthritis receiving long term, low dose chlorambucil treatment. Thirty five patients with juvenile chronic arthritis who had not been treated with cytotoxic drugs served as controls. All of the treated patients have cells with abnormal sister chromatid exchange frequencies. Damage is related to the daily dose and may, in part, be determined by the duration of treatment. Sister chromatid exchanges from nine patients who had received chlorambucil at some time in the past remained high for at least five months after stopping the drug. Long term follow up will determine whether sister chromatid exchange analysis can help predict those most at risk of drug induced malignancies. Images Fig. 1 PMID:4073932

  9. Canakinumab for the treatment of systemic juvenile idiopathic arthritis.

    Science.gov (United States)

    Grom, Alexei A

    2014-11-01

    The introduction of methotrexate in the 1980s and of TNF-inhibiting agents and abatacept in the late 1990s led to a dramatic improvement in the outcomes of non-systemic categories of juvenile idiopathic arthritis. By contrast, the same treatment approaches had no strong impact on the outcome of systemic juvenile idiopathic arthritis (SJIA), and the main effective treatment in these patients remained glucocorticoids with their known side effects. Encouraging findings in small studies involving SJIA patients treated with IL-1 and IL-6 inhibitors led to large Phase III trials, and the results in these trials provide hope that substantial joint damage and disability seen in the majority of patients with persistent SJIA can be prevented. The purpose of this review is to discuss the safety and efficacy of the IL-1 and IL-6 inhibiting agents in SJIA with the main focus on canakinumab, a fully human monoclonal anti-IL-1β antibody.

  10. Surgical management of the juvenile idiopathic arthritis patient with multiple joint involvement.

    Science.gov (United States)

    Abdel, Matthew P; Figgie, Mark P

    2014-10-01

    Juvenile idiopathic arthritis (JIA) is recognized as a heterogenous group of disorders in which the common factor is persistent arthritis in at least 1 joint occurring before the age of 16 years. Although conservative management with nonsteroidal anti-inflammatory drugs and disease-modifying antirheumatic drugs can be effective, approximately 10% of JIA patients have end-stage degenerative changes requiring total hip arthroplasties (THAs) and total knee arthroplasties (TKAs). This article discusses the overall epidemiology, coordination of care, and medical and surgical management of JIA patients undergoing THA and TKA.

  11. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis

    DEFF Research Database (Denmark)

    Poulsen, Anne Havemose; Westergaard, Jytte; Stoltze, Kaj

    2006-01-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile...

  12. Polyarticular juvenile idiopathic arthritis associated with Fahr′s syndrome

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    U Dundar

    2007-01-01

    Full Text Available Bilateral symmetric calcification involving striatum pallidum with or without deposits in the dentate nucleus, thalamus and white matter is commonly referred to as Fahr′s syndrome. Symptoms of the disorder may include deterioration of motor function, spasticity, spastic paralysis, dysarthria, dementia, seizures, headache and athetosis. The clinical and imaging abnormalities are restricted to the central nervous system (CNS. We report an unusual association of Fahr′s syndrome with polyarticular juvenile idiopathic arthritis in a girl.

  13. Improving adherence to medical regimens for juvenile rheumatoid arthritis

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    Lindsley Carol B

    2007-05-01

    Full Text Available Abstract Poor adherence to medical regimens can compromise the efficacy of treatments for children and adolescents with juvenile rheumatoid arthritis (JRA. The purpose of this review is to describe medical regimens for the treatment of JRA and the rates of adherence to these regimens. We also summarize and critically the few research studies aimed at improving adherence to regimens for JRA. Finally, we summarize strategies for enhancing adherence in clinical practice.

  14. Longitudinal growth attainments of Indian boys with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Aggarwal, Bimal; Bhalla, Anil K; Singh, Surjit

    2011-05-01

    The objective is to study the pattern of distance and velocity growth in terms of weight and height in adolescent boys with Juvenile Rheumatoid Arthritis (JRA). This study was conducted on children diagnosed to have JRA (Cassidy and Petty in Juvenile Rheumatoid Arthritis, WB Saunders Co., Philadelphia, 2005) at the Pediatric Rheumatology and Immunology Clinic of Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh. A total of 203 observations made on 70 boys with JRA, between 9 and 17 years of age, comprised the sample for this prospective mixed-longitudinal growth study. Each subject was measured for body weight and standing height using standardized anthropometric techniques (Eveleth and Tanner in Worldwide variation in human growth, Cambridge University Press, New York, 1990) at half yearly age intervals. All anthropometric measurements were carried out in the Growth Laboratory of Advanced Pediatrics Centre. Boys with polyarticular and systemic onset types of JRA in general measured lighter than their pauciarticular counterparts throughout the period of study. Height attainments in boys with polyarticular and systemic onset JRA measured shorter than their pauciarticular counterparts till 15 years and 12 years, respectively, where-after they became comparable to boys with pauciarticular JRA. As compared to normal Indian (Bhalla and Kumar in Int J Anthropol 18:113-125, 2003; Aggarwal et al. in Indian Pediatr 29:1203-1282, 1992) and American (Ogden et al. in Pediatrics 109:45-60, 2002) counterparts boys representing all categories of JRA remained lighter and shorter. Onset of Peak Height Velocity (PHV) in boys with polyarticular JRA (i.e. 12.5 years) was delayed by 1 year as compared to boys with pauciarticular JRA (i.e. 11.5 years). Attainment of Peak Weight Velocity (PWV) in boys with polyarticular JRA (i.e. 13.5 years) was also delayed by 1 year when compared to those with pauciarticular type (i.e. 12.5 years). In

  15. THE EXPERIENCE OF ADALIMUMAB USE IN A PATIENT WITH PAUTSIARTICULAR JUVENILE ARTHRITIS AND UVEITIS

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    E.I. Alexeeva

    2011-01-01

    Full Text Available The article presents the observation of early debut and the severe course of the juvenile polyarthritis involving the eyes, refractory to treatment by classical immunosuppressants. Successful use of the genetically engineered biological drug — adalimumab is described:  by the 4th week of therapy the acute inflammatory changes in affected joints were stopped, range of motion in them fully recovered; to the 8th week uveitis remission was registered, laboratory values of the disease activity were normalized: erythrocyte sedimentation rate, serum concentration of C-reactive protein. Key words: children, juvenile rheumatoid arthritis, rheumatoid uveitis, adalimumab. (Pediatric pharmacology. — 2011; 8 (6: 119–124.

  16. Estudo comparativo de testes diagnósticos para olho seco entre crianças saudáveis e portadoras de artrite reumatóide juvenil Comparative study of diagnostic tests for dry eye disease between healthy and juvenile rheumatoid arthritis children

    Directory of Open Access Journals (Sweden)

    Jayter Silva de Paula

    2004-10-01

    clinically and underwent tests for keratoconjunctivitis sicca: Schirmer's 1, tear film break-up time and rose bengal staining tests. RESULTS: Six children with juvenile rheumatoid arthritis presented one or more symptoms of keratoconjunctivitis sicca (40% and five of them (83.3% presented meibomitis or other signs of this disease. In group 2, no child presented symptoms or signs of keratoconjunctivitis sicca. Mean Schirmer test did not differ between group 1 and 2 (p=0.156. However, the mean tear film break-up time was significantly reduced in group 1 (p=0.0005 and the mean rose Bengal staining score in group 1 was significantly greater than in group 2 (p=0.0038. Five of the fifteen children of group 1 (33% have two or more abnormal tests and were diagnosed as having definite keratoconjunctivitis sicca, while four children (26% were labeled with probable keratoconjunctivitis sicca. No child of group 2 had more than one positive test. CONCLUSIONS: Signs and symptoms of keratoconjunctivitis sicca appear to be a common ocular finding in juvenile rheumatoid arthritis children. Although only tear film break-up time and rose bengal staining score were significantly different in these groups, there was a trend toward worsening of the other dry eye tests in juvenile rheumatoid arthritis children.

  17. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Barut, Kenan; Sahin, Sezgin; Adrovic, Amra

    2017-01-01

    Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.

  18. Metabolism of glycosaminoglycans in the course of juvenile idiopathic arthritis

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    Katarzyna Winsz-Szczotka

    2016-03-01

    Full Text Available Juvenile idiopathic arthritis (JIA is a non-homogeneous autoimmune children’s disease which, despite the applied therapy, has a progressive character with recurrences, leading to damage of joint structures. Progressive wearing of the joint cartilage in the course of JIA, which results from the imbalance between the biological strength of the cartilage, its function and exerted pressure forces, is linked to metabolic disorders of extracellular matrix (ECM components. Among the latter compounds, the proteoglycan (PG aggrecan plays a particular role in maintaining the mechanical-immunological properties of the cartilage. These functions are directly related to chains of glycosaminoglycans (GAGs, covalently linked to the core protein of PGs. Therefore, every change of GAGs metabolism linked to an increase of the rate of degradation or with a decrease of their biosynthesis may have pathological consequences. In this paper we aim to describe plausible mechanisms leading to observed disorders of aggrecan transformation in children, which are reflected in the profile of plasma GAGs. Therefore, we describe the plausible role of factors related to catabolism and synthesis of PGs/GAGs as well as the contribution of immunological processes to shaping the changes of extracellular matrix components in the course of JIA.

  19. Juvenile Idiopathic Arthritis in the Era of International Cooperation

    Science.gov (United States)

    Uziel, Yosef

    2017-01-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic disease of childhood. Improved understanding of its pathogenesis has led to international cooperation in clinical studies. Multicenter, international collaborations and research facilitate rapid enrollment of enough patients to enable a variety of studies, including those of epidemiology, diagnostic and classification criteria, genetic disease predisposition, pathogenesis, outcomes, and treatment protocols. In the last 20 years, the vision of the Pediatric Rheumatology International Trial Organization (PRINTO) has become a reality of worldwide collaboration in pediatric rheumatology research, including North American and European research groups. Major advances have been made in treating systemic JIA and its main complication, macrophage-activating syndrome (MAS). Single Hub and Access Point to Pediatric Rheumatology in Europe (SHARE) is a project of the European Society of Pediatric Rheumatology with the goal of improving clinical care. Based on evidence in the scientific literature, position papers regarding optimal clinical approaches and care have been published. Formal, validated assessment tools to evaluate response to treatment have been developed. Recommendations have been established to encourage international research collaborations, especially in light of major advances achieved in the genetics of pediatric rheumatologic diseases and the need to share biological samples among different countries and continents. Every participating country has disease information available for patients and families. Additionally, educational programs and updated syllabi for pediatric rheumatology have been written to promote similar, high-level academic training in different countries. These efforts have resulted in significant improvements in treatment and in patient prognosis. However, improved cooperation is needed to enhance research with biological and genetic samples. The Israeli Research Group for

  20. Juvenile Idiopathic Arthritis in the Era of International Cooperation

    Directory of Open Access Journals (Sweden)

    Yosef Uziel

    2017-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA is the most common chronic disease of childhood. Improved understanding of its pathogenesis has led to international cooperation in clinical studies. Multicenter, international collaborations and research facilitate rapid enrollment of enough patients to enable a variety of studies, including those of epidemiology, diagnostic and classification criteria, genetic disease predisposition, pathogenesis, outcomes, and treatment protocols. In the last 20 years, the vision of the Pediatric Rheumatology International Trial Organization (PRINTO has become a reality of worldwide collaboration in pediatric rheumatology research, including North American and European research groups. Major advances have been made in treating systemic JIA and its main complication, macrophage-activating syndrome (MAS. Single Hub and Access Point to Pediatric Rheumatology in Europe (SHARE is a project of the European Society of Pediatric Rheumatology with the goal of improving clinical care. Based on evidence in the scientific literature, position papers regarding optimal clinical approaches and care have been published. Formal, validated assessment tools to evaluate response to treatment have been developed. Recommendations have been established to encourage international research collaborations, especially in light of major advances achieved in the genetics of pediatric rheumatologic diseases and the need to share biological samples among different countries and continents. Every participating country has disease information available for patients and families. Additionally, educational programs and updated syllabi for pediatric rheumatology have been written to promote similar, high-level academic training in different countries. These efforts have resulted in significant improvements in treatment and in patient prognosis. However, improved cooperation is needed to enhance research with biological and genetic samples. The Israeli Research

  1. Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis.

    Science.gov (United States)

    Hahn, Youn-Soo; Kim, Joong-Gon

    2010-11-01

    Juvenile rheumatoid arthritis (JRA) is the most common rheumatic childhood disease; its onset is before 16 years of age and it persists for at least 6 weeks. JRA encompasses a heterogeneous group of diseases that is classified according to 3 major presentations: oligoarthritis, polyarthritis, and systemic onset diseases. These presentations may originate from the same or different causes that involve interaction with specific immunogenetic predispositions, and result in heterogeneous clinical manifestations. An arthritic joint exhibits cardinal signs of joint inflammation, such as swelling, pain, heat, and loss of function; any joint can be arthritic, but large joints are more frequently affected. Extra-articular manifestations include high fever, skin rash, serositis, and uveitis. The first 2 types of JRA are regarded as T helper 1 (Th1) cell-mediated inflammatory disorders, mainly based on the abundance of activated Th1 cells in the inflamed synovium and the pathogenetic role of proinflammatory cytokines that are mainly produced by Th1 cell-stimulated monocytes. In contrast, the pathogenesis of systemic onset disease differs from that of other types of JRA in several respects, including the lack of association with human leukocyte antigen type and the absence of autoantibodies or autoreactive T cells. Although the precise mechanism that leads to JRA remains unclear, proinflammatory cytokines are thought to be responsible for at least part of the clinical symptoms in all JRA types. The effectiveness of biologic therapy in blocking the action of these cytokines in JRA patients provides strong evidence that they play a fundamental role in JRA inflammation.

  2. THERAPEUTIC CAPABILITIES OF ETHANERCEPT IN TREATMENT OF SYSTEMIC JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E.I. Alexeeva

    2011-01-01

    Full Text Available The article presents a clinical case of severe systemic juvenile rheumatoid arthritis, refractory to traditional immunosuppressive treatment and with insufficient efficacy of chimeric homogenous anti-TNF-a antibodies. The patient received 2 courses of anti-Blymphocyte’s- CD20-antibodies that helped to arrest all systemic manifestations of the disease. Articular syndrome was arrested only with the help of soluble receptors to TNF a — ethanercept, given in following dose — 0.4 mg per kg of body mass. The patient has been on treatment with ethanercept for 24 weeks. Joint tenderness and exudation were diminished already after the first 4 weeks of treatment, also there was a dramatic increase in joint motion range. After 6 months of therapy we have managed to improve the patients and his family quality of life.Key words: children, juvenile rheumatoid arthritis, ethanercept, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3: 141–149

  3. Cartilage oligomeric matrix protein in patients with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Bjørnhart, Birgitte; Juul, Anders; Nielsen, Susan

    2009-01-01

    Cartilage oligomeric matrix protein (COMP) has been identified as a prognostic marker of progressive joint destruction in rheumatoid arthritis. In this population based study we evaluated associations between plasma concentrations of COMP, disease activity, and growth velocity in patients...

  4. Radiological improvement by tocilizumab in polyarticular juvenile idiopathic arthritis.

    Science.gov (United States)

    Tozawa, Yusuke; Fujita, Shouji; Abe, Shuji; Kitamura, Koichi; Kobayashi, Ichiro

    2015-04-01

    Recent advances in biologic therapy have enabled reduction of the progression of destructive arthritis in rheumatoid arthritis. Once destroyed, however, the affected bones and cartilage are not fully repaired. We describe the case of an 8-year-old girl with anti-citrullinated peptide antibody (ACPA)-positive polyarticular juvenile idiopathic arthritis (p-JIA). Destructive arthritis progressed during combination therapy with infliximab, methotrexate, mizoribine and prednisolone. Clinical remission was achieved, however, after switching the biologic agent to tocilizumab, a humanized monoclonal antibody to interleukin-6 receptor. Both bone erosion and bone marrow edema on magnetic resonance imaging were repaired in association with restoration of joint spaces. Furthermore, there was no relapse of arthritis on weekly methotrexate alone for 2 years after discontinuation of the tocilizumab. Tocilizumab led to radiological repair of both bone and cartilage destruction and long-term biologics-free remission in a patient with ACPA-positive p-JIA, and should be considered for tumor necrosis factor inhibitor-resistant cases.

  5. Refraction in juvenile chronic arthritis: a long-term follow-up study, with emphasis on myopia

    DEFF Research Database (Denmark)

    Fledelius, H; Zak, M; Pedersen, F K

    2001-01-01

    Assessment of refraction anomalies in juvenile chronic arthritis (JCA) on a long-term follow-up basis.......Assessment of refraction anomalies in juvenile chronic arthritis (JCA) on a long-term follow-up basis....

  6. Bone mineral density in patients with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Sušić Gordana

    2009-01-01

    Full Text Available Introduction. It is well known that juvenile idiopathic arthritis (JIA as a chronic inflammatory disease with onset during the childhood, beside other complication, can lead to bone metabolism disturbance and osteoporosis. Objective. To assess bone mineral density (BMD in children with JIA and to identify factors playing role in bone mineral disturbance. Methods. Seventy-five patients (26 male and 49 female average disease duration 7.2 (2.4-16.8 years, and 73 age matched healthy control subjects (29 male and 44 female participated in the study. Mean age of the groups was about 14.5 years. BMD was determined by dual x-ray absorptiometry (DEXA of the lumbar spine (L2-L4. For further analysis we used the absolute value of BMD, expressed as g/cm2, Z score expressed as SD (relative value as standard deviation decline of normal BMD values of referent Italian population with identical age and gender, bone mineral content (BMC as g/cm, and corrected BMD - BMDv as g/cm3. Results. Z score in the group of patients was significantly lower (-1.02±1.6 in comparison to the control group (-0.09±1.4; p<0.001. BMD, BMDv and BMC were also statistically lower in patients with JIA. The lowest Z score was found in patients with systemic onset (-2.63 SD. Z score showed a statistically significant positive correlation with arthritis course (polyarticular course had lower Z score, body mass index and standard deviation score for height and weight. Statistically significant negative correlation was detected in regard to Z score and glucocorticoid (GC treatment duration, GC cumulative dose, number of joints with limited range of motion, radiological stage and functional class. Conclusion. The results showed a decreased BMD in patients with JIA in comparison to the control group. Systemic onset, polyarthritis, longer treatment with GC and higher cumulative dosage, as well as higher damage level (functional status and radiological stage are factors playing negative role

  7. A case with Trico-rhino-phalangeal syndrome type 1 case with a preliminary diagnosis of Juvenile rheumatoid arthritis

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    Ömer Cevit

    2011-12-01

    Full Text Available Thrico-rhino-falangeal syndrome is a rare disease characterized by a bulbous nose, sparse hairs, a long flat philtrum, and epiphyseal coning. We describe here in a 15 year old girl with Thrico-rhino-falangeal syndrome type 1 (TRPS I. Physical examination demonstrated bulbous nose, hair abnormalities, deformity of the phalanges and winged scapulae. Genetic study and radiologic findings confirmed the diagnosis. Thrico-rhino-falangeal syndrome is characterized by musculoskeletal deformities that at the first view may simulate juvenile idiopathic arthritis. These musculoskeletal deformities could imply the differential diagnosis with juvenile idiopathic arthritis. J Clin Exp Invest 2011; 2 (4: 441-442

  8. Juvenile chronic arthritis and imaging: comparison of different techniques

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    C. Cervini

    2011-09-01

    Full Text Available Objective: The aim of this study was to compare imaging findings obtained with different techniques in a patient with juvenile chronic arthritis. Methods: The patient was a 12 years-old child with a 7-months history of arthritis of the first metatarsophalangeal joint of the right foot. The involved area was explored with the following imaging techniques: X-ray, technetium bone scintigraphy, magnetic resonance, gray-scale and power-Doppler ultrasonography. Results: No abnormalities were detected with conventional X-ray. Scintigraphy showed an abnormal uptake of the radionuclide in the first metatarsophalangeal joint of the right foot. Magnetic resonance without contrast revealed clearly evident features of an active process of synovitis. Ultrasonography was able to detect the presence of joint effusion, synovial proliferation, bone erosion of the first metatarsal head. Power-Doppler examination revealed evident signs of soft tissue hyperemia. Conclusions: Comparative assessment of different imaging techniques in this patient with recent-onset juvenile chronic arthritis indicates that high resolution ultrasonography provides the most detailed evaluation of the joint involvement with respect to the other imaging techniques.

  9. An evaluation of dry eye symptoms and signs in a cohort of children with juvenile idiopathic arthritis

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    Kaisu M Kotaniemi

    2009-03-01

    Full Text Available Kaisu M Kotaniemi1, Pirjo M Salomaa1, Kristiina Sihto-Kauppi1, Hanna M Säilä2, Markku J Kauppi31Department of Ophthalmology;2Pediatric Rheumatology;3Rheumatology, Rheumatism Foundation Hospital, Heinola, FinlandObjective: To determine the prevalence of dry eye symptoms and signs in children with juvenile idiopathic arthritis (JIA.Patients and methods: A total of 192 children with JIA: 48 oligo-, 39 extended oligo-, 79 polyarthritis, and 26 with other types of arthritis (eight juvenile spondyloarthritis, five juvenile psoriatic arthritis, three mixed connective tissue diseases, two systemic onset arthritis, and eight undetermined arthritis were interviewed for dry eye symptoms and tested with Schirmer test with anesthetic. Two thirds of the patients were female and the mean age of the patients was 13.1 years (range 10–16 and the mean duration of arthritis was six years (SD 4, 4. Thirty-one percent of the patients had a history of uveitis. Dry eye was defined as Schirmer test score ≤5 mm in five minutes. The type of arthritis, a history of uveitis, and the ocular and systemic medication used were evaluated for their correlation with dry eye symptoms and signs by using chi-square tests and the Mann–Whitney Monte Carlo analysis.Results: Altogether 17% of this cohort had decreased basal tear secretion. The most common symptoms of dry eye were discharge secretion, itching, and watering. The intensity of symptoms and signs did not correlate. The type of arthritis, a history or presence of uveitis, and the medication used did not correlate with the occurrence of dry eyes.Conclusion: Dry eye symptoms and signs are common in JIA, and Schirmer test with anesthetic is a useful tool in evaluating these patients.Keywords: dry eyes, Schirmer test, juvenile idiopathic arthritis

  10. Tumour necrosis factor (TNF)-blocking agents in juvenile psoriatic arthritis: are they effective?

    NARCIS (Netherlands)

    Otten, M.H.; Prince, F.H.; Cate, R. ten; Rossum, M.A. van; Twilt, M.; Hoppenreijs, E.P.A.H.; Koopman-Keemink, Y.; Oranje, A.P.; Waard-van der Spek, F.B. de; Gorter, S.L.; Armbrust, W.; Dolman, K.M.; Wulffraat, N.M.; Suijlekom-Smit, L.W. van

    2011-01-01

    OBJECTIVES: To evaluate the effectiveness of tumour necrosis factor (TNF) blockers in juvenile psoriatic arthritis (JPsA). METHODS: The study was a prospective ongoing multicentre, observational study of all Dutch juvenile idiopathic arthritis (JIA) patients using biologicals. The response of arthri

  11. Tumour necrosis factor (TNF)-blocking agents in juvenile psoriatic arthritis : are they effective?

    NARCIS (Netherlands)

    Otten, Marieke H; Prince, Femke H M; Ten Cate, Rebecca; van Rossum, Marion A J; Twilt, Marinka; Hoppenreijs, Esther P A H; Koopman-Keemink, Yvonne; Oranje, Arnold P; de Waard-van der Spek, Flora B; Gorter, Simone L; Armbrust, Wineke; Dolman, Koert M; Wulffraat, Nico M; van Suijlekom-Smit, Lisette W A

    2011-01-01

    OBJECTIVES: To evaluate the effectiveness of tumour necrosis factor (TNF) blockers in juvenile psoriatic arthritis (JPsA). METHODS: The study was a prospective ongoing multicentre, observational study of all Dutch juvenile idiopathic arthritis (JIA) patients using biologicals. The response of arthri

  12. Studies on physical performance and functional ability in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.

    2003-01-01

    There is a growing interest in the physical training possibilities of children with juvenile arthritis. In the first Chapter a brief introduction on physical fitness and physical training is given including an overview of the existing studies in juvenile arthritis patients. In Chapter 2, we describe

  13. Proposed outcome measures for prospective clinical trials in juvenile idiopathic arthritis-associated uveitis

    DEFF Research Database (Denmark)

    Heiligenhaus, Arnd; Foeldvari, Ivan; Edelsten, Clive

    2012-01-01

    To develop a set of core outcome measures for use in randomized controlled trials (RCTs) and longitudinal observational studies in juvenile idiopathic arthritis (JIA)-associated uveitis.......To develop a set of core outcome measures for use in randomized controlled trials (RCTs) and longitudinal observational studies in juvenile idiopathic arthritis (JIA)-associated uveitis....

  14. INFLUENCE OF PHYSIOTHERAPY ON CLINICAL AND IMMUNOLOGICAL PARAMETERS IN CHILDREN WITH JUVENILE RHEUMATOID ARTHRITIS

    OpenAIRE

    T.L. Nastausheva; L.T. Dmitrieva

    2008-01-01

    Clinical and immunological status has been evaluated in 85 children with juvenile rheumatoid arthritis (RA) before and after physiotherapeutic procedures: electrophoresis with dimexid and magnetotherapy. The control group of 31 children did not follow physiotherapeutic procedures. The following results were fixed: clinical indices and immunological status of children with juvenile rheumatoid arthritis have been changed in a larger degree in case of magnetotherapy.

  15. Studies on physical performance and functional ability in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.

    2003-01-01

    There is a growing interest in the physical training possibilities of children with juvenile arthritis. In the first Chapter a brief introduction on physical fitness and physical training is given including an overview of the existing studies in juvenile arthritis patients. In Chapter 2, we describe

  16. TNF and LT binding capacities in the plasma of arthritis patients: effect of etanercept treatment in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Gudbrandsdottir, S; Larsen, R; Sørensen, L K;

    2004-01-01

    Etanercept (Enbrel) induces a rapid and sustained decline in disease activity in the majority of patients with refractory juvenile idiopathic arthritis (JIA). For unknown reasons, however, a number of JIA patients fail to respond to this therapy. During this treatment neutralisation of tumour...... necrosis factor (TNF, previously termed TNF alpha) and lymphotoxin (LT, previously termed TNF beta) may be mediated by etanercept itself as well as by naturally occurring soluble TNF receptors. In light of this, it was of interest to study the total TNF neutralizing capacity in plasma before and during...... treatment with etanercept....

  17. A study on the physical fitness of children with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Kwon, Hyo-Jeong; Kim, You Lim; Lee, Hyun Soo; Lee, Suk Min

    2017-03-01

    [Purpose] This study was conducted to assess the physical fitness of children with juvenile rheumatoid arthritis (JRA). [Subjects and Methods] In total, 26 children with juvenile rheumatoid arthritis (JRA) and 25 healthy controls participated in this study. Using the physical fitness measurement instruments, the Inbody 720 and Quark b2, the elements of physical fitness that were assessed included muscular strength, muscular endurance, flexibility, lung capacity, and body composition. [Results] The results revealed significant differences in muscular strength, muscular endurance, lung capacity, body composition, functional ability, and health-related quality of life between the children with juvenile rheumatoid arthritis (JRA) and the control group. [Conclusion] These results suggested that children with juvenile rheumatoid arthritis (JRA) have inferior physical fitness when compared to healthy children. The present study was conducted to develop an accurate evaluation standard to assess the physical fitness of children with juvenile rheumatoid arthritis (JRA).

  18. Adalimumab for juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Magli, Adriano; Forte, Raimondo; Navarro, Pasqualina; Russo, Giustina; Orlando, Francesca; Latanza, Loredana; Alessio, Maria

    2013-06-01

    To assess the long-term outcomes and complications of patients with uveitis from juvenile idiopathic arthritis (JIA) treated with adalimumab. Prospective interventional case series. All patients who underwent treatment with adalimumab for JIA and anterior uveitis were prospectively included in the study. The anterior chamber inflammation was evaluated according to the Standardization of Uveitis Nomenclature criteria. Twenty-one patients (16 females, five males, 38 eyes) were included in the study. Mean age of patients at referral was 11.1 ± 3.8 (5-17) years. Before initiation of treatment, mean duration of arthritis was 7.0 ± 5.5 (median, 6) months, mean duration of uveitis was 7.0 ± 4.4 (median, 7) months. Oligoarticular arthritis was present in 15 cases (71 %), polyarticular arthritis in six cases (28 %). After a mean follow-up of 18.2 ± 7.7 (9-41) months, resolution of anterior chamber inflammation was obtained in 29/38 eyes (76 %). The anterior uveitis flare rate during the 12 months prior to enrollment was 1.6 ± 0.4/year, and was reduced during adalimumab treatment to 0.7 ± 0.3/year (p0.05). Adalimumab showed to be effective and relatively safe for treatment of JIA-associated uveitis.

  19. Long-term follow-up on effectiveness and safety of etanercept in juvenile idiopathic arthritis : the Dutch national register

    NARCIS (Netherlands)

    Prince, F H M; Twilt, M; ten Cate, R; van Rossum, M A J; Armbrust, W; Hoppenreijs, E P A H; van Santen-Hoeufft, M; Koopman-Keemink, Y; Wulffraat, N M; van Suijlekom-Smit, L W A

    2009-01-01

    OBJECTIVE: We undertook an observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different juvenile idiopathic arthritis (JIA) subtypes. METHODS: At baseline we collected patient and disease characteristics of all Dutch patients wi

  20. Long-term follow-up on effectiveness and safety of etanercept in juvenile idiopathic arthritis : the Dutch national register

    NARCIS (Netherlands)

    Prince, F. H. M.; Twilt, M.; ten Cate, R.; van Rossum, M. A. J.; Armbrust, Wineke; Hoppenreijs, E. P. A. H.; van Santen-Hoeufft, M.; Koopman-Keemink, Y.; Wulffraat, N. M.; van Suijlekom-Smit, L. W. A.

    2009-01-01

    Objective: We undertook an observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different juvenile idiopathic arthritis (JIA) subtypes. Methods: At baseline we collected patient and disease characteristics of all Dutch patients wi

  1. Long-term follow-up on effectiveness and safety of etanercept in juvenile idiopathic arthritis: the Dutch national register

    NARCIS (Netherlands)

    Prince, F.H.M.; Twilt, M.; ten Cate, R.; van Rossum, M.A.J.; Armbrust, W.; Hoppenreijs, E.P.A.H.; van Santen-Hoeufft, M.; Koopman-Keemink, Y.; Wulffraat, N.M.; van Suijlekom-Smit, L.W.A.

    2009-01-01

    OBJECTIVE: We undertook an observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different juvenile idiopathic arthritis (JIA) subtypes. METHODS: At baseline we collected patient and disease characteristics of all Dutch patients wi

  2. Combined pre-injection wrist and ankle MRI protocol and steroid joint injections in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Kan, J.H. [Texas Children' s Hospital, Department of Radiology, Houston, TX (United States); Graham, T.B. [Monroe Carell Jr. Children' s Hospital at Vanderbilt, Department of Pediatric Rheumatology, Nashville, TN (United States)

    2011-10-15

    Precise localization of affected compartments of the wrist and ankle in children with an established diagnosis of juvenile idiopathic arthritis (JIA) is clinically challenging. The purpose of this paper is to describe our experience utilizing a pre-injection MRI protocol of the wrist and ankle for localizing disease activity followed by fluoroscopically guided joint injections in children with JIA. (orig.)

  3. A decade of biologic treatment observation in juvenile idiopathic arthritis: Lessons learned from the Dutch ABC Register

    NARCIS (Netherlands)

    M.H. Otten (Marieke)

    2012-01-01

    textabstractSince 1999, the treatment of juvenile idiopathic arthritis (JIA) has been extended with a new category of drugs: biologic agents (also known as biologicals or biologic disease modifying anti-rheumatic drugs). Biologic agents consist of natural proteins, like antibodies and cytokines, and

  4. Intra-Articular Osteoid Osteoma Mimicking Juvenile Arthritis

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    Sidi Yaya Traore

    2014-01-01

    Full Text Available In case of intra-articular osteoid osteoma, misdiagnosis as juvenile arthritis may occur, delaying adequate treatment. We report cases of intra-articular osteoid osteomas in children that were misdiagnosed and initially inappropriately treated with intra-articular corticoid injection. Diagnosis of osteoid osteoma was finally given by CT-scan and appropriate treatment by radiofrequency ablation or surgical ablation was performed. Clinicians and radiologists should be aware of the potentially confusing clinical and imaging findings associated with intra-articular osteoid osteoma.

  5. Sexual maturation in boys with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Aggarwal, Bimal; Bhalla, A K; Singh, Surjit

    2011-11-01

    This paper aimed to study sexual maturation of boys with juvenile rheumatoid arthritis (JRA) during adolescence. This study was carried out in the Department of Pediatrics, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India. A total of 70 boys (between 9 and 17 years of age) diagnosed as cases of JRA comprised the sample for this study. Every child was examined for the development of genitalia as per criteria given by Tanner (Growth at adolescence, 2nd edn, Blackwell, Oxford, 1962) at half-yearly age intervals. However, with regard to development of hair (pubic, axillary and facial) mere presence or absence was noted. Mean (±SD) age of attainment of different stages of genitalia development as well as for eruption of hair was ascertained amongst boys who entered puberty using conventional statistics. Initiation of genitalia development (i.e. appearance of G-2 stage) was earliest among boys with systemic onset JRA (10.8 ± 1.3 years). None of the boys with JRA could attain final stage (G-5) of genitalia development by the age of 17 years, as compared with normal Chandigarh boys who had attained this by 15.2 years. Age of appearance of axillary, pubic, and facial hair was also earlier in systemic onset type of disease as compared with those with pauciarticular and polyarticular JRA. The timing of initiation of sexual maturity in boys with different types of JRA remains variably affected, and appears to experience substantial delay in completion of puberty.

  6. Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Lefèvre-Utile, Alain; Galeotti, Caroline; Koné-Paut, Isabelle

    2014-05-01

    Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  7. [Guidelines for diagnosis and treatment of oligoarticular and polyarticular juvenile idiopathic arthritis].

    Science.gov (United States)

    Bader-Meunier, B; Wouters, C; Job-Deslandre, C; Cimaz, R; Hofer, M; Pillet, P; Quartier, P

    2010-07-01

    A guideline group of pediatric rheumatologist experts elaborated guidelines related to the management of idiopathic juvenile arthritis in association with the Haute Autorité de santé (HAS). A systematic search of the literature published between 1998 and August 2008 and indexed in Pubmed was undertaken. Here, we present the guidelines for diagnosis and treatment in oligoarticular and polyarticular juvenile idiopathic arthritis (except for spondylarthropathy and rheumatoid arthritis).

  8. The Toll-like receptor 4 agonist MRP8/14 protein complex is a sensitive indicator for disease activity and predicts relapses in systemic-onset juvenile idiopathic arthritis.

    NARCIS (Netherlands)

    Holzinger, D.; Frosch, M.; Kastrup, A.; Prince, F.H.; Otten, M.H.; Suijlekom-Smit, L.W. van; Cate, R. ten; Hoppenreijs, E.P.A.H.; Hansmann, S.; Moncrieffe, H.; Ursu, S.; Wedderburn, L.R.; Roth, J.; Foell, D.; Wittkowski, H.

    2012-01-01

    BACKGROUND: Analysis of myeloid-related protein 8 and 14 complex (MRP8/14) serum concentrations is a potential new tool to support the diagnosis of systemic-onset juvenile idiopathic arthritis (SJIA) in the presence of fever of unknown origin. OBJECTIVE: To test the ability of MRP8/14 serum concentr

  9. Gene engineering biological therapy for juvenile arthritis

    Directory of Open Access Journals (Sweden)

    Kh Mikhel's

    2011-01-01

    However, GEBA therapy cannot completely cure the disease as before despite the progress achieved. GEBAs have potentially a number of serious side effects, among which there are severe infections and there is a risk of developing malignancies and autoimmune processes. Their administration requires careful monitoring to reveal the early development of serious adverse reactions, thus preventing a poor outcome.

  10. Juvenile Idiopathic Arthritis in Olmsted County, Minnesota, 1960–2013

    Science.gov (United States)

    Krause, Megan L.; Crowson, Cynthia S.; Michet, C. John; Mason, Thomas; Muskardin, Theresa Wampler; Matteson, Eric L.

    2016-01-01

    Objective To evaluate the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Olmsted County, Minnesota in 1994–2013 and trends in juvenile rheumatoid arthritis (JRA) in 1960–2013. Methods Cases of arthritis in 1994–2013 were identified by diagnosis code with medical chart review to confirm diagnosis separately for JIA and JRA. Overall incidence rates with 95% confidence intervals (95% CIs) were age and sex adjusted to the 2010 US white population. Comparisons were made with an earlier (1960–1993) cohort from this same population. Results Seventy-one incident cases of JIA in 1994–2013 were identified, with an overall age- and sex-adjusted incidence rate of 10.3 per 100,000 (95% CI 7.9–12.7). Forty-two (59%) were female, with an incidence of 12.4 per 100,000 (95% CI 8.6–16.2), as compared to 8.3 per 100,000 (95% CI 5.2–11.3) in males. The most common subtype was oligoarthritis (63%). The mean ± SD age at diagnosis was 8.2 ± 5.3 years. The prevalence of JIA on January 1, 2000 and January 1, 2010 was 51.0 per 100,000 (95% CI 25.2–76.8) and 57.6 per 100,000 (95% CI 31.0–94.5), respectively. When the annual incidence of JRA was compared over time from 1960 to 2013, there was no significant change in incidence overall; however, the incidence decreased among females (P = 0.003). A cyclic pattern of incidence was observed, with peaks approximately every 10 years. Similar to the findings with regard to incidence, prevalence did not change overall, but decreased among females (P = 0.048). There were 4 deaths in the cohort of JRA patients diagnosed in 1960–2013; the standardized mortality ratio was 1.50 (95% CI 0.41–3.83). Conclusion Incidence of juvenile arthritis overall in Olmsted County, Minnesota has not changed significantly in the past 53 years. A consistent cyclic pattern was noted. PMID:26316119

  11. Evolving spectrum of LRBA deficiency-associated chronic arthritis: is there a causative role in juvenile idiopathic arthritis?

    Science.gov (United States)

    Al-Mayouf, Sulaiman M; Naji, Hamzah; Alismail, Khalid; Alazami, Anas M; Sheikh, Farrukh; Conca, Walter; Al-Mousa, Hamoud

    2017-01-01

    Lipopolysaccharide-responsive, beige-like anchor protein (LRBA) deficiency causes common variable immunodeficiency (CVID) disorders and autoimmunity. LRBA deficiency has become a clinically variable syndrome with a wide spectrum of clinical manifestations. We report a patient with LRBA deficiency associated chronic non-erosive arthritis. This report highlights the spectrum of arthritis in such patients and the potential causative role of LRBA gene in juvenile arthritis.

  12. Polyarticular juvenile idiopathic arthritis – epidemiology and management approaches

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    Oberle EJ

    2014-10-01

    Full Text Available Edward J Oberle, Julia G Harris, James W VerbskyDepartment of Pediatrics, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, WI, USAAbstract: Juvenile idiopathic arthritis (JIA is a group of disorders characterized by arthritis persisting for at least 6 weeks with onset before the age of 16 years. Within this cluster of conditions, the polyarticular form (involving more than four joints within the first 6 months is further divided based on the presence of rheumatoid factor. Children with polyarticular JIA pose unique diagnostic and therapeutic challenges compared to children with involvement of fewer joints. Polyarticular JIA patients tend to have a more refractory course and therefore are at increased risk for joint damage, resulting in poorer functional outcomes and decreased quality of life. Although the ability to treat this disorder continues to improve, especially with the advent of biologic agents, there is still much about the epidemiology and pathogenesis of polyarticular JIA that is unknown. The epidemiology of polyarticular JIA varies worldwide with a vast difference in reported cases between different global regions as well as within individual countries. Several genetic risk loci have been identified conferring increased susceptibility to JIA, many within the human leukocyte antigen region. Beyond the genome, environmental factors also seem to contribute to the etiology of polyarticular JIA. This review article will focus on the epidemiology and current treatments of polyarticular JIA and briefly discuss genetic and environmental influences on the pathogenesis of JIA as well as new and emerging therapies.Keywords: juvenile arthritis, polyarticular, epidemiology, treatment, rheumatology

  13. Application of the Yamaguchi criteria for classification of “suspected” systemic juvenile idiopathic arthritis (sJIA

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    Kumar Sharath

    2012-11-01

    Full Text Available Abstract Background Many children with sJIA may have a delayed onset of arthritis and so fail to fulfil the ILAR criteria for sJIA. This study was undertaken to determine whether the Yamaguchi criteria (for adult onset Still’s disease is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA particularly in “pre-arthritic”, pure systemic, phase of the illness. A secondary objective was to determine the time delay between disease onset and onset of arthritis in our sJIA cohort. Methods Retrospective chart review all patients with a diagnosis of systemic juvenile arthritis in our department from Jan 1, 2004 to Jan 1, 2010. Results Twenty boys and eleven girls formed the study cohort. Thirteen patients were diagnosed with “suspected” sJIA due to typical systemic features but an absence of arthritis. Overall, the Yamaguchi criteria was fulfilled in a higher number of patients in the study (n=23 as compared to the ILAR criteria (n=18. Among the 13 “suspected” sJIA patients, 12 fulfilled the Yamaguchi criteria. Overall, either ILAR criteria or Yamaguchi criteria was fulfilled in 30 patients (96.8% of patients. The degree of association between the two criteria was poor (Phi coefficient = -0.352, p=0.05. Eleven out of eighteen patients with arthritis gave a history of delay in onset of arthritis (range=15 days to more than a year; median=30 days. Thus a total of 24 patients (75% had a delay in onset of arthritis at onset of disease. Conclusion Patients with sJIA can have a significant period during their course (particularly at onset when they do not have arthritis. The Yamaguchi criteria may be useful in this subset of patients in the “pre-arthritic” phase of the disease. Future criteria should incorporate the strengths of both, the Yamaguchi and the ILAR criteria.

  14. AUTOINFLAMMATORY DISEASES IN CHILDREN(The Lecture from 18th of September 2013, Conference «Topical Problems of Diagnostics and Treatment of Juvenile Rheumatoid Arthritis» (18–20 of September, 2013, St. Petersburg

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    M. Gattorno

    2014-01-01

    Full Text Available Data about clinical signs, diagnostics and treatment of hereditary autoinflammatory syndromes, e.g. cryopyrin-associated periodic syndrome (CAPS, familial Mediterranean fever (FMF, TNF-receptor associated periodic syndrome (TRAPS-syndrome, hyperimmunoglobulinemia D syndrome (HIDS, Pyogenic Sterile Arthritis, Pyoderma Gangrenosum and Acne (PAPA syndrome, juvenile sarcoidosis (Blau-syndrome are shown in the article. These diseases are typically genetic disease with autosomal dominant and autosomal recessive type of inheritance. All diseases have common pathogenic features, such as spontaneous activation of innate immunity, maintaining of uncontrolled inflammation, absence of auto-antibodies and antigen-specific T-lymphocytes, over-secretion of interleukin-1 and good response to anti-interleukin-1 treatment. In this article you can see the basis of pathogenesis of the diseases, which determine the choice of treatment modalities and diagnostic algorhythms. Differences between clinical phenotypes of cryopyrin-associated periodic syndrome, such as familial cold urticaria (FCAS, Muckle-Wells syndrome and CINCA / NOMID syndrome are described thoroughly. You can find information about the whole group of periodic fevers and their differentiation. Data about international project «EuroFever» which can facilitate international collaboration in the fields of periodic fever are available.

  15. The role of heme oxygenase-1 in systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Takahashi, Akitaka; Mori, Masaaki; Naruto, Takuya; Nakajima, Shoko; Miyamae, Takako; Imagawa, Tomoyuki; Yokota, Shumpei

    2009-01-01

    We have determined the serum levels of heme oxygenase-1 (HO-1) in 56 patients with systemic-onset juvenile idiopathic arthritis (s-JIA) and compared these with serum HO-1 levels in healthy controls and patients with other pediatric rheumatic diseases. Serum HO-1 levels were measured by the sandwich enzyme-linked immunosorbent assay. The mean serum HO-1 level in s-JIA patients during the active phase was 123.6 +/- 13.83 ng/ml, which was significantly higher than that in patients with polyarticular juvenile idiopathic arthritis (p-JIA), Kawasaki disease, systemic lupus erythematosus or mixed connective tissue disease (P < 0.0005). The serum levels of HO-1, cytokines and cytokine receptors in patients with s-JIA were also assessed at both the active and inactive phases. The serum HO-1 level in patients with s-JIA in the active phase was found to be significantly greater than that in patients with the disease in the inactive phase (P < 0.0001). An assessment of the relationships between serum HO-1 levels and other laboratory parameters or cytokines in patients with s-JIA did not reveal any strong correlations. These results suggest that the serum level of HO-1 may be a useful marker for the differential diagnosis of s-JIA. Further study will be necessary to elucidate the mechanism of HO-1 production and to clarify the role of HO-1 in the disease process.

  16. Effectiveness and safety of TNF inhibitors in adults with juvenile idiopathic arthritis

    Science.gov (United States)

    McErlane, Flora; Foster, Helen E; Lunt, Mark; Watson, Kath D; Hyrich, Kimme L

    2016-01-01

    Introduction Many children with juvenile idiopathic arthritis (JIA) continue to have active disease into adulthood. Adults with JIA are a heterogeneous group, and the effects of tumour necrosis factor inhibitor (TNFi) therapies are not well described. This analysis aims to describe treatment outcomes among patients with JIA starting TNFi for the first time in adulthood. Methods Patients with arthritis onset polyarticular JIA were compared with a cohort (weighted for age and gender) of patients with rheumatoid arthritis (RA). Results In 443 adults with JIA starting a first TNFi, disease activity over 1 year improved across all measures. There were 58 first serious infections (IR 22.3/1000 pyrs); 4 cardiovascular events (IR 1.4/1000 pyrs); 11 uveitis events (IR 4.0/1000 pyrs) and 16 malignancies (IR 3.9/1000 pyrs). Compared with the weighted RA cohort, disease activity improvement was similar; malignancy rates were lower and uveitis rates much higher. While crude IR were similar, JIA patients had a lower risk of serious infection (HR 0.5 (95% CI 0.3 to 0.9)). Conclusions This is the largest study to describe disease activity and safety outcomes in adults with JIA receiving TNFi. Disease activity improved after 1 year in all disease patterns, suggesting TNFi is an effective therapy in this population. PMID:27843575

  17. A commentary on TREAT: The trial of early aggressive drug therapy in juvenile idiopathic arthritis

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    Baildam Eileen

    2012-06-01

    Full Text Available Abstract Polyarticular juvenile idiopathic arthritis (JIA is a category of JIA where multiple joints are affected by chronic inflammation, and where serious and lasting damage to joints is the expected natural history in untreated disease. There is evidence of response to disease-modifying antirheumatic and biologic drugs, but little evidence of permanent remission from any of the existing therapeutic trials. The TREAT trial by Wallace et al., recently published in Arthritis and Rheumatism, used a collaborative multicenter approach to studying early aggressive treatment of polyarticular JIA in an attempt to achieve full clinical inactive disease after 6 months of treatment. The study's main finding that the earlier in the disease course that treatment is started, the better the chance of disease control, has provided evidence that there is a 'window of opportunity' for treating JIA as there is in adult rheumatoid arthritis (RA. The study provides both a platform and an impetus for concentrating future treatment trials on early rather than established disease and investigating a standard of starting treatment within 10 to 12 weeks.

  18. Clinical Observation of Employment of Umbilical Cord Derived Mesenchymal Stem Cell for Juvenile Idiopathic Arthritis Therapy

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    Liming Wang

    2016-01-01

    Full Text Available Juvenile idiopathic arthritis (JIA, known as Juvenile rheumatoid arthritis, is the most common type of arthritis in children aged under 17. It may cause sequelae due to lack of effective treatment. The goal of this study is to explore the therapeutic effect of umbilical cord mesenchymal stem cells (UC-MSCs for JIA. Ten JIA patients were treated with UC-MSCs and received second infusion three months later. Some key values such as 28-joint disease activity score (DAS28, TNF-α, IL-6, and regulatory T cells (Tregs were evaluated. Data were collected at 3 months and 6 months after first treatment. DAS28 score of 10 patients was between 2.6 and 3.2 at three months after infusion. WBC, ESR, and CRP were significantly decreased while Tregs were remarkably increased and IL-6 and TNF-α were declined. Similar changes of above values were found after 6 months. At the same time, the amount of NSAIDS and steroid usage in patients was reduced. However, no significant changes were found comparing the data from 3 and 6 months. These results suggest that UC-MSCs can reduce inflammatory cytokines, improve immune network effects, adjust immune tolerance, and effectively alleviate the symptoms and they might provide a safe and novel approach for JIA treatment.

  19. Radiographic abnormalities of the temporomandibular joint in children with juvenile rheumatoid arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Larheim, T.A.; Dale, K.; Tveito, L. (Oslo Sanitetsforening Rheumatism Hospital, University of Oslo, Oslo, Norway)

    1981-01-01

    Radiographic lesion of the temporomandibular joint was found in 41 of 100 children with juvenile rheumatoid arthritis using a combination of radiographic techniques. In patients with abnormality, unilateral lesion occurred in 41 per cent. In patients with definite affection, a varying degree of destruction was observed in 93 per cent, restricted translatory movement of the mandibular head in 83 per cent. In many cases dystrophic or dysplasia-like changes occurred, with stump and thick, anteriorly positioned mandibular head and flat fossa. The lesions seemed to be most frequently associated with the polyarticular type, early onset and long duration of the disease.

  20. PROBLEM OF METABOLIC DISORDERS IN CHILDREN WITH JUVENILE ARTHRITIS LIVING IN THE REPUBLIC OF MORDOVIA

    OpenAIRE

    A. V. Krasnopolskaya; L. A. Balykova; T. I. Kornilova; A. A. Shirokova

    2014-01-01

    It is assumed that juvenile idiopathic arthritis (JIA), as many other rheumatic diseases, is in close pathogenic connection with metabolic disorders and early atherosclerosis. However, the prevalence of metabolic syndrome and its components both in healthy Finno-Ugrian children and teens and JIA patients is unknown.Objective of the present work was to study the prevalence of metabolic disorders in children with JIA, living in the Republic of Mordovia.Subjects and methods. Authors have examine...

  1. Glucocorticoid receptor gene polymorphism and juvenile idiopathic arthritis

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    Scheplyagina Larisa A

    2011-01-01

    Full Text Available Abstract Background The glucocorticoid receptor gene (NR3C1 has been suggested as a candidate gene affecting juvenile idiopathic arthritis (JIA course and prognosis. The purpose of this study is to investigate the glucocorticoid receptor gene BclI polymorphism (rs41423247 in JIA patients, the gene's role in susceptibility to juvenile idiopathic arthritis, and its associations with JIA activity, course and bone mineralization. Methods One hundred twenty-two Caucasian children with JIA and 143 healthy ethnically matched controls were studied. We checked markers of clinical and laboratory activity: morning stiffness, Ritchie Articular Index (RAI, swollen joint count (SJC, tender joint count (TJC, physician's visual analog scale (VAS, hemoglobin level (Hb, leukocyte count (L, platelet count (Pl, Westergren erythrocyte sedimentation rate (ESR, C-reactive protein (CRP, albumin, DAS and DAS28. Bone mineralization was measured by dual-energy X-ray absorptiometry (DXA of lumbar spine L1-L4. Assessments of bone metabolism included osteocalcin, C-terminal telopeptide (CTT, parathyroid hormone (PTH, total and ionized calcium, inorganic phosphate and total alkaline phosphatase (TAP. BclI polymorphism was genotyped by polymerase chain reaction restriction fragment length polymorphism. Results No association was observed between glucocorticoid receptor gene polymorphism and the presence or absence of JIA. In girls with JIA, the presence of the G allele was associated with an unfavorable arthritis course, a younger age of onset of arthritis (p = 0.0017, and higher inflammatory activity. The higher inflammatory activity was demonstrated by the following: increased time of morning stiffness (p = 0.02, VAS (p = 0.014, RAI (p = 0.048, DAS (p = 0.035, DAS28 (p = 0.05, Pl (p = 0.003, L (p = 0.046, CRP (p = 0.01. In addition, these patients had bone metabolism disturbances as follows: decreased BA (p = 0.0001, BMC (p = 0.00007, BMD (0.005 and Z score (p = 0.002; and

  2. Substance use and sexual function in juvenile idiopathic arthritis

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    Marlon van Weelden

    Full Text Available ABSTRACT Objective: To evaluate alcohol/tobacco/illicit drug use and sexual function in adolescent juvenile idiopathic arthritis (JIA and healthy controls. Methods: 174 adolescents with pediatric rheumatic diseases were selected. A cross-sectional study with 54 JIA patients and 35 controls included demographic/anthropometric data and puberty markers assessments, physician-conducted CRAFFT (car/relax/alone/forget/friends/trouble screen tool for substance abuse/dependence high risk and a questionnaire that evaluated sexual function, bullying and alcohol/tobacco/illicit drug use. Clinical/laboratorial data and treatment were also assessed in JIA. Results: The median current age was similar between JIA patients and controls [15(10–19 vs. 15(12–18 years, p = 0.506]. Frequencies of alcohol/tobacco/illicit drug use were high and similar in both JIA and controls (43% vs. 46%, p = 0.829. However, age at alcohol onset was significantly higher in those with JIA [15(11–18 vs. 14(7–18 years, p = 0.032], particularly in polyarticular onset (p = 0.040. High risk for substance abuse/dependence (CRAFFT score ≥ 2 was found in both groups (13% vs. 15%, p = 1.000, likewise bullying (p = 0.088. Further analysis of JIA patients regarding alcohol/tobacco/illicit drug use showed that the median current age [17(14–19 vs. 13(10–19years, p < 0.001] and education years [11(6–13 vs. 7(3–12years, p < 0.001] were significant higher in those that used substances. Sexual activity was significantly higher in the former group (48% vs. 7%, p < 0.001. A positive correlation was evidenced between CRAFFT score and current age in JIA patients (p = 0.032, r = +0.296. Conclusion: A high risk for substance abuse/dependence was observed in both JIA and controls. JIA substance users were more likely to have sexual intercourse. Therefore, routine screening is suggested in all visits of JIA adolescents.

  3. Substance use and sexual function in juvenile idiopathic arthritis.

    Science.gov (United States)

    van Weelden, Marlon; Lourenço, Benito; Viola, Gabriela R; Aikawa, Nadia E; Queiroz, Lígia B; Silva, Clovis A

    2016-01-01

    To evaluate alcohol/tobacco/illicit drug use and sexual function in adolescent juvenile idiopathic arthritis (JIA) and healthy controls. 174 adolescents with pediatric rheumatic diseases were selected. A cross-sectional study with 54 JIA patients and 35 controls included demographic/anthropometric data and puberty markers assessments, physician-conducted CRAFFT (car/relax/alone/forget/friends/trouble) screen tool for substance abuse/dependence high risk and a questionnaire that evaluated sexual function, bullying and alcohol/tobacco/illicit drug use. Clinical/laboratorial data and treatment were also assessed in JIA. The median current age was similar between JIA patients and controls [15(10-19) vs. 15(12-18) years, p=0.506]. Frequencies of alcohol/tobacco/illicit drug use were high and similar in both JIA and controls (43% vs. 46%, p=0.829). However, age at alcohol onset was significantly higher in those with JIA [15(11-18) vs. 14(7-18) years, p=0.032], particularly in polyarticular onset (p=0.040). High risk for substance abuse/dependence (CRAFFT score≥2) was found in both groups (13% vs. 15%, p=1.000), likewise bullying (p=0.088). Further analysis of JIA patients regarding alcohol/tobacco/illicit drug use showed that the median current age [17(14-19) vs. 13(10-19)years, p<0.001] and education years [11(6-13) vs. 7(3-12)years, p<0.001] were significant higher in those that used substances. Sexual activity was significantly higher in the former group (48% vs. 7%, p<0.001). A positive correlation was evidenced between CRAFFT score and current age in JIA patients (p=0.032, r=+0.296). A high risk for substance abuse/dependence was observed in both JIA and controls. JIA substance users were more likely to have sexual intercourse. Therefore, routine screening is suggested in all visits of JIA adolescents. Copyright © 2016 Elsevier Editora Ltda. All rights reserved.

  4. PHARMACOECONOMIC ISSUES OF ADALIMUMAB THERAPY IN JUVENILE IDIOPATHIC ARTHRITIS

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    K. Simpson

    2012-01-01

    Full Text Available Background. Juvenile idiopathic arthritis (JIA is the most common type of arthritis in children and is associated with reduced quality of life and increased health care costs. Objective. To evaluate the cost effectiveness of the tumour necrosis factor inhibitor adalimumab (ADA vs. non-biologic therapy for the treatment of JIA in Russian children and adolescents. Materials and Methods. A Markov model was developed on the basis of the DE038 clinical trial, which compared ADA plus methotrexate (MTX vs. placebo plus MTX for the treatment of JIA in children aged 4–17 years. Cost-effectiveness analyses were performed from the standpoint of the Russian health care system and society as a whole. Base case analyses followed 11-year-old patients with JIA for a period of 7 years (until the age of 18 years or over an expected lifetime. Additional analyses followed patients aged 7 years at treatment initiation for a period of 11 years or over a simulated lifetime. The cost of treating severe JIA was assumed to be the same as reported in a published investigation. The cost of ADA therapy was based on the expected cost assuming inclusion in the List of Vital and Essential Medicinal Products. This took into account the Value Added Tax and a 10% trade mark-up. Treatment outcomes were measured in quality-adjusted life years (QALYs. Results and Discussion. Over a 7-year time horizon, the incremental cost-utility ratio (ICUR for ADA vs. conventional nonbiologic therapy in the treatment of JIA in 11-year-old patients was 1,571,500 roubles/QALY when using a health care system perspective and 1,515,000 roubles/QALY when using a societal perspective. Over a simulated patient lifetime, the corresponding ICURs were 286,300 roubles/QALY and 275,300 roubles/QALY, respectively. Over an 11-year time horizon, the ICUR for ADA vs. conventional non-biologic therapy in the treatment of JIA in patients aged 7 years at the start of therapy was 852,400 roubles/QALY when

  5. Association of neopterin as a marker of immune system activation and juvenile rheumatoid arthritis activity

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    Mones M. Abu Shady

    2015-08-01

    Full Text Available OBJECTIVE: To evaluate neopterin plasma concentrations in patients with active juvenile idiopathic arthritis (JIA and correlate them with disease activity.METHODS: Sixty patients diagnosed as active JIA, as well as another 60 apparently healthy age- and gender-matched children as controls, were recruited from the Pediatrics Allergy and Immunology Clinic, Ain Shams University. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score 27 (JADAS-27. Laboratory investigations were performed for all patients, including determination of hemoglobin concentration (Hgb, erythrocyte sedimentation rate (ESR, and C-reactive protein. Serum concentrations of tumor necrosis factor-alpha (TNF-a, interleukin-6 (IL-6, monocyte chemoattractant protein-1 (MCP-1, and neopterin were measured.RESULTS: Significant differences were found between JIA patients and controls with regard to the mean levels of Hgb, ESR, TNF-a, IL-6, and MCP-1 (p 0.05. Multiple linear regression analysis showed that JADAS- 27 and ESR were the main variables associated with serum neopterin in JIA patients (p < 0.05.CONCLUSION: The elevation of plasma neopterin concentrations in early JIA patients may indicate stimulation of immune response. Serum neopterin can be used as a sensitive marker for assaying background inflammation and disease activity score in JIA patients.

  6. [Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].

    Science.gov (United States)

    Tavares, Anna Carolina Faria Moreira Gomes; Ferreira, Gilda Aparecida; Guimarães, Luciano Junqueira; Guimarães, Raquel Rosa; Santos, Flávia Patrícia Sena Teixeira

    2015-01-01

    Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.

  7. Ureaplasma septic arthritis in an immunosuppressed patient with juvenile idiopathic arthritis.

    Science.gov (United States)

    George, Michael David; Cardenas, Ana Maria; Birnbaum, Belinda K; Gluckman, Stephen J

    2015-06-01

    Mycoplasmas, including Ureaplasma and Mycoplasma species, are uncommon but important causes of septic arthritis, especially affecting immunosuppressed patients. Many of the reported cases have been associated with congenital immunodeficiency disorders, especially hypogammaglobulinemia. Mycoplasmas are difficult to grow in the laboratory, and these infections may be underdiagnosed using culture techniques. We report a case of a 21-year-old woman with juvenile idiopathic arthritis and hip arthroplasties treated with rituximab and adalimumab who developed urogenital infections and soft tissue abscesses followed by knee arthritis with negative routine cultures. Ureaplasma species was identified from synovial fluid on 2 separate occasions using a broad-range 16S ribosomal RNA gene polymerase chain reaction. Azithromycin led to rapid improvement in symptoms, but after completion of therapy, involvement of the hip prosthesis became apparent, and again, 16S rRNA gene polymerase chain reaction was positive for Ureaplasma species. The literature is reviewed with a discussion of risk factors for Mycoplasma septic arthritis, clinical presentation, methods of diagnosis, and treatment.

  8. The importance of an ophthalmologic examination in patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Rodríguez-García, Alejandro

    2015-01-01

    Uveitis occurs within the first year of arthritis onset in 73% of patients with juvenile idiopathic arthritis (JIA) considered at risk. The intraocular inflammation is characterized by an insidious onset and a silent and chronic clinical course capable of producing significant visual loss due to complications such as: cataract formation, secondary glaucoma, maculopathy and optic neuropathy. The absence of initial signs and symptoms, along with a deficient ophthalmic monitoring produce a delay in diagnosis with serious consequences. It has been estimated that 47% of JIA patients at risk for developing uveitis are legally blind (20/200 or worse) at least in one eye at the time of their first visit to the ophthalmologist. To reduce ocular complications and improve their visual outcome, it is necessary that rheumatologists refer all patients recently diagnosed (within the first month) with JIA for an ophthalmic evaluation, and maintain periodical follow-up visits based on classification and risk category of the disease.

  9. Novel strategies for immune therapy of arthritis - Towards sustained disease remission.

    NARCIS (Netherlands)

    Roord, S.T.A.

    2009-01-01

    The current treatment of Juvenile Idiopathic Arthritis and Rheumatoid Arthritis consists of a generalized suppression of the immune system. Despite improvement in disease outcome, there are some major disadvantages. The drugs need to be administered continuously in order to remain effective, do not

  10. Uveitis in juvenile chronic arthritis: incidence, clinical features and prognosis.

    Science.gov (United States)

    Kanski, J J

    1988-01-01

    Three hundred and fifteen patients with anterior uveitis and juvenile chronic arthritis were reviewed in order to determine the incidence, visual prognosis, and the clinical characteristics of the intraocular inflammation. The overall incidence of uveitis was 20%. Approximately 25% of patients had relatively mild and/or transient involvement and an excellent visual prognosis. In 50% the uveitis was more severe but could be controlled with topical medication. In the remaining 25% the visual prognosis was poor due to the intractable nature of the uveitis and the subsequent development of vision-threatening complications. The majority of patients (74%) were under the age of 8 years when the uveitis was first diagnosed. Clinically, the intraocular inflammation was most frequently an asymptomatic, chronic, non-granulomatous, iridocyclitis which was bilateral in 71% of cases. Other ocular lesions, which were rare, included keratoconjunctivitis sicca and corneal melting.

  11. Impact of juvenile arthritis on families. An educational assessment.

    Science.gov (United States)

    Konkol, L; Lineberry, J; Gottlieb, J; Shelby, P E; Miller, J J; Lorig, K

    1989-06-01

    State of the art patient education programs have as their goals changes in behaviors, coping styles, health status, and/or costs. The accomplishment of these goals often involves not only the patient but also his or her whole family. This is especially true if the patient is a child. Based on this premise, we undertook an educational needs assessment of 50 children with juvenile arthritis (JA) and their families. Through utilization of a grounded theory methodology, open-ended questionnaires were completed by JA children, their parents, and their siblings. The resulting analysis suggests (1) the need for family-based education, (2) differing needs of various family members, and (3) several hypotheses for further study.

  12. [Juvenile monomelic amyotrophy: Hirayama disease].

    Science.gov (United States)

    Drozdowski, W; Baniukiewicz, E; Lewonowska, M

    1998-01-01

    We present three patients with unilateral upper limb weakness (with muscular atrophy)-two of them with distal and one with proximal localization. The disease onset was between 18th end 35-th year of life; the disease course was biphasic (i.e. progressive within first 1 to 3 years, and stabilized during following 4-24 years). The laboratory investigations permitted to diagnose juvenile monomelic amyotrophy, an entity that is very rare outside Japan. Electromyography revealed neurogenic involvement with spinal features also in clinically unaffected muscles. We suggest that these results may support the hypothesis of this disease being a benign variant of spinal muscular atrophy.

  13. Effects of sulfasalazine treatment on serum immunoglobulin levels in children with juvenile chronic arthritis

    NARCIS (Netherlands)

    van Rossum, MAJ; Fiselier, TJW; Franssen, MJAN; ten Cate, R; van Suijlekom-Smit, LWA; Wulffraat, NM; van Luijk, WHJ; Oostveen, JCM; Kuis, W; Dijkmans, BAC; van Soesbergen, RM

    2001-01-01

    This article describes the effects of sulfasalazine (SSZ) treatment on serum immunoglobulin (Ig) levels in 6 children with oligoarticular- or polyarticular onset juvenile chronic arthritis (JCA). None of the children who developed dysimmunoglobulinemia during treatment showed clinical symptoms of th

  14. Polyarticular juvenile idiopathic arthritis - epidemiology and management approaches.

    Science.gov (United States)

    Oberle, Edward J; Harris, Julia G; Verbsky, James W

    2014-01-01

    Juvenile idiopathic arthritis (JIA) is a group of disorders characterized by arthritis persisting for at least 6 weeks with onset before the age of 16 years. Within this cluster of conditions, the polyarticular form (involving more than four joints within the first 6 months) is further divided based on the presence of rheumatoid factor. Children with polyarticular JIA pose unique diagnostic and therapeutic challenges compared to children with involvement of fewer joints. Polyarticular JIA patients tend to have a more refractory course and therefore are at increased risk for joint damage, resulting in poorer functional outcomes and decreased quality of life. Although the ability to treat this disorder continues to improve, especially with the advent of biologic agents, there is still much about the epidemiology and pathogenesis of polyarticular JIA that is unknown. The epidemiology of polyarticular JIA varies worldwide with a vast difference in reported cases between different global regions as well as within individual countries. Several genetic risk loci have been identified conferring increased susceptibility to JIA, many within the human leukocyte antigen region. Beyond the genome, environmental factors also seem to contribute to the etiology of polyarticular JIA. This review article will focus on the epidemiology and current treatments of polyarticular JIA and briefly discuss genetic and environmental influences on the pathogenesis of JIA as well as new and emerging therapies.

  15. QUALITY OF LIFE IN CHILDREN WITH JUVENILE RHEUMATOID ARTHRITIS TREATED WITH INFLIXIMAB

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    R.V. Denisova

    2008-01-01

    Full Text Available Juvenile rheumatoid arthritis (JRA is chronic disease, leading to early incapacitating injury in patients. Treatment of JRA with new expensive biological agents allows obtaining long term remission of disease and improving its prognosis. Estimation of quality of life is one of the main effectiveness criteria of treatment. A quality of life in children who were 2–4 years old treated with infliximab was estimated. 43 patients with different types of JRA were examined. A quality of life was estimated with the help of questionnaire PEDSQL generic core scale, PEDSQL rheumatology module. Index of functional disability was estimated by childhood health assessment questionnaire (CHAQ. Significant increase of quality of life rates and decrease of index of functional disability was registered in 6 weeks of therapy with infliximab. The rates of quality of life in patients with JRA treated with infliximab were significantly equal to that in healthy children in the same age in 6, 12 and 24 months of treatment. Thus, treatment with infliximab significantly increases quality of life in children in 2–4 years old with JRA and their families, decreases negative influence of disease on child's living, improves physical activity and emotional state of patients, and allows improving contact between patients and healthy children in the same age.Key words: children, juvenile rheumatoid arthritis, quality of life, infliximab.

  16. Contrast-enhanced MRI features in the early diagnosis of Juvenile Idiopathic Arthritis

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    Hemke, Robert; Maas, Mario [University of Amsterdam, Department of Radiology Academic Medical Center, Amsterdam (Netherlands); Kuijpers, Taco W.; Schonenberg-Meinema, Dieneke [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Nusman, Charlotte M. [University of Amsterdam, Department of Radiology Academic Medical Center, Amsterdam (Netherlands); University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Rossum, Marion A.J. van; Berg, J.M. van den [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Department of Pediatric Rheumatology, Reade, Amsterdam (Netherlands); Dolman, Koert M. [Department of Pediatric Rheumatology, Reade, Amsterdam (Netherlands); St. Lucas Andreas Hospital, Department of Pediatrics, Amsterdam (Netherlands)

    2015-11-15

    To determine whether clinical, laboratory or Magnetic Resonance Imaging (MRI) measures differentiate Juvenile Idiopathic Arthritis (JIA) from other forms of active childhood arthritis. We prospectively collected data of 80 treatment-naive patients clinically suspected of JIA with active non-infectious arthritis of (at least) one knee for <12 months duration. Upon presentation patients underwent clinical and laboratory assessments and contrast-enhanced MRI. MRI was not used as a diagnostic criterion. Forty-four (55 %) patients were clinically diagnosed with JIA, whereas in 36 (45 %) patients the diagnosis of JIA was discarded on clinical or laboratory findings. MRI-based synovitis was present in 27 (61.4 %) JIA patients and in 7 (19.4 %) non-JIA patients (P < 0.001). Five factors (male gender, physician's global assessment of overall disease activity, joints with limited range of motion, HLA-B27, MRI-based synovitis) were associated with the onset of JIA. In multivariate analysis MRI-based synovitis proved to be independently associated with JIA (OR 6.58, 95 % CI 2.36-18.33). In patients with MRI-based synovitis, the RR of having JIA was 3.16 (95 % CI 1.6-6.4). The presence of MRI-based synovitis is associated with the clinical onset of JIA. Physical examination could be supported by MRI, particularly to contribute in the early differentiation of different forms of non-infectious childhood arthritis. (orig.)

  17. INFLUENCE OF PHYSIOTHERAPY ON CLINICAL AND IMMUNOLOGICAL PARAMETERS IN CHILDREN WITH JUVENILE RHEUMATOID ARTHRITIS

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    T.L. Nastausheva

    2008-12-01

    Full Text Available Clinical and immunological status has been evaluated in 85 children with juvenile rheumatoid arthritis (RA before and after physiotherapeutic procedures: electrophoresis with dimexid and magnetotherapy. The control group of 31 children did not follow physiotherapeutic procedures. The following results were fixed: clinical indices and immunological status of children with juvenile rheumatoid arthritis have been changed in a larger degree in case of magnetotherapy.

  18. Medication use in juvenile uveitis patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry.

    Science.gov (United States)

    Henderson, Lauren A; Zurakowski, David; Angeles-Han, Sheila T; Lasky, Andrew; Rabinovich, C Egla; Lo, Mindy S

    2016-02-16

    There is not yet a commonly accepted, standardized approach in the treatment of juvenile idiopathic uveitis when initial steroid therapy is insufficient. We sought to assess current practice patterns within a large cohort of children with juvenile uveitis. This is a cross-sectional cohort study of patients with uveitis enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRAnet) registry. Clinical information including, demographic information, presenting features, disease complications, and medications were collected. Chi-square and Fisher's exact tests were used to assess for associations between medications and clinical characteristics. Ninety-two children with idiopathic and 656 with juvenile idiopathic arthritis (JIA)-associated uveitis were identified. Indication (arthritis or uveitis) for medication use was not available for JIA patients; therefore, detailed analysis was limited to children with idiopathic uveitis. In this group, 94 % had received systemic steroids. Methotrexate (MTX) was used in 76 % of patients, with oral and subcutaneous forms given at similar rates. In multivariable analysis, non-Caucasians were more likely to be treated initially with subcutaneous MTX (P = 0.003). Of the 53 % of patients treated with a biologic DMARD, all received a tumor necrosis factor (TNF) inhibitor. TNF inhibitor use was associated with a higher frequency of cataracts (52 % vs 21 %; P = 0.001) and antinuclear antibody positivity (49 % vs 29 %; P = 0.04), although overall complication rates were not higher in these patients. Among idiopathic uveitis patients enrolled in the CARRAnet registry, MTX was the most commonly used DMARD, with subcutaneous and oral forms equally favored. Patients who received a TNF inhibitor were more likely to be ANA positive and have cataracts.

  19. Genetics Home Reference: juvenile Paget disease

    Science.gov (United States)

    ... Information & Resources MedlinePlus (1 link) Health Topic: Bone Diseases Genetic and Rare Diseases Information Center (1 link) Juvenile ... on PubMed Daroszewska A, Ralston SH. Mechanisms of disease: genetics of Paget's disease of bone and related disorders. ...

  20. Bruton’s Disease Presenting With Arthritis; A Case Report

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    Ramezanali Yakhchali

    2015-06-01

    Full Text Available Introduction X-linked Agammaglobulinemia (XLA is one of the primary humoral immunodeficiencies. It usually presents symptoms of recurrent infections, but in some unusual cases it may present rheumatologic manifestations. Case Presentation The current paper presents the cases of two boys with arthritis treated for juvenile rheumatoid arthritis (JRA without proper responses. Addition of some recurrent infections in the course of their disease led to work-up them for immunodeficiencies. Conclusions According to the results of these work-ups, XLA was diagnosed for the cases.

  1. Correlation analyses of clinical and molecular findings identify candidate biological pathways in systemic juvenile idiopathic arthritis

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    Ling Xuefeng B

    2012-10-01

    Full Text Available Abstract Background Clinicians have long appreciated the distinct phenotype of systemic juvenile idiopathic arthritis (SJIA compared to polyarticular juvenile idiopathic arthritis (POLY. We hypothesized that gene expression profiles of peripheral blood mononuclear cells (PBMC from children with each disease would reveal distinct biological pathways when analyzed for significant associations with elevations in two markers of JIA activity, erythrocyte sedimentation rate (ESR and number of affected joints (joint count, JC. Methods PBMC RNA from SJIA and POLY patients was profiled by kinetic PCR to analyze expression of 181 genes, selected for relevance to immune response pathways. Pearson correlation and Student's t-test analyses were performed to identify transcripts significantly associated with clinical parameters (ESR and JC in SJIA or POLY samples. These transcripts were used to find related biological pathways. Results Combining Pearson and t-test analyses, we found 91 ESR-related and 92 JC-related genes in SJIA. For POLY, 20 ESR-related and 0 JC-related genes were found. Using Ingenuity Systems Pathways Analysis, we identified SJIA ESR-related and JC-related pathways. The two sets of pathways are strongly correlated. In contrast, there is a weaker correlation between SJIA and POLY ESR-related pathways. Notably, distinct biological processes were found to correlate with JC in samples from the earlier systemic plus arthritic phase (SAF of SJIA compared to samples from the later arthritis-predominant phase (AF. Within the SJIA SAF group, IL-10 expression was related to JC, whereas lack of IL-4 appeared to characterize the chronic arthritis (AF subgroup. Conclusions The strong correlation between pathways implicated in elevations of both ESR and JC in SJIA argues that the systemic and arthritic components of the disease are related mechanistically. Inflammatory pathways in SJIA are distinct from those in POLY course JIA, consistent with

  2. Pilot study comparing the Childhood Arthritis & Rheumatology Research Alliance (CARRA) systemic Juvenile Idiopathic Arthritis Consensus Treatment Plans

    OpenAIRE

    Kimura, Yukiko; Grevich, Sriharsha; Beukelman, Timothy; Morgan, Esi; Peter A. Nigrovic; Mieszkalski, Kelly; Graham, T. Brent; Ibarra, Maria; Ilowite, Norman; Klein-Gitelman, Marisa; Onel, Karen; Prahalad, Sampath; Punaro, Marilynn; Ringold, Sarah; Toib, Dana

    2017-01-01

    Objectives To assess the feasibility of studying the comparative effectiveness of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans (CTPs) for systemic Juvenile Idiopathic Arthritis (JIA) using an observational registry. Methods Untreated systemic JIA patients enrolled in the CARRA Registry were begun on one of 4 CTPs chosen by the treating physician and patient/family (glucocorticoid [GC] alone; methotrexate [MTX]???GC; IL1 inhibitor [IL1i]???GC; IL...

  3. TNF-α Polymorphisms in Juvenile Idiopathic Arthritis: Which Potential Clinical Implications?

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    A. Scardapane

    2012-01-01

    Full Text Available Whether tumor necrosis factor alpha (TNF-α gene polymorphisms (SNPs influence disease susceptibility and treatment of patients with juvenile idiopathic arthritis (JIA is presently uncertain. TNF-α is one of the most important cytokine involved in JIA pathogenesis. Several single nucleotide polymorphisms (SNPs have been identified within the region of the TNF-α gene but only a very small minority have proven functional consequences and have been associated with susceptibility to JIA. An association between some TNF-α SNPs and adult rheumatoid arthritis (RA susceptibility, severity and clinical response to anti-TNF-α treatment has been reported. The most frenquetly studied TNF-α SNP is located at −308 position, where a substitution of the G allele with the rare A allele has been found. The presence of the allele −308A is associated to JIA and to a poor prognosis. Besides, the −308G genotype has been associated with a better response to anti-TNF-α therapy in JIA patients, confirming adult data. Psoriatic and oligoarticular arthritis are significantly associated to the −238 SNP only in some works. Studies considering other SNPs are conflicting and inconclusive. Large scale studies are required to define the contribution of TNF-α gene products to disease pathogenesis and anti-TNF-α therapeutic efficacy in JIA.

  4. O adulto com artrite idiopática juvenil poliarticular The adult patient with polyarticular juvenile idiopathic arthritis

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    Liz Wallin

    2009-08-01

    Full Text Available Crianças com a forma poliarticular da artrite idiopática juvenil (AIJ, ao entrarem na idade adulta, têm um quadro similar ao de pacientes com artrite reumatoide (AR de início no adulto. No presente estudo comparam-se características clínicas e imunológicas desses dois grupos de pacientes. Para isso, foram estudados vinte adultos com AIJ poliarticular e cinquenta pacientes com AR (pareados para sexo e tempo de duração de doença, para presença de autoanticorpos, nódulos subcutâneos, síndrome de Sjögren secundária, hipotireoidismo e para a determinação de índices funcionais e antropométricos. Encontraram-se, nesses dois grupos, características similares, exceto pela presença de fator reumatoide (menor no grupo de AIJ poliarticular; P = 0,026 e menor IMC nos pacientes com AIJ poliarticular (P When children with polyarticular juvenile idiopathic arthritis (JIA reach adulthood, they have a condition similar to that of patients with adult onset rheumatoid arthritis (RA. In the present study, the clinical and immunological characteristics of these two groups of patients are compared. The presence of autoantibodies, subcutaneous nodules, secondary Sjögren syndrome, and hypothyroidism, was determined in 20 adult patients with polyarticular JIA and in 50 patients with RA (paired for gender and duration of the disease, as well as the determination of functional and anthropometric indexes. Both groups had similar characteristics, except for the presence of rheumatoid factor (lower in the polyarticular JIA group; P = 0.026 and lower BMI in patients with polyarticular JIA (P < 0.001.

  5. [Biologics for treatment of juvenile idiopathic arthritis. Consensus statement of the 7th Wörlitzer Expertengespräche 2004 for the German Arbeitsgemeinschaft Kinder- und Jugendrheumatologie].

    Science.gov (United States)

    Horneff, G

    2006-03-01

    The group of biologics for the treatment of rheumatic diseases is continuously growing. They have become an important option not only for treatment of so far untreatable chronic inflammatory or rheumatic disease, but also for juvenile idiopathic arthritis. In addition, the velocity and the degree of improvement is better than with to conventional therapies. Furthermore, toxicity and risks seem to be lower with higher safety and compatibility. Although the data are scarce, they are widely used. Therefore, the German Arbeitsgemeinschaft Kinder- und Jugendrheumatologie is updating the current recommendation for the treatment of juvenile idiopathic arthritis using biologics.

  6. Juvenile idiopathic arthritis-associated uveitis: a nationwide population-based study in Taiwan.

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    Hsin-Hui Yu

    Full Text Available OBJECTIVE: The incidence and prevalence of juvenile idiopathic arthritis (JIA vary widely across the world but data in East Asia is lacking. Uveitis is a serious cause of morbidity in JIA. This study aimed to analyze the incidence and prevalence of JIA, and the characteristics of JIA-associated uveitis in Taiwan. METHODS: A population-based cohort study was conducted using the Taiwan National Health Insurance Research Database. Each patient was individually tracked from 1999 to 2009 to identify the diagnosis of JIA and uveitis using the International Classification of Diseases diagnostic codes. Multivariate logistic regression was used to determine the risk factors and complications of uveitis in patients with JIA. RESULTS: The study cohort had 2636 cases of JIA and included juvenile rheumatoid arthritis (57.7%, enthesitis-related arthritis (ERA (39.2%, and psoriatic arthritis (3.1%. The average annual incidence of JIA and JIA-associated uveitis were 4.93 (range, 3.93-6.23 and 0.25 (range, 0.12-0.37 cases per 100,000 population, respectively. The average period prevalence of JIA was 33.8 cases per 100,000 population. Uveitis occurred in 4.7% of patients with JIA, while JIA-associated uveitis was complicated by cataract (11.2% and glaucoma (24.8%. Enthesitis-related arthritis was significantly associated with uveitis (OR: 3.47; 95% CI: 2.24-5.37 (p<0.0001. Uveitis diagnosed before JIA was the most significant risk factor for complications of glaucoma or cataract (OR: 3.54; 95% CI: 1.44-8.72 (p = 0.006. CONCLUSIONS: The incidence of JIA is low but that of JIA-associated uveitis is increasing. Higher percentage of males in patients with ERA and the strong association between ERA and uveitis are unique for children with JIA in Taiwan. Uveitis diagnosed before arthritis is an important risk factor for complications. Continuous ophthalmologic follow-up is needed for children with JIA or uveitis of unknown etiology.

  7. Juvenile Idiopathic Arthritis-Associated Uveitis: A Nationwide Population-Based Study in Taiwan

    Science.gov (United States)

    Yu, Hsin-Hui; Chen, Pau-Chung; Wang, Li-Chieh; Lee, Jyh-Hong; Lin, Yu-Tsan; Yang, Yao-Hsu; Lin, Chang-Ping; Chiang, Bor-Luen

    2013-01-01

    Objective The incidence and prevalence of juvenile idiopathic arthritis (JIA) vary widely across the world but data in East Asia is lacking. Uveitis is a serious cause of morbidity in JIA. This study aimed to analyze the incidence and prevalence of JIA, and the characteristics of JIA-associated uveitis in Taiwan. Methods A population-based cohort study was conducted using the Taiwan National Health Insurance Research Database. Each patient was individually tracked from 1999 to 2009 to identify the diagnosis of JIA and uveitis using the International Classification of Diseases diagnostic codes. Multivariate logistic regression was used to determine the risk factors and complications of uveitis in patients with JIA. Results The study cohort had 2636 cases of JIA and included juvenile rheumatoid arthritis (57.7%), enthesitis-related arthritis (ERA) (39.2%), and psoriatic arthritis (3.1%). The average annual incidence of JIA and JIA-associated uveitis were 4.93 (range, 3.93–6.23) and 0.25 (range, 0.12–0.37) cases per 100,000 population, respectively. The average period prevalence of JIA was 33.8 cases per 100,000 population. Uveitis occurred in 4.7% of patients with JIA, while JIA-associated uveitis was complicated by cataract (11.2%) and glaucoma (24.8%). Enthesitis-related arthritis was significantly associated with uveitis (OR: 3.47; 95% CI: 2.24–5.37) (pUveitis diagnosed before JIA was the most significant risk factor for complications of glaucoma or cataract (OR: 3.54; 95% CI: 1.44–8.72) (p = 0.006). Conclusions The incidence of JIA is low but that of JIA-associated uveitis is increasing. Higher percentage of males in patients with ERA and the strong association between ERA and uveitis are unique for children with JIA in Taiwan. Uveitis diagnosed before arthritis is an important risk factor for complications. Continuous ophthalmologic follow-up is needed for children with JIA or uveitis of unknown etiology. PMID:23940609

  8. Fever as an initial manifestation of enthesitis-related arthritis subtype of juvenile idiopathic arthritis: retrospective study.

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    Ruru Guo

    Full Text Available We wished to determine the prevalence of fever as one of the first symptoms of the enthesitis-related arthritis (ERA subtype of juvenile idiopathic arthritis. Also, we wished to ascertain if ERA patients with fever at disease onset differed from those without fever.Consecutive cases of ERA were diagnosed and followed in a retrospective observational study from 1998 to 2013. Information about clinical/laboratory data, medications, magnetic resonance imaging (MRI, and disease activity during the study period was also recorded.A total of 146 consecutive ERA patients were assessed. Among them, 52 patients (35.6% had fever as one of the first symptoms at disease onset. Compared with ERA patients without fever at disease onset, patients with fever had significantly more painful joints (3.5 vs. 2.8, more swollen joints (1.1 vs. 0.8, and more enthesitis (1.0 vs. 0.4 (p<0.05 for all comparisons. Patients with fever had significantly higher mean values of erythrocyte sedimentation rate, C-reactive protein, platelet count, and child health assessment questionnaire (CHAQ scores (40.8 vs. 26.4 mm/h; 20.7 vs. 9.7 mg/dL; 353.2×109/L vs. 275.6×109/L; 1.0 vs. 0.8, respectively; all p<0.05. During two-year follow-up, CHAQ score, number of flares, as well as the number of patients treated with oral non-steroidal anti-inflammatory drugs, corticosteroids and combination therapy with disease-modifying anti-rheumatic drugs, were significantly higher in ERA patients with fever.Fever was a frequent manifestation of ERA. ERA patients with fever had more active disease at disease onset and poorer outcomes than ERA patients without fever.

  9. Immunogenetics of juvenile idiopathic arthritis: A comprehensive review.

    Science.gov (United States)

    Hersh, Aimee O; Prahalad, Sampath

    2015-11-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthropathy of childhood. Juvenile idiopathic arthritis is believed to be a complex genetic trait influenced by both genetic and environmental factors. Twin and family studies suggest a substantial role for genetic factors in the predisposition to JIA. Describing the genetics is complicated by the heterogeneity of JIA; the International League of Associations for Rheumatology (ILAR) has defined seven categories of JIA based on distinct clinical and laboratory features. Utilizing a variety of techniques including candidate gene studies, the use of genotyping arrays such as Immunochip, and genome wide association studies (GWAS), both human leukocyte antigen (HLA) and non-HLA susceptibility loci associated with JIA have been described. Several of these polymorphisms (e.g. HLA class II, PTPN22, STAT4) are shared with other common autoimmune conditions; other novel polymorphisms that have been identified may be unique to JIA. Associations with oligoarticular and RF-negative polyarticular JIA are the best characterized. A strong association between HLA DRB1:11:03/04 and DRB1:08:01, and a protective effect of DRB1:15:01 have been described. HLA DPB1:02:01 has also been associated with oligoarticular and RF-negative polyarticular JIA. Besides PTPN22, STAT4 and PTPN2 variants, IL2, IL2RA, IL2RB, as well as IL6 and IL6R loci also harbor variants associated with oligoarticular and RF-negative polyarticular JIA. RF-positive polyarticular JIA is associated with many of the shared epitope encoding HLA DRB1 alleles, as well as PTPN22, STAT4 and TNFAIP3 variants. ERA is associated with HLA B27. Most other associations between JIA categories and HLA or non-HLA variants need confirmation. The formation of International Consortia to ascertain and analyze large cohorts of JIA categories, validation of reported findings in independent cohorts, and functional studies will enhance our understanding of the genetic

  10. Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome - effective treatment with tocilizumab.

    Science.gov (United States)

    Kwiatkowska, Małgorzata; Jednacz, Ewa; Rutkowska-Sak, Lidia

    2015-01-01

    A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5(th) year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  11. An Unexpected Cause of Knee Pain in a Patient with Juvenile Idiopathic Arthritis: Osteoid Osteoma

    Directory of Open Access Journals (Sweden)

    Mehmet Eroğlu

    2014-06-01

    Full Text Available Patients with chronic diseases may sometimes be underestimated because of the relapsing nature of the disease and thus some newly developing phenomena may be overlooked. In this case we present a 12- year old female patient who was followed up for juvenile idiopathic arthritis and applied to us as an exacerbation of the disease. After initiation of therapy all her complaints but the right knee improved. In the examination of knee, limitation in hip movements was detected. X- ray of the hip revealed a mass neighboring the minor trochanter. On magnetic resonance imaging the mass was detected to be an osteoid osteoma. The patient is free of pain with conservative treatment for tumor after twelve months. It is important to evaluate the patient thoroughly without focusing on a single point and keep in mind that in especially skeletally immature patients hip pain can be felt in the knee.

  12. Novel self-epitopes derived from aggrecan, fibrillin, and matrix metalloproteinase-3 drive distinct autoreactive T-cell responses in juvenile idiopathic arthritis and in health

    NARCIS (Netherlands)

    S.S.M. Kamphuis (Sylvia); K. Hrafnkelsdóttir (Kolbrún); M. Klein (Mark); W. de Jager (Wilco); M.H. Haverkamp (Margje); J.H.M. van Bilsen (Jolanda); S. Albani (Salvatore); W. Kuis (Wietse); M.H.M. Wauben (Marca); B.J. Prakken (Berent)

    2006-01-01

    textabstractJuvenile idiopathic arthritis (JIA) is a heterogeneous autoimmune disease characterized by chronic joint inflammation. Knowing which antigens drive the autoreactive T-cell response in JIA is crucial for the understanding of disease pathogenesis and additionally may provide targets for an

  13. Pharmacokinetics of fenclofenac in children with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Mäkelä, A L; Scheinin, M; Iisalo, E; Salonen, J S

    1983-01-01

    Twenty eight children (age range 3-17 years) with juvenile rheumatoid arthritis (JRA) received fenclofenac 10-25 mg/kg body weight daily on an open basis. Pharmacokinetic analysis was undertaken on plasma fenclofenac levels measured during the first 3 weeks of treatment. The peak concentration after the first dose was achieved in 2-8 h in non-fasting subjects and was linearly related to dose. The plasma level then decayed biexponentially, as in adults, the initial distribution phase extending to about 12 h after dosing. After treatment for 18 days, blood samples were taken during the 96 h following the last dose of the drug to define the steady state elimination profile. The elimination half-life was 25.4 +/- 7.9 h (n = 17) and did not appear to be dependent on the daily dosage. A therapeutic drug concentration of greater than or equal to 100 micrograms/ml emerged from subjective and objective estimates of the response to treatment and measurement of steady state fenclofenac concentration. Treatment response could be more accurately predicted with the aid of drug concentrations than from dosage alone, although the dose and the steady state drug concentration were positively and linearly correlated (r = 0.61, p less than 0.01). Of 16 children receiving doses in excess of 20 mg/kg/day, 3 experienced dose-related adverse effects, increased serum transaminase activity, vertigo and dyspnoea.

  14. Dosing celecoxib in pediatric patients with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Krishnaswami, Sriram; Hutmacher, Matt M; Robbins, Jeffery L; Bello, Akintunde; West, Christine; Bloom, Bradley J

    2012-08-01

    The objective was to derive dosing recommendations for the use of celecoxib in patients with juvenile rheumatoid arthritis (JRA) using pharmacokinetic (PK) and exposure-response data. PK and efficacy data from a randomized, double-blind, 12-week study of celecoxib dosed at 3 and 6 mg/kg twice a day (bid) as an investigational suspension formulation in 152 JRA patients aged 2 to 17 years, PK data from 36 adult RA patients, and relative bioavailability data in healthy adults comparing suspension or capsule sprinkles with the commercial capsule were analyzed. Typical oral clearance (L/h) values were 40% and 24% lower in patients weighing 10 and 25 kg, respectively, compared with a 70-kg patient. Longitudinal, logistic pharmacodynamic models incorporating linear effects of dose/area under the plasma concentration-time curve (AUC) over 0 to 12 hours (AUC(0-12)) suggested that the percentage of responders increased with celecoxib exposure. Systemic exposures (AUC) were similar for the suspension, capsule sprinkles, and intact capsule. Administration of a 50-mg bid capsule (or sprinkles) for patients weighing 10 to 25 kg and 100 mg bid for patients >25 kg was predicted to yield similar exposures and response rates as those observed in the JRA trial. Doses and dosage forms not studied in the JRA trial were approved based on the results of this analysis.

  15. [Uveitis associated with juvenile idiopathic arthritis : Optimization of immunomodulatory therapy].

    Science.gov (United States)

    Heiligenhaus, A; Tappeiner, C; Walscheid, K; Heinz, C

    2016-05-01

    Uveitis associated with juvenile idiopathic arthritis (JIA-associated uveitis) is a vision-threatening disorder with a high complication rate. Besides early diagnosis within screening programs an adequate therapy is essential for improvement of the long-term prognosis. Corticosteroid therapy is often insufficient. In addition to conventional immunosuppression, immunomodulatory drugs, so-called biologicals, are novel highly effective treatment modalities. A systematic search of the literature was carried out for biologicals currently used in the treatment of JIA-associated uveitis. Review of current publications, summary of treatment guidelines and discussion of treatment options for therapy refractive patients. In accordance with the current recommendations tumor necrosis factor (TNF) inhibitors are administered if uveitis inactivity cannot be achieved with topical corticosteroids and in the next stage with immunosuppressants (methotrexate preferred). According to the currently available data adalimumab is then preferred. When the effectiveness of TNF inhibitors ceases during long-term administration and/or recurrences, other biological response modifiers are attractive treatment options (e. g. lymphocyte inhibitors or specific receptor antagonists). The TNF inhibitors are of major importance for the treatment of JIA-associated uveitis. Prospective studies and registries would be desirable in order to be able to compare the value of TNF inhibitors and other biologicals and for optimization of treatment recommendations.

  16. A comprehensive review of the genetics of juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Glass David N

    2008-07-01

    Full Text Available Abstract Juvenile idiopathic arthritis (JIA is the most common chronic arthropathy of childhood which is believed to be influenced by both genetic and environmental factors. The progress in identifying genes underlying JIA susceptibility using candidate gene association studies has been slow. Several associations between JIA and variants in the genes encoding the human leukocyte antigens (HLA have been confirmed and replicated in independent cohorts. However it is clear that genetic variants outside the HLA also influence susceptibility to JIA. While a large number of non-HLA candidate genes have been tested for associations, only a handful of reported associations such as PTPN22 have been validated. In this review we discuss the principles behind genetic studies of complex traits like JIA, and comprehensively catalogue non-HLA candidate-gene association studies performed in JIA to date and review several validated associations. Most candidate gene studies are underpowered and do not detect associations, and those that do are often not replicated. We also discuss the principles behind genome-wide association studies and discuss possible implications for identifying genes underlying JIA. Finally we discuss several genetic variants underlying multiple clinically distinct autoimmune phenotypes.

  17. A critical appraisal of radiographic scoring systems for assessment of juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Doria, Andrea S.; Babyn, Paul S. [The Hospital for Sick Children, Department of Diagnostic Imaging, Toronto, Ontario (Canada); University of Toronto, Department of Medical Imaging, Toronto, Ontario (Canada); Feldman, Brian [The Hospital for Sick Children, Department of Rheumatology, Toronto, Ontario (Canada); University of Toronto, Department of Population Health Sciences, Toronto (Canada)

    2006-08-15

    Assessing structural damage to joints over time is essential for evaluating the effectiveness of therapeutic interventions for patients with inflammatory arthritis. Although radiography is able to quantify joint damage, the changes found with conventional radiography early in the disease course are nonspecific, and late radiographic changes are often irreversible. Although many clinical trials on drug development for children still use radiographic scales as endpoints for the study, more specific therapies have been developed for juvenile idiopathic arthritis (JIA) that would enable imaging to ''fine-tune'' patients to placement into specific treatment algorithms. As a result, new imaging scales to identify early abnormalities are clearly needed. Many pediatric rheumatology centers around the world persistently apply adult-designed radiographic scoring systems to evaluate the progression of JIA. Few pediatric-targeted radiographic scales are available for assessment of progression of JIA in growing joints, and the clinimetric and psychometric properties of such scales have been poorly investigated. We present a critique to the evaluative, discriminative, and predictive roles of the van der Heijde modification of Sharp's radiographic method, a scale originally designed to assess damage to joints of adults with rheumatoid arthritis, when it is applied to a pediatric population. We discuss the advantages and drawbacks of this radiographic scoring system for assessing growing joints and the ability of MRI to overcome inadequacies of conventional radiography. (orig.)

  18. Juvenile rheumatoid arthritis in velo-cardio-facial syndrome: Coincidence of unusual complication?

    Energy Technology Data Exchange (ETDEWEB)

    Rasmussen, S.A.; Williams, C.A.; Gray, B.A. [Univ. of Florida, Gainesville, FL (United States)] [and others

    1996-09-06

    We report on two patients with velo-cardio-facial syndrome (VCFS) and juvenile rheumatoid arthritis (JRA). The first, a 9-year-old girl, presented with microcephaly, characteristic face, congenital heart disease, and velopharyngeal insufficiency. Fluorescence in situ hybridization (FISH) study showed deletion of D22S75 (N25), confirming the diagnosis of VCFS. At age 7, she developed joint pain, and polyarticular JRA was diagnosed. Awareness of this case led to the subsequent diagnosis of VCFS (also confirmed by FISH) in another, unrelated 12-year-old girl with characteristic face, hypernasal speech, and obesity. JRA was first diagnosed in this case at age 5 years, and she subsequently developed severe polyarticular disease. Neither patient had clinical or laboratory evidence of immunodeficiency. This observation represents the first report of the association of JRA with VCFS and raises the question of whether this is a coincidental association or a rare complication of this condition. 33 refs., 4 figs., 1 tab.

  19. Accelerometry-based monitoring of daily physical activity in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Nørgaard, M; Twilt, M; Andersen, L B

    2015-01-01

    Objectives: Juvenile idiopathic arthritis (JIA) may cause functional impairment, reduced participation in physical activity (PA) and, over time, physical deconditioning. The aim of this study was to objectively monitor daily free-living PA in 10-16-year-old children with JIA using accelerometry...... with regard to disease activity and physical variables and to compare the data with those from healthy age- and gender-matched controls.Method: Patients underwent an evaluation of disease activity, functional ability, physical capacity, and pain. Accelerometer monitoring was assessed using the GT1M Acti...... range of motion (ROM). No correlation was found between PA and pain scores, functional ability, and hypermobility. Patients with involvement of ankles or hips demonstrated significantly lower levels of PA.Conclusions: Children with JIA are less physically active and have lower physical capacity...

  20. MACROPHAGE ACTIVATION SYNDROME AS A COMPLICATION OF SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS – CASE REPORT

    Directory of Open Access Journals (Sweden)

    Viktorija Kerin

    2014-05-01

    Full Text Available 800x600 Abstract Macrophage activation syndrome (MAS is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA. MAS is characterized by systemic inflammation caused by excessive or uncontrolled release of proinflammatory cytokines (cytokine storm. The diagnostic hallmark are hemophagocytic macrophages, that could be present in bone marrow, liver, spleen or lymph nodes. Clinical features are similar to a flare of the underlying rheumatic disease which makes early recognition and choice of the appropriate treatment difficult. Diagnosis is made according to the preliminary diagnostic guidelines for MAS complicating SJIA.We report a case of an 11 years old girl with MAS as an initial presentation of SJIA. She was successfully treated with high doses of glucocorticoid and cyclosporine. After discontinuation of glucocorticoid therapy she developed a new flare of the disease which was successfully treated with interleukin 1 blocking agent anakinra.         

  1. Gene Expression Deconvolution for Uncovering Molecular Signatures in Response to Therapy in Juvenile Idiopathic Arthritis.

    Directory of Open Access Journals (Sweden)

    Ang Cui

    Full Text Available Gene expression-based signatures help identify pathways relevant to diseases and treatments, but are challenging to construct when there is a diversity of disease mechanisms and treatments in patients with complex diseases. To overcome this challenge, we present a new application of an in silico gene expression deconvolution method, ISOpure-S1, and apply it to identify a common gene expression signature corresponding to response to treatment in 33 juvenile idiopathic arthritis (JIA patients. Using pre- and post-treatment gene expression profiles only, we found a gene expression signature that significantly correlated with a reduction in the number of joints with active arthritis, a measure of clinical outcome (Spearman rho = 0.44, p = 0.040, Bonferroni correction. This signature may be associated with a decrease in T-cells, monocytes, neutrophils and platelets. The products of most differentially expressed genes include known biomarkers for JIA such as major histocompatibility complexes and interleukins, as well as novel biomarkers including α-defensins. This method is readily applicable to expression datasets of other complex diseases to uncover shared mechanistic patterns in heterogeneous samples.

  2. Disk abnormality coexists with any degree of synovial and osseous abnormality in the temporomandibular joints of children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Kirkhus, Eva; Smith, Hans-Joergen [Oslo University Hospital, Rikshospitalet, Department of Radiology and Nuclear Medicine, Oslo (Norway); University of Oslo, Institute of Clinical Medicine, Oslo (Norway); Arvidsson, Linda Z.; Larheim, Tore A. [University of Oslo, Department of Maxillofacial Radiology, Institute of Clinical Dentistry, Oslo (Norway); Flatoe, Berit; Hetlevik, Siri O. [Oslo University Hospital, Rikshospitalet, Department of Rheumatology, Oslo (Norway); University of Oslo, Institute of Clinical Medicine, Oslo (Norway)

    2016-03-15

    MRI manifestation of temporomandibular joint arthritis is frequently reported in children with juvenile idiopathic arthritis. However, little attention has been paid to temporomandibular joint disk abnormalities. To assess combinations of MRI findings in the symptomatic temporomandibular joint in children with juvenile idiopathic arthritis with focus on disk abnormalities. This was a retrospective study of 46 patients with juvenile idiopathic arthritis, mean age 12 years (range: 5-17 years). Mean disease duration was 70 months (standard deviation: 61 months). MR images of 92 temporomandibular joints were scored for thickness of abnormally enhancing synovium (synovitis), joint effusion, bone marrow oedema, abnormal bone shape, bone erosion and disk abnormalities. The 92 temporomandibular joints were categorized as A: No synovitis and normal bone shape (30/92; 33%), B: Synovitis and normal bone shape (14/92: 15%), C: Synovitis and abnormal bone shape (38/92; 41%) and D: No synovitis but abnormal bone shape (10/92; 11%). Thirty-six of the 46 patients (78%) had synovitis and 33/46 (72%) had abnormal bone shape, most frequently in combination (30/46; 65%). Disk abnormalities (flat disk, fragmented disk, adherent disk and displaced disk) were found in 29/46 patients (63%). Disk abnormalities were found in all categories of juvenile idiopathic arthritis involved temporomandibular joints (B: 8/14 [57%]; C: 25/38 [66%] and D: 7/10 [70%]). Disk displacement was found in half of the joints (7/14) in category B. Synovitis was most pronounced in this category. Disk abnormalities were frequent. Disk displacement also occurred in joints with early temporomandibular joint arthritis, i.e., with normal bone shape. Other disk abnormalities were found in joints with bone abnormalities. Attention should be paid to disk abnormalities both in early and long-standing temporomandibular joint arthritis in children with juvenile idiopathic arthritis. (orig.)

  3. Disk abnormality coexists with any degree of synovial and osseous abnormality in the temporomandibular joints of children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Kirkhus, Eva; Arvidsson, Linda Z; Smith, Hans-Jørgen; Flatø, Berit; Hetlevik, Siri O; Larheim, Tore A

    2016-03-01

    MRI manifestation of temporomandibular joint arthritis is frequently reported in children with juvenile idiopathic arthritis. However, little attention has been paid to temporomandibular joint disk abnormalities. To assess combinations of MRI findings in the symptomatic temporomandibular joint in children with juvenile idiopathic arthritis with focus on disk abnormalities. This was a retrospective study of 46 patients with juvenile idiopathic arthritis, mean age 12 years (range: 5-17 years). Mean disease duration was 70 months (standard deviation: 61 months). MR images of 92 temporomandibular joints were scored for thickness of abnormally enhancing synovium (synovitis), joint effusion, bone marrow oedema, abnormal bone shape, bone erosion and disk abnormalities. The 92 temporomandibular joints were categorized as A: No synovitis and normal bone shape (30/92; 33%), B: Synovitis and normal bone shape (14/92: 15%), C: Synovitis and abnormal bone shape (38/92; 41%) and D: No synovitis but abnormal bone shape (10/92; 11%). Thirty-six of the 46 patients (78%) had synovitis and 33/46 (72%) had abnormal bone shape, most frequently in combination (30/46; 65%). Disk abnormalities (flat disk, fragmented disk, adherent disk and displaced disk) were found in 29/46 patients (63%). Disk abnormalities were found in all categories of juvenile idiopathic arthritis involved temporomandibular joints (B: 8/14 [57%]; C: 25/38 [66%] and D: 7/10 [70%]). Disk displacement was found in half of the joints (7/14) in category B. Synovitis was most pronounced in this category. Disk abnormalities were frequent. Disk displacement also occurred in joints with early temporomandibular joint arthritis, i.e., with normal bone shape. Other disk abnormalities were found in joints with bone abnormalities. Attention should be paid to disk abnormalities both in early and long-standing temporomandibular joint arthritis in children with juvenile idiopathic arthritis.

  4. Juvenile idiopathic arthritis: how can the radiologist help the clinician?

    Energy Technology Data Exchange (ETDEWEB)

    Azouz, E.M. [Children' s Hospital of Eastern Ontario, Radiology Department, Ottawa, ON (Canada)

    2008-06-15

    The classification of the International League of Associations for Rheumatology (ILAR) is based on clinical criteria and includes: 1. Systemic arthritis 2. Oligoarthritis 3. Polyarthritis, rheumatoid factor positive 4. Polyarthritis, rheumatoid factor negative 5. Enthesitis-related arthritis 6. Psoriatic arthritis 7. Undifferentiated arthritis. Systematic arthritis is different from the other arthritides. It is associated with fever, rash, hepatosplenomegaly and lymphadenopathy. The arthritis is polyarticular and symmetrical. The enlarged liver, spleen and lymph nodes may be detected and followed clinically and, more accurately, with the help of cross-sectional imaging modality such as US or MRI. CT should be avoided in children because of the ionizing radiation. (orig.)

  5. Juvenile Huntington disease in Argentina.

    Science.gov (United States)

    Gatto, Emilia Mabel; Parisi, Virginia; Etcheverry, José Luis; Sanguinetti, Ana; Cordi, Lorena; Binelli, Adrian; Persi, Gabriel; Squitieri, Ferdinando

    2016-01-01

    We analyzed demographic, clinical and genetic characteristics of juvenile Huntington disease (JHD) and it frequency in an Argentinean cohort. Age at onset was defined as the age at which behavioral, cognitive, psychiatric or motor abnormalities suggestive of JHD were first reported. Clinical and genetic data were similar to other international series, however, in this context we identified the highest JHD frequency reported so far (19.72%; 14/71). Age at onset of JHD is challenging and still under discussion. Our findings reinforce the hypothesis that clinical manifestations, other than the typical movement disorder, may anticipate age at onset of even many years. Analyses of JHD cohorts are required to explore it frequency in populations with different backgrounds to avoid an underestimation of this rare phenotype. Moreover, data from selected populations may open new pathways in therapeutic approaches and may explain new potential correlations between HD presentations and environmental or biological factors.

  6. Psychological Profile in Children and Adolescents with Severe Course Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Emanuela Russo

    2012-01-01

    Full Text Available Objective. Juvenile Idiopathic Arthritis (JIA is the most common chronic pediatric rheumatic disease. It is recognized that only reliance on clinical signs of disease outcome is inadequate for understanding the impact of illness and its treatment on child’s life and functioning. There is a need for a multidisciplinary and holistic approach to children with arthritis which considers both physical and emotional functioning. This study investigated the psychosocial functioning of children and adolescent with JIA and the disease-related changes in their family. Methods. The sample consisted of 33 hospitalized patients, aged 6–16 years. Both parents and the children were given a number of questionnaire to fill out. Clinical information was extracted from the interviews. Results. Self-reported psychological functioning (depression, anxiety, and behavior was not different from the normal population; however significant psychological suffering was detected by the clinical interview. Conclusions. Children and adolescents with JIA do not show overt psychopathology by structured assessment; nevertheless a more clinically oriented holistic approach confirms JIA as a disrupting event causing relevant changes in the quality of life of the affected families.

  7. Increasing feasibility and patient comfort of MRI in children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hemke, Robert [Academic Medical Center, Department of Radiology, Amsterdam (Netherlands); Emma Children' s Hospital AMC, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Amsterdam (Netherlands); Veenendaal, Mira van; Kuijpers, Taco W. [Emma Children' s Hospital AMC, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Amsterdam (Netherlands); Rossum, Marion A.J. van [Emma Children' s Hospital AMC, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Amsterdam (Netherlands); Jan van Breemen Institute, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Maas, Mario [Academic Medical Center, Department of Radiology, Amsterdam (Netherlands)

    2012-04-15

    MRI is the most sensitive imaging modality in juvenile idiopathic arthritis (JIA), but has practical limitations. Optimizing the scanning protocol is, therefore, necessary to increase feasibility and patient comfort. To determine the feasibility of bilateral non-contrast-enhanced open-bore MRI of knees and to assess the presence of literature-based MRI features in unsedated children with JIA. Children were classified into two clinical subgroups: active arthritis (group 1; n = 29) and inactive disease (group 2; n = 18). MRI features were evaluated using a literature-based score, comprising synovial hypertrophy, cartilage lesions, bone erosions, bone marrow changes, infrapatellar fat pad heterogeneity, effusion, tendinopathy and popliteal lymphadenopathy. The MRI examination was successfully completed in all 47 children. No scan was excluded due to poor image quality. Synovial hypertrophy was more frequent in group 1 (36.2%), but was also seen in 19.4% of the knees in group 2. Infrapatellar fat pad heterogeneity was more prevalent in group 2 (86.1%; P = 0.008). Reproducibility of the score was good (Cohen kappa, 0.49-0.96). Bilateral non-contrast-enhanced open-bore knee MRI is feasible in the assessment of disease activity in unsedated children with JIA. Signs differing among children with active and inactive disease include infrapatellar fat pad heterogeneity and synovial hypertrophy. (orig.)

  8. PROBLEM OF METABOLIC DISORDERS IN CHILDREN WITH JUVENILE ARTHRITIS LIVING IN THE REPUBLIC OF MORDOVIA

    Directory of Open Access Journals (Sweden)

    A. V. Krasnopolskaya

    2014-01-01

    Full Text Available It is assumed that juvenile idiopathic arthritis (JIA, as many other rheumatic diseases, is in close pathogenic connection with metabolic disorders and early atherosclerosis. However, the prevalence of metabolic syndrome and its components both in healthy Finno-Ugrian children and teens and JIA patients is unknown.Objective of the present work was to study the prevalence of metabolic disorders in children with JIA, living in the Republic of Mordovia.Subjects and methods. Authors have examined 82 children (among them 44 girls with JIA aged 10–18 years. Results. Full complex of metabolic syndrome symptoms was revealed in 36.6% of patients, most of which had arthritis. Dyslipidaemia, obesity and arterial hypertension were recorded most frequently and correlated with activity of the disease and the dose of systemic glucocorticoids.Conclusion. JIA is associated with high prevalence of metabolic disorders which only partially (arterial hypertension and carbohydrate metabolism disorders are connected with glucocorticoid therapy and mainly determined by the high inflammatory activity of the disease.

  9. Tocilizumab: The evidence for its place in the treatment of juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Troels Herlin

    2009-08-01

    Full Text Available Troels HerlinDepartment of Pediatrics, Aarhus University Hospital, Skejby, Aarhus, DenmarkIntroduction: Juvenile idiopathic arthritis (JIA is one of the most common chronic diseases with childhood onset. It comprises different subtypes of which the systemic onset subtype is often resistant to treatment. With the advent of biological treatment with tumor necrosis factor-α (TNFα-inhibitors, the clinical outcome of JIA has improved considerably, but only for subtypes other than systemic JIA. Substantial evidence shows that the proinflammatory cytokine interleukin-6 (IL-6 plays a pivotal role in systemic JIA. The blockage of IL-6 action by tocilizumab, a humanized anti-IL-6-receptor monoclonal antibody, could therefore be an effective treatment of systemic JIA.Aims: The purpose of this article was to review the clinical trials of tocilizumab and to discuss its place in the treatment of JIA with the focus on the systemic onset of disease. Evidence review: Two phase II studies and one phase III clinical trial of tocilizumab demonstrating the clinical efficacy and safety in systemic onset JIA have been published. Within those studies, sustained and high response rates of clinical improvement have been achieved with American College of Rheumatology Pediatric criteria (ACRPed 30, 50, and 70 observed in 98%, 94%, and 90% of patients, respectively, after 48 weeks. One study regarding the clinical efficacy of tocilizumab for the treatment of oligo- and polyarticular JIA has been presented only as a conference abstract.Place in therapy: The very promising results seen so far in patients with severe systemic JIA and acceptable tolerability gives tocilizumab a central role in the future therapy in controlling this disease. No other biological therapy has achieved similar high response rates when treating with tocilizumab 8 mg/kg every two weeks to patients with systemic onset JIA, but direct comparison of the efficacy of different biological agents are not

  10. Incidence of herpes zoster infections in juvenile idiopathic arthritis patients.

    Science.gov (United States)

    Nimmrich, S; Horneff, G

    2015-03-01

    The risk of herpes zoster among patients with juvenile idiopathic arthritis (JIA) exposed to biologics has not been evaluated. We determined incidence rates of herpes zoster among children with JIA in correlation with medication at time of occurrence and total drug exposure. The German biologics register database was used to identify patients with herpes zoster. Crude infection rates and incidence ratios (IRR) were compared to published rates. Demographics and overall exposure and particular exposure time to corticosteroids, immunosuppressive drugs and biologics were analyzed. The JIA cohort included 3,042 patients with 5,557.9 person-years of follow-up; 1,628 have used corticosteroids, 2,930 methotrexate and 1,685 etanercept. In total, 17 herpes zoster events have been documented [6/1,000 patients (3.5-9.0); 3.1/1,000 patient-years (1.9-4.9)]. Thus, the incidence rate in JIA patients was higher than expected [IRR 2.9 (1.8-4.5), p herpes zoster. Compared to the healthy population, a significant higher IRR is observed in JIA patients who received a monotherapy with etanercept or in combination with steroids and methotrexate, but not in JIA patients exposed to methotrexate without biologics. In comparison with our control group of patients treated with methotrexate, the IRR was higher for exposure to etanercept monotherapy and combination of etanercept and corticosteroids irrespective of methotrexate use. A generally higher incidence rate in JIA patients treated with etanercept was observed. No serious or refractory manifestations occurred.

  11. Sleep Fragmentation and Biomarkers in Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Ward, Teresa M; Yuwen, Weichao; Voss, Joachim; Foell, Dirk; Gohar, Faekah; Ringold, Sarah

    2016-05-01

    (1) To compare sleep (nighttime sleep duration and sleep efficiency) and sleep fragmentation (movement and fragmentation index), as measured by actigraphy, and symptoms (pain and fatigue) in 8- to 14-year-old children with polyarticular and extended oligoarticular juvenile idiopathic arthritis (JIA) and (2) to examine the associations between sleep fragmentation (movement and fragmentation index) and the calcium-binding protein biomarkers S100A12 and myeloid-related protein (MRP8/14). Participants included 40 children with extended oligoarticular (n = 15) or polyarticular (n = 25) JIA and their parents. Serum protein samples were obtained during routine rheumatology clinic visits. Children completed the PedsQL Multidimensional Fatigue Scale and daily pain and sleep diaries and wore actigraphy monitors for 9 consecutive days. Parents completed the Children's Sleep Habits Questionnaire (CSHQ). Of the 40 children, 68% scored above the CSHQ clinical cutoff score for sleep disturbances. Mean nighttime sleep duration was 7.5 hr, and mean sleep efficiency was 85.3%. Group differences were not found for nighttime sleep duration, sleep efficiency, movement and fragmentation index, or S100A12 and MRP8/14 protein concentrations. In a stepwise regression, medications, joint count, and movement and fragmentation index explained 21% of the variance in MRP8/14 concentration. Decreased nighttime sleep duration, poor sleep efficiency, and fragmented sleep were observed in our sample, regardless of JIA category. Sleep fragmentation was a significant predictor of MRP8/14 protein concentration. Additional research is needed to understand the interrelations among sleep fragmentation, effects of medication, and S100A12 and MRP8/14 protein biomarkers in JIA. © The Author(s) 2015.

  12. [Magnetic resonance imaging in juvenile idiopathic arthritis: peculiarities of imaging children].

    Science.gov (United States)

    Navallas, M; Rebollo Polo, M; Riaza, L; Muchart López, J; Maristany, T

    2013-09-01

    The term juvenile idiopathic arthritis (JIA) encompasses a heterogeneous group of arthritides with no known cause that begin before the age of 16 years and persist for at least 6 weeks. In recent decades, imaging techniques have acquired a fundamental role in the diagnosis and follow-up of JIA, owing to the unification of the different criteria for classification, which has strengthened the research in this field, and to the development of disease-modifying antirheumatic drugs. In this article, we briefly explain what JIA is. Moreover, we describe the role and limitations of plain-film radiography, ultrasonography, and magnetic resonance imaging (MRI). Finally, we review the MRI protocol and findings, and we comment on the differential diagnosis. Copyright © 2012 SERAM. Published by Elsevier Espana. All rights reserved.

  13. A biopsychosocial investigation of pediatric chronic pain with special focus on juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Lomholt, Johanne Jeppesen

    Our understanding and management of pediatric chronic pain have advanced markedly over the last half century. Chronic pain is pain that persists for a usually more than three months and is highly prevalent in children and adolescents. Juvenile idiopathic arthritis (JIA) can be characterized...... increased quality of life, reductions in anxiety levels and pain catastrophizing, and improvements in adaptive pain cognitions; the latter were expressed as strengthened beliefs in the ability to control pain and self-efficacy. After controlling for disease activity, no differences between the intervention...... and waitlist condition were found in measures of pain and functional disability. The feasibility of the CBT program was supported by a low drop-out rate, and high levels of reported intervention credibility and satisfaction with the treatment. In study 4 differences in pain and health complaints was examined...

  14. Decreased levels of sCD21 and sCD23 in blood of patients with systemic-juvenile arthritis, polyarticular-juvenile arthritis, and pauciarticular-juvenile arthritis.

    Science.gov (United States)

    Singh, Anjana; Vastert, Sebastiaan J; Prakken, Berent J; Illges, Harald

    2012-06-01

    A soluble form of CD21 (sCD21) and CD23 (sCD23) is released from the surface of human white blood cells upon shedding of the extracellular domain. sCD21 circulates in a complex with cleavage fragments of C3 and sCD23, which were previously identified as ligands of membrane and soluble CD21. sCD21 seems to be a marker of chronic inflammatory disease. To assess the sCD21 and sCD23 status in patients with subsets of juvenile arthritis (JA), we determined plasma levels sCD21 and sCD23. Plasma sCD21 levels were significantly decreased in all JA subtypes (O-JA P < 0.0068; P- and S-JA P < 0.0001) compared to healthy controls. Plasma sCD23 levels were significantly decreased in P-JA and S-JA (both P < 0.0001), but not in O-JA (P < 0.3843) in comparison with healthy controls, and data statistically analyzed. Our results suggest that pathological mechanisms relevant to autoimmune disorders interfere with the regulation of both CD21 and CD23 shedding.

  15. TOTAL JOINT REPLACEMENT OF THE LOWER EXTREMITY IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS

    OpenAIRE

    Стюарт Б. Гудмэн

    2014-01-01

    Joint replacement of the lower extremity in Juvenile Idiopathic Arthritis (JIA) is becoming more commonly performed worldwide. These young adults experience severe pain and disability from end-stage arthritis, and require joint replacement of the hip or knee to alleviate pain, and restore ambulation and function. These procedures are very challenging from the anesthesia and surgical point of view, due to small overall proportions, numerous bony and other deformities and soft tissue contractur...

  16. Changes of Platelet Indices in Juvenile Idiopathic Arthritis in Acute Phase and After Two Months Treatment

    Directory of Open Access Journals (Sweden)

    Marjan Vakili

    2016-05-01

    Full Text Available Background Various indices have been raised as predictors of activity and severity of juvenile idiopathic arthritis. Objectives This study was conducted to investigate the changes of platelet indices in acute phase and two months after treatment in these patients. Patients and Methods In a cohort study, platelet count, mean platelet volume (MPV, platelet distribution width (PDW, plateletcrit (PCT were evaluated in children referred to children’s medical center, Tehran due to juvenile idiopathic arthritis from March 2013 to March 2014 during the acute phase and two months after standard treatment. The statistical data were analyzed by SPSS 19 software, and the significance level was set as P < 0.05. Results In this study, 55 children (24 boys and 31 girls with mean ± SD age of 7.50 ± 3.35 years were studied. The mean ± SD value of platelet count was 441872.7 ± 151836.9 in the acute phase and reached 395418.2 ± 119601.6 two months after treatment (P = 0.01. The mean ± SD PCT in the acute phase of various subtypes of the disease was 0.32 ± 0.11, which reached 0.29 ± 0.10 after treatment (P = 0.09. However, the PDW range in different subtypes of the disease reached 13.4 ± 8.0 from 13.9 ± 2.9 and MPV reached 8.7 ± 0.9 from 8.8 ± 1.1 after treatment, but they were not significantly different from the results in the acute phase (P = 0.5. Conclusions Platelet count is one of the most remarkable indices in JIA. Evaluation of PCT can also help determine the severity of the inflammatory process in the follow-up and treatment process.

  17. Juvenile idiopathic arthritis and athletic participation: are we adequately preparing for sports integration?

    Science.gov (United States)

    Taxter, Alysha; Foss, Kim Barber; Melson, Paula; Ford, Kevin R; Shaffer, Michael; Myer, Gregory D

    2012-09-01

    Children with juvenile idiopathic arthritis (JIA) now have well-controlled disease due to improved therapies and management strategies. Children with JIA are more active than in the past and often participate in dynamic, high-loading sports. Standard measures of disease control include examination findings, laboratory values, and patient-directed surveys. However, these standards do not address the subtle deficits in biomechanics and neuromuscular control, which could place affected joints at higher risk for injury. Currently, there are limited evidence-based guidelines to structure conditioning recommendations as to the fitness and mechanics needed to provide safe integration into sports in this population; therefore, tools that objectively measure function with high accuracy and precision may be warranted. Previous work using 3-dimensional motion analysis demonstrated usefulness in guiding physical therapy treatment to correct these deficits. The use of a multidisciplinary team, including physical therapy, rheumatology, and sports medicine, is crucial for preparing these children to return to play. We suggest that the child transition into a sport preparatory-conditioning program to address any underlying deficits. A pediatric exercise specialist who is sensitive to the needs of this population can work with a physical therapist to then appropriately integrate the child safely into sport. Encouraging an active lifestyle is vital to the management of JIA and does not worsen the symptoms associated with childhood arthritis.

  18. Orofacial symptoms related to temporomandibular joint arthritis in juvenile idiopathic arthritis: smallest detectable difference in self-reported pain intensity.

    Science.gov (United States)

    Stoustrup, Peter; Kristensen, Kasper D; Verna, Carlalberta; Küseler, Annelise; Herlin, Troels; Pedersen, Thomas K

    2012-12-01

    Temporomandibular joint (TMJ) inflammation in patients with juvenile idiopathic arthritis (JIA) may lead to mandibular growth disturbances and interfere with optimal joint and muscle function. Orofacial symptoms are common clinical findings in relation to TMJ arthritis in adolescence. Knowledge about their clinical manifestation is important for TMJ arthritis diagnosis, treatment choice, and outcome evaluation. The aim of our prospective observational study was to evaluate and describe the frequency, the main complaints, and the localization of TMJ arthritis-related orofacial symptoms. The smallest detectable differences (SDD) for minimal, average, and maximal pain were estimated. Thirty-three patients with JIA and arthritis-related orofacial symptoms in relation to 55 affected TMJ were included in our questionnaire study (mean age 14.11 yrs). Calculation of the SDD was based on a duplicate assessment 45 min after the first questionnaire was completed. The majority of the patients had common orofacial symptoms during mastication and maximal mouth opening procedures. Persistent orofacial symptoms were rare. The TMJ area in combination with the masseter muscle region was the orofacial region where symptoms were most common. The SDD for minimal, average, and maximal pain were between 10 and 14 mm on a visual analog scale. Our study offers new knowledge about TMJ arthritis-related orofacial symptoms that may aid diagnosis and clinical decision-making. We suggest that TMJ arthritis-related orofacial symptoms could be understood as products of the primary TMJ inflammation in combination with secondary myogenic and functional issues.

  19. Biological therapies for the treatment of juvenile idiopathic arthritis: Lessons from the adult and pediatric experiences

    Directory of Open Access Journals (Sweden)

    Matthew L Stoll

    2008-06-01

    Full Text Available Matthew L Stoll, Alisa C GotteDepartment of Pediatrics, Division of Rheumatology, UT Southwestern Medical Center, Dallas, TX, USAAbstract: Biologics have advanced the therapy of adult and pediatric arthritis. They have been linked to rare serious adverse outcomes, but the actual risk of these events is controversial in adults, and largely unknown in pediatrics. Because of the paucity of safety and efficacy data in children, pediatric rheumatologists often rely on the adult literature. Herein, we reviewed the adult and pediatric literature on five classes of medicines: Tumor necrosis factor (TNF inhibitors, anakinra, rituximab, abatacept, and tocilizumab. For efficacy, we reviewed randomized controlled studies in adults, but did include lesser qualities of evidence for pediatrics. For safety, we utilized prospective and retrospective studies, rarely including reports from other inflammatory conditions. The review included studies on rheumatoid arthritis and spondyloarthritis, as well as juvenile idiopathic arthritis. Overall, we found that the TNF inhibitors have generally been found safe and effective in adult and pediatric use, although risks of infections and other adverse events are discussed. Anakinra, rituximab, abatacept, and tocilizumab have also shown positive results in adult trials, but there is minimal pediatric data published with the exception of small studies involving the subgroup of children with systemic onset juvenile idiopathic arthritis, in whom anakinra and tocilizumab may be effective therapies.Keywords: juvenile idiopathic arthritis, biologics, rheumatoid arthritis

  20. Investigation of rheumatoid arthritis susceptibility loci in juvenile idiopathic arthritis confirms high degree of overlap

    Science.gov (United States)

    Hinks, Anne; Cobb, Joanna; Sudman, Marc; Eyre, Stephen; Martin, Paul; Flynn, Edward; Packham, Jonathon; Barton, Anne; Worthington, Jane; Langefeld, Carl D; Glass, David N; Thompson, Susan D; Thomson, Wendy

    2012-01-01

    Objectives Rheumatoid arthritis (RA) shares some similar clinical and pathological features with juvenile idiopathic arthritis (JIA); indeed, the strategy of investigating whether RA susceptibility loci also confer susceptibility to JIA has already proved highly successful in identifying novel JIA loci. A plethora of newly validated RA loci has been reported in the past year. Therefore, the aim of this study was to investigate these single nucleotide polymorphisms (SNP) to determine if they were also associated with JIA. Methods Thirty-four SNP that showed validated association with RA and had not been investigated previously in the UK JIA cohort were genotyped in JIA cases (n=1242), healthy controls (n=4281), and data were extracted for approximately 5380 UK Caucasian controls from the Wellcome Trust Case–Control Consortium 2. Genotype and allele frequencies were compared between cases with JIA and controls using PLINK. A replication cohort of 813 JIA cases and 3058 controls from the USA was available for validation of any significant findings. Results Thirteen SNP showed significant association (p<0.05) with JIA and for all but one the direction of association was the same as in RA. Of the eight loci that were tested, three showed significant association in the US cohort. Conclusions A novel JIA susceptibility locus was identified, CD247, which represents another JIA susceptibility gene whose protein product is important in T-cell activation and signalling. The authors have also confirmed association of the PTPN2 and IL2RA genes with JIA, both reaching genome-wide significance in the combined analysis. PMID:22294642

  1. [Three cases with familial Mediterranean fever misdiagnosed as juvenile idiopathic arthritis].

    Science.gov (United States)

    Li, J; Zhang, Y; Wang, W; Zhong, L Q; Song, H M

    2017-05-04

    Objective: To explore the key points of diagnosis and treatment of familial Mediterranean fever(FMF). Method: The clinical data of 3 cases with FMF misdiagnosed as Juvenile idiopathic arthritis(JIA)seen from January 2014 to June 2016 in Peking Union Medical College Hospital were retrospectively collected. The clinical manifestations, gene mutation characteristics, treatment and prognosis were also evaluated. Result: Two cases were male and 1 was female. The mean age of onset was 17 months (3 months to 36 months), while the average age of diagnosis was 6 years and 8 months (24 months to 11 years). All the 3 cases presented with periodic fever, red rash and arthritis.Two of them suffered from anemia, 2 of them showed lymphadenopathy, and 1 of them presented with hepatosplenomegaly. All of the 3 cases were diagnosed as JIA by excluding infectious diseases and neoplastic diseases and respondiug poorly to anti-infection treatment, but they benefitted little from glucocorticoids and a variety of immunosuppressive therapy. The mutations of MEFV gene were found in 3 cases by gene detection, and all of them were complex heterozygous mutations. Four reported pathogenic mutations were found: R202Q, E148Q, L110P, P369S. All the 3 cases are currently receiving oral colchicine (in accordance with the initial dose of children under the age of 5 recommended ≤ 0.5 mg/d, 5 to 10 years old children 0.5-1.0 mg/d, 10 years old children and older children 1.0-1.5 mg / d) , and the symptoms were significantly improved. Conclusion: The familial Mediterranean fever can be characterized by repeated remittent fever, red rash, arthritis, and is easy to be confused with JIA in clinical manifestation.In this paper, 3 cases were diagnosed as complex heterozygous MEFV gene mutation by gene analysis.During the 6 months follow-up, all of the 3 patients responded well to colchicine.

  2. Rheumatoid Arthritis and Periodontal Disease. An Update

    National Research Council Canada - National Science Library

    Venkataraman, Archana; Almas, Khalid

    2015-01-01

    ...: rheumatoid arthritis (RA) and periodontal disease (PD). RA is a chronic inflammatory disease of the joints, characterized by loss of connective tissue and mineralized structures, the so-called "synovial membrane...

  3. US findings of metacarpophalangeal joints in children with idiopathic juvenile arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Karmazyn, Boaz [Riley Hospital for Children, Radiology, Indianapolis, IN (United States); Bowyer, Suzanne L.; Murphy Schmidt, Kara; Ballinger, Susan H.; Beam, Thuy T. [Indiana University, Pediatric Rheumatology, Indianapolis, IN (United States); Buckwalter, Kenneth [University Hospital, Radiology, Indianapolis, IN (United States); Ying, Jun [University of Cincinnati, Biostatistics, Institute for the Study of Health, Cincinnati, OH (United States)

    2007-05-15

    Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children, with frequent involvement of the metacarpophalangeal joints (MCPJ). To compare US findings with those of radiography and clinical examination. All MCPJs in 20 children with JIA (17 females, median age 9.7 years, range 3.6 to 16.8 years) were evaluated clinically and imaged with gray-scale and color Doppler US, and 90 MCPJs were also imaged radiographically. Each MCPJ was graded on physical examination from 0 (normal) to 4 (severe) by the patient's rheumatologist. US demonstrated abnormalities in 64 of 200 MCPJs (32.0%), including pannus vascularity and/or tenosynovitis in 55 joints (27.5%) (pannus vascularity in 43, tenosynovitis in 40) and bone destruction in 25 joints (12.5%). Overall, US abnormalities and physical examination scores were significantly associated (P < 0.001). However, interobserver agreement between US and clinical evaluation was poor (kappa 0.1) and between US and radiography was only fair (kappa 0.4). US of the MCPJ in children with JIA can demonstrate cartilage thinning, bone erosions, and pannus vascularity. Abnormal US findings are significantly correlated with severity of disease as evaluated clinically. (orig.)

  4. Clinical predictors of temporomandibular joint arthritis in juvenile idiopathic arthritis: A systematic literature review.

    Science.gov (United States)

    Kristensen, Kasper Dahl; Stoustrup, Peter; Küseler, Annelise; Pedersen, Thomas Klit; Twilt, Marika; Herlin, Troels

    2016-06-01

    To assess the level of evidence for subjective and objective parameters in clinical orofacial examination and determine if predictors for temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) patients exist in the current literature. A comprehensive systematic electronic search strategy was performed in all major medical databases in June 2015. Studies were selected independently by two reviewers in accordance with a prespecified protocol and a risk of bias assessment for all included studies. Subjective examination outcome measures assessed were pain, decreased TMJ function, and TMJ sounds. The objective outcome measures assessed were maximal incisor opening, mandibular asymmetric opening, condylar translation, protrusion, myofascial pain on palpation, facial asymmetry, and micro- or retrognathism. The electronic database search identified 345 unique citations. After application of our strict, predefined inclusion and exclusion criteria, 21 articles were included and data extracted. The study heterogeneity did not allow for meta-analyses. No singular outcome measure can be suggested as a predictor of TMJ involvement in JIA, as sensitivity and/or specificity is too low compared to contrast-enhanced magnetic resonance imaging. The current low level of evidence and study heterogeneity do not allow us to conclude on singular clinical outcome measures. To increase study comparability, we call for a standardized terminology and evidence-based guidelines for clinical orofacial examination parameters in JIA patients. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Comics as an educational tool for children with juvenile idiopathic arthritis.

    Science.gov (United States)

    Mendelson, Amir; Rabinowicz, Noa; Reis, Yonit; Amarilyo, Gil; Harel, Liora; Hashkes, Philip J; Uziel, Yosef

    2017-09-02

    This study examined whether the comic book Neta and the Medikidz Explain JIA would improve disease-related knowledge and treatment adherence among patients with juvenile idiopathic arthritis (JIA). In this prospective cohort study, JIA patients answered 20 multiple-choice knowledge questions about their disease, before and after reading the comic book. Demographic, clinical, health-related quality of life and adherence data were recorded and correlated to the responses. We studied 61 patients with a mean age of 14 ± 3.3 (range 8-18) years, 67% female, 83% Jewish and 17% non-Jewish. Thirty-nine percent had oligoarthritis, 13% systemic, 32% polyarthritis 11% psoriatic and 5% enthesitis-related type JIA. The disease was active in 46%, 40% were treated with biologics/disease modifying anti-rheumatic drugs, and 34% were in remission on medication. Among the 53 patients who completed before and after quizzes, average score increased from 63 to 80% (P comic book. Twenty-seven patients who also completed the quiz 1 year after the first reading retained their knowledge (79%). We did not find a statistically significant correlation between knowledge and age, sex, disease subtype, or Child Health Questionnaire quality of life scores. Adherence to medication use, physical therapy and rheumatology clinic visits were high at baseline; thus, these did not change after reading the comic. The comic booklet Neta and the Medikidz Explain JIA is a good educational tool for increasing disease-related knowledge in children with JIA.

  6. DNA polymorphism of HLA class II genes in pauciarticular juvenile rheumatoid arthritis

    DEFF Research Database (Denmark)

    Morling, N; Friis, J; Fugger, L;

    1991-01-01

    We investigated the DNA restriction fragment length polymorphism (RFLP) of the major histocompatibility complex (MHC) class II genes: HLA-DRB, -DQA, -DQB, DPA, and -DPB in 54 patients with pauciarticular juvenile rheumatoid arthritis (PJRA) and in healthy Danes. The frequencies of DNA fragments a...

  7. Increased frequency of HLA-DPw2 in pauciarticular onset juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Ødum, Niels; Morling, Niels; Friis, J;

    1986-01-01

    Thirty-six unrelated Danish patients with pauciarticular Juvenile Chronic Arthritis (PJCA) and 120 controls were typed for HLA-DPw1-w6 and the local specificity CDPHEI with bulk-expanded Primed Lymphocyte Typing (PLT) cells. The frequency of HLA-DPw2 was 52.8% in PJCA patients and 16.7% in contro...

  8. Are Bicipital Synovial Cysts in Children with Systemic Juvenile Idiopathic Arthritis still a Significant Clinical Challenge?

    DEFF Research Database (Denmark)

    Kyvsgaard, Nini; Herlin, Troels

    2015-01-01

    BACKGROUND: Large synovial cysts are rarely seen in juvenile idiopathic arthritis. When they do appear, they usually appear in the popliteal space (Baker’s cyst). Less commonly, they occur in the antecubital area or as bicipital synovial cysts. Bicipital synovial cysts present as a sudden...

  9. Constructing an Explanation of Illness with Children: A Sample Case Study of Juvenile Arthritis

    Science.gov (United States)

    Capurso, Michele; Lo Bianco, Maria; Cortis, Elisabetta; Rossetti, Corrado

    2016-01-01

    This study aimed to create a book to explain juvenile arthritis to newly diagnosed children, starting with the narratives of currently ill children. The development of the book followed a socio-constructivist approach and occurred over several stages, including: design of a comic-based workbook; conducting a workshop with ill children to listen to…

  10. Early predictors of prognosis in juvenile idiopathic arthritis : a systematic literature review

    NARCIS (Netherlands)

    van Dijkhuizen, E. H. Pieter; Wulffraat, NM

    2015-01-01

    Objectives Juvenile idiopathic arthritis (JIA) is subdivided into seven categories. Even within these categories, the prognosis varies markedly. To start appropriate treatment in patients with JIA and to inform patients and their parents correctly, it is essential to know the individual prognosis, p

  11. Depression, anxiety and pain in children with juvenile idiopathic arthritis (JIA).

    Science.gov (United States)

    Margetić, Branimir; Aukst-Margetić, Branka; Bilić, Ernest; Jelusić, Marija; Tambić Bukovac, Lana

    2005-05-01

    The aim of this study was to assess relations among depression, anxiety and pain in children with juvenile idiopathic arthritis (JIA). Pain was measured with the visual analogue scale (VAS), and depression and anxiety with depression and anxiety subscales from the Trauma Symptom Checklist for Children (TSC-C). Pain perception was significantly correlated with depression scores.

  12. Family Health and Characteristics in Chronic Fatigue Syndrome, Juvenile Rheumatoid Arthritis, and Emotional Disorders of Childhood.

    Science.gov (United States)

    Rangel, Luiza; Garralda, M. Elena; Jeffs, Jim; Rose, Gillian

    2005-01-01

    Objective: To compare family health and characteristics in children with chronic fatigue syndrome (CFS), in juvenile rheumatoid arthritis (JRA), and emotional disorders. Method: Parents of 28 children and adolescents aged 11 to 18 years with CFS, 30 with JRA, and 27 with emotional disorders (i.e., anxiety and/or depressive disorders) were…

  13. Delayed clinical response in patients with juvenile idiopathic arthritis treated with etanercept

    NARCIS (Netherlands)

    Otten, Marieke H; Prince, Femke H M; Twilt, Marinka; van Rossum, Marion A J; Armbrust, Wineke; Hoppenreijs, Esther P A H; Kamphuis, Sylvia; Koopman-Keemink, Yvonne; Wulffraat, Nico M; Gorter, Simone L; Ten Cate, Rebecca; van Suijlekom-Smit, Lisette W A

    2010-01-01

    OBJECTIVE: To evaluate response in patients with juvenile idiopathic arthritis (JIA) who failed to meet response criteria after 3 months of etanercept treatment. METHODS: This was a prospective ongoing multicenter observational study of all Dutch patients with JIA using etanercept. Response accordin

  14. The evaluation of uveitis in juvenile idiopathic arthritis (JIA) patients : are current ophthalmologic screening guidelines adequate?

    NARCIS (Netherlands)

    Reininga, J K; Los, L I; Wulffraat, N M; Armbrust, W

    2008-01-01

    OBJECTIVE: The aims of this study are to examine in our juvenile idiopathic arthritis (JIA) population: 1) the prevalence and characteristics of uveitis, 2) the complications and outcome of uveitis, 3) prognostic factors, and 4) the adequacy of the current ophthalmologic screening guidelines. METHOD

  15. The clinical course of juvenile idiopathic arthritis-associated uveitis in childhood and puberty

    NARCIS (Netherlands)

    Hoeve, Maretta; Ayuso, Viera Kalinina; Schalij-Delfos, Nicoline E.; Los, Leonoor I.; Rothova, Aniki; de Boer, Joke H.

    2012-01-01

    Aim The long-term course of juvenile idiopathic arthritis (JIA)-associated uveitis is not known yet. This study investigates the course and activity of JIA-associated uveitis in childhood and puberty. Design Retrospective study of the clinical data of 62 JIA patients with uveitis. The main outcome m

  16. Uveitis in childhood : Complications and treatment with emphasis on juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Sijssens, K.M.

    2008-01-01

    The aim of this study was to gain more insight into the development of complications in childhood uveitis and to evaluate the treatment options for these mostly sight-threatening conditions with emphasis on juvenile idiopathic arthritis (JIA)-associated uveitis. The second aim was to investigate whi

  17. The clinical course of juvenile idiopathic arthritis-associated uveitis in childhood and puberty

    NARCIS (Netherlands)

    Hoeve, Maretta; Ayuso, Viera Kalinina; Schalij-Delfos, Nicoline E.; Los, Leonoor I.; Rothova, Aniki; de Boer, Joke H.

    2012-01-01

    Aim The long-term course of juvenile idiopathic arthritis (JIA)-associated uveitis is not known yet. This study investigates the course and activity of JIA-associated uveitis in childhood and puberty. Design Retrospective study of the clinical data of 62 JIA patients with uveitis. The main outcome m

  18. Uveitis in childhood : Complications and treatment with emphasis on juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Sijssens, K.M.

    2008-01-01

    The aim of this study was to gain more insight into the development of complications in childhood uveitis and to evaluate the treatment options for these mostly sight-threatening conditions with emphasis on juvenile idiopathic arthritis (JIA)-associated uveitis. The second aim was to investigate whi

  19. The evaluation of uveitis in juvenile idiopathic arthritis (JIA) patients : are current ophthalmologic screening guidelines adequate?

    NARCIS (Netherlands)

    Reininga, J K; Los, L I; Wulffraat, N M; Armbrust, W

    2008-01-01

    OBJECTIVE: The aims of this study are to examine in our juvenile idiopathic arthritis (JIA) population: 1) the prevalence and characteristics of uveitis, 2) the complications and outcome of uveitis, 3) prognostic factors, and 4) the adequacy of the current ophthalmologic screening guidelines. METHOD

  20. Avaliação de provas de fase aguda em crianças e adolescentes com artrite idiopática juvenil e sua correlação com atividade da doença Acute phase reactants evaluation in children and adolescents with juvenile idiopathic arthritis and its correlation with disease activity

    Directory of Open Access Journals (Sweden)

    Aline Alencar M. F. Nicácio

    2009-06-01

    Full Text Available OBJETIVO:Analisar a relação entre as provas de fase aguda e a atividade clínica da artrite idiopática juvenil e avaliar a concordância entre velocidade de hemossedimentação e proteína C reativa (VHS e PCR na fase aguda da doença. MÉTODOS: Foi realizado estudo retrospectivo tipo coorte a partir da análise de prontuários de 30 crianças e adolescentes que preenchiam os critérios diagnósticos para artrite idiopática juvenil, estavam em atendimento em ambulatório de Reumatologia Pediátrica e haviam realizado as provas de fase aguda (VHS e PCR. RESULTADOS: Dos 30 pacientes, 21 (70% eram do sexo feminino e 19 (63,3% apresentavam o subtipo oligoarticular da doença. A média de idade de início dos sintomas foi 65,6 meses, a idade de diagnóstico de 85,3 e o tempo de evolução, 57,2 meses. As provas de fase aguda mostraram associação positiva com a atividade de doença. A anemia não teve relação com a atividade de doença. A concordância entre as duas provas de fase aguda foi superior a 80%. CONCLUSÕES: As provas de fase aguda mantêm relação positiva com a atividade da doença e o seu uso concomitante aumenta a especificidade.OBJECTIVE:To analyze the relationship between the acute phase reactants and the disease activity of Juvenile Idiopathic Arthritis (JIA and to evaluate the agreement between erythrocyte sedimentation rate and C-reactive protein during the acute phase of the disease. METHODS: a cohort retrospective study has been conducted based on the analysis of 30 children and adolescents who fulfilled the diagnostic criteria of JIA. All of them were in current follow-up at the pediatric rheumatology outpatient clinic and had acute phase reactants blood tests performed. RESULTS: Studied population comprised 30 patients: 21 (70% of them were females and 19 (63.3% presented oligoarticular subtype. The mean age at disease onset was 65.6 months; the age at diagnosis was 85.3 months and the follow-up had 57.2 months of

  1. Serum microRNAs as Potential Biomarkers of Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Kamiya, Yasuko; Kawada, Jun-ichi; Kawano, Yoshihiko; Torii, Yuka; Kawabe, Shinji; Iwata, Naomi; Ito, Yoshinori

    2015-10-01

    MicroRNAs (miRNAs) are non-coding RNAs that regulate gene expression of targeted mRNAs, which are important in the pathogenesis of autoimmune diseases. MiRNAs may have the potential to serve as biomarkers of disease. We evaluated serum levels of selected miRNAs and their associations with disease activity in juvenile idiopathic arthritis (JIA). Sera and peripheral blood leukocytes were collected from patients with JIA (8 systemic onset, 16 polyarthritis) and healthy controls. Levels of miR-16, miR-132, miR-146a, miR-155, and miR-223 were quantified. Levels of miR-223 in sera were significantly higher in patients in the active phase of systemic onset JIA than in controls. MiRNAs of peripheral blood leukocytes did not exhibit any difference between patients with JIA and controls. In both systemic onset JIA and polyarthritis patients, levels of miR-223 and miR-16 correlated with erythrocyte sedimentation rate and matrix metalloproteinase-3, respectively. MiR-146a and miR-223 in polyarthritis showed correlations with matrix metalloproteinase-3. Expressions of miRNAs were altered in patients with JIA. Serum levels of miR-223 may be a potential disease biomarker. Investigation of miRNAs could be helpful in understanding the pathogenesis of JIA and could aid in the identification of additional disease biomarkers.

  2. Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis.

    Science.gov (United States)

    Dimopoulou, Despoina; Dimosiari, Athina; Mandala, Eudokia; Dimitroulas, Theodoros; Garyfallos, Alaxandros

    2017-01-01

    Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far. The first is a 27-year-old woman with a history of polyarticular rheumatoid factor negative juvenile idiopathic arthritis, who presented with thrombocytopenia, anemia, schistocytes on blood smear, headache, and active arthritis. Originally she was treated successfully with plasma exchange, intravenous prednisone, and vincristine, and a few months after the TTP episode, she was commenced on rituximab, resulting in remission of primary disease and no relapse of TTP. The second case refers to a 29-year-old pregnant woman complaining of dizziness and fatigue with microangiopathic hemolytic anemia. She was treated with plasma exchanges, intravenous prednisolone, and INN human normal immunoglobulin with full remission of the TTP episode. Six and half years later, she was diagnosed with multiple sclerosis and was commenced on interferon beta-1 alpha, with no recurrent episode of TTP. These cases broaden the spectrum of autoimmune disorders manifested or complicated clinically by TTP. Furthermore, biological agents such as rituximab appear to be an effective treatment option for refractory cases of TTP related to systemic rheumatic disease, indicating an alternative therapeutic solution in persistent cases of this disorder.

  3. Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    Despoina Dimopoulou

    2017-07-01

    Full Text Available Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far. The first is a 27-year-old woman with a history of polyarticular rheumatoid factor negative juvenile idiopathic arthritis, who presented with thrombocytopenia, anemia, schistocytes on blood smear, headache, and active arthritis. Originally she was treated successfully with plasma exchange, intravenous prednisone, and vincristine, and a few months after the TTP episode, she was commenced on rituximab, resulting in remission of primary disease and no relapse of TTP. The second case refers to a 29-year-old pregnant woman complaining of dizziness and fatigue with microangiopathic hemolytic anemia. She was treated with plasma exchanges, intravenous prednisolone, and INN human normal immunoglobulin with full remission of the TTP episode. Six and half years later, she was diagnosed with multiple sclerosis and was commenced on interferon beta-1 alpha, with no recurrent episode of TTP. These cases broaden the spectrum of autoimmune disorders manifested or complicated clinically by TTP. Furthermore, biological agents such as rituximab appear to be an effective treatment option for refractory cases of TTP related to systemic rheumatic disease, indicating an alternative therapeutic solution in persistent cases of this disorder.

  4. [Relationship between periodontal disease and rheumatoid arthritis].

    Science.gov (United States)

    Zhang, Dai-zun; Zhong, De-yu; Deng, Jing; Wang, Ji-bo

    2005-12-01

    To study a population of rheumatoid arthritis patients and determine the extent of periodontal disease in these patients, in order to investigate the relationship between periodontal disease and rheumatoid arthritis. The experimental group was composed of 70 patients with rheumatoid arthritis and the control group consisted of 70 age- and gender-matched individuals without rheumatoid arthritis. The relationship between periodontal status in rheumatoid arthritis and control groups as well as the relationship between periodontal status and rheumatological findings in patients were analyzed. The percentage of periodontal disease was statistically significant between experimental and control group (P 0.05). There were more number of periodontal disease index 5 or 6 in experimental group than in control group ( P Rheumatoid arthritis patients with moderate to severe bone loss had deeper degree of morning stiffness, erythrocyte sedimentation rate levels and serum C-reactive protein levels than patients with no or mild bone loss. Individuals with rheumatoid arthritis are more likely to experience periodontal disease compares to healthy subjects. They are also very likely to suffer from moderate to severe periodontitis.

  5. Three-dimensional morphological condylar and mandibular changes in a patient with juvenile idiopathic arthritis: interdisciplinary treatment

    Directory of Open Access Journals (Sweden)

    G. Farronato

    2014-11-01

    Full Text Available Temporomandibular joint (TMJ involvement is common but usually delayed in patients with juvenile idiopathic arthritis (JIA. We describe the case of a JIA patient with bilateral TMJ involvement, mandibular retrognathia, bone erosion, and severely restricted mouth opening. The use of cone beam computed tomography and a 3D diagnostic protocol in young patients with JIA provides reliable, accurate and precise quantitative data and images of the condylar structures and their dimensional relationships. Analgesics and conventional disease modifying antirheumatic drugs were ineffective, but interdisciplinary treatment with etanercept and a Herbst functional appliance improved functional TMJ movement and bone resorption.

  6. Sequential treatment of juvenile idiopathic arthritis%幼年特发性关节炎的序贯治疗

    Institute of Scientific and Technical Information of China (English)

    曾萍; 曾华松

    2014-01-01

    随着对幼年特发性关节炎治疗药物的不断认识和治疗方案的不断完善,幼年特发性关节炎患儿的治疗目标有了新的提升,科学的、个体化的序贯治疗方案的选择有利丁疾病的恢复.%With the improvement of the therapy and the drugs we known more results in a therapeutic ambitious goals of juvenile idiopathic arthritis.Selection of individualized sequential therapy is beneficial to the recovery of the disease.

  7. Osteoprotegerin in juvenile rheumatoid arthritis: cross talk between ...

    African Journals Online (AJOL)

    Ehab

    Osteoporosis in rheumatoid arthritis is characterized by a complexity of risk ... Methods: The study included 40 children and adolescents with JRA, as well as, 20 clinically ..... possible cause of this non significant relation. Also, none of the ...

  8. Diagnosis of juvenile idiopathic arthritis%幼年特发性关节炎的诊断

    Institute of Scientific and Technical Information of China (English)

    宋红梅

    2011-01-01

    Juvenile idiopathic arthritis (JIA), the most common rheumatologic disease in childhood, is characterized by arthritis beginning before the age of 16 years with symptoms persisting for more than 6 weeks with unknown causes.It was divided into seven subtypes by the International League of Associations for Rheumatology (ILAR): systemic arthritis, oligoarthritis, polyarthritis rheumatoid factor (RF) negative, polyarthritis RF positive,psoriatic arthritis, enthestis related arthritis (ERA), and undifferentiated arthritis.The appropriate auxiliary examinations should be chosen to differentiate it carefully from other causes that presented similar manifestations to JIA.Attention should be paid to assess the disease activity, prognosis and treatment outcomes.Meanwhile, complications needs to be watched.%幼年特发性关节炎(JIA)是指16岁以下儿童持续6周以上原因不明的关节炎.是儿童时期最常见的风湿性疾病.JIA按照国际风湿病学联盟的分类标准分为7个亚型,包括全身型、少关节炎型、多关节炎型类风湿因子(RF)阴性、多关节炎型RF阳性、银屑病件关节炎、附着点炎症相关的关节炎和分类不明的关节炎.应该选择适当的辅助检查与可能引起相似表现的其他原因相鉴别,并且应用适当的工具评价其活动程度,同时要注意并发症的诊断.

  9. A descriptive study of foot problems in children with juvenile rheumatoid arthritis (JRA).

    Science.gov (United States)

    Spraul, G; Koenning, G

    1994-09-01

    In this study, we evaluated the feet of 144 consecutive children with juvenile rheumatoid arthritis (JRA) during a routine outpatient visit to discover patterns of foot problems. We found that all but nine subjects had at least 1 of 21 foot problems, categorized as inflammation, limitation of motion, and abnormal alignment. Overall, pronated rearfoot and midfoot were observed in 73% and 72% of JRA patients, respectively. Additionally, 36% had splayfoot, whereas 35% of subjects had ankle limitation of motion. Other common foot problems included pronated forefoot, rearfoot and forefoot synovitis, forefoot limitation of motion, and toe valgus. Significant differences in the occurrence of various foot problems were observed among JRA onset/course subgroups and were influenced by both age and disease duration. Specifically, subjects with polyarticular JRA had more forefoot limitation and toe valgus, whereas subjects with pauciarticular JRA had pronated forefoot more often. Ankle limitation of motion, although unrelated to the JRA sub-group, was related to the duration of JRA. Subjects with longer disease histories also had toe valgus more often. Conversely, forefoot limitation of motion seemed to be more a function of age than of disease duration. These results indicate that foot problems are common in the JRA population, and they underscore the need for thorough evaluation and physical therapy management.

  10. High prevalence of methotrexate intolerance in juvenile idiopathic arthritis: development and validation of a methotrexate intolerance severity score

    NARCIS (Netherlands)

    Bulatovic, M.; Heijstek, M.W.; Verkaaik, M.; Dijkhuizen, E.H. van; Armbrust, W.; Hoppenreijs, E.P.A.H.; Kamphuis, S.; Kuis, W.; Egberts, T.C.; Sinnema, G.; Rademaker, C.M.A.; Wulffraat, N.M.

    2011-01-01

    OBJECTIVE: To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. METHODS: The MTX Intolerance Severity Score (MISS)

  11. High prevalence of methotrexate intolerance in juvenile idiopathic arthritis : development and validation of a methotrexate intolerance severity score

    NARCIS (Netherlands)

    Bulatović, Maja; Heijstek, Marloes W; Verkaaik, Marleen; van Dijkhuizen, E H Pieter; Armbrust, Wineke; Hoppenreijs, Esther P A; Kamphuis, Sylvia; Kuis, Wietse; Egberts, Toine C G; Sinnema, Gerben; Rademaker, Carin M A; Wulffraat, Nico M

    OBJECTIVE: To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. METHODS: The MTX Intolerance Severity Score (MISS)

  12. Predictors of poor response to methotrexate in polyarticular-course juvenile idiopathic arthritis: analysis of the PRINTO methotrexate trial

    NARCIS (Netherlands)

    Vilca, I.; Munitis, P.G.; Pistorio, A.; Ravelli, A.; Buoncompagni, A.; Bica, B.; Campos, L.; Häfner, R.; Hofer, M.; Ozen, S.; Huemer, C.; Bae, S.C.; Sztajnbok, F.; Arguedas, O.; Foeldvari, I.; Huppertz, H.I.; Gamir, M.L.; Magnusson, B.; Dressler, F.; Uziel, Y.; van Rossum, M.A.J.; Hollingworth, P.; Cawkwell, G.; Martini, A.; Ruperto, N.

    2010-01-01

    Objectives To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis. Methods Patients newly treated for 6 months with MTX enrolled in the Paediatric Rheumatology Internationa

  13. Juvenile idiopathic arthritis (JIA): a screening study to measure class II skeletal pattern, TMJ PDS and use of systemic corticosteroids.

    LENUS (Irish Health Repository)

    Mandall, Nicky A

    2010-03-01

    To screen patients with oligoarticular and polyarticular forms of Juvenile Idiopathic Arthritis (JIA) to determine (i) the severity of their class II skeletal pattern; (ii) temporomandibular joint signs and symptoms and (iii) use of systemic corticosteroids.

  14. Protocol for the Foot in Juvenile Idiopathic Arthritis trial (FiJIA: a randomised controlled trial of an integrated foot care programme for foot problems in JIA

    Directory of Open Access Journals (Sweden)

    Hendry Gordon J

    2009-06-01

    Full Text Available Abstract Background Foot and ankle problems are a common but relatively neglected manifestation of juvenile idiopathic arthritis. Studies of medical and non-medical interventions have shown that clinical outcome measures can be improved. However existing data has been drawn from small non-randomised clinical studies of single interventions that appear to under-represent the adult population suffering from juvenile idiopathic arthritis. To date, no evidence of combined therapies or integrated care for juvenile idiopathic arthritis patients with foot and ankle problems exists. Methods/design An exploratory phase II non-pharmacological randomised controlled trial where patients including young children, adolescents and adults with juvenile idiopathic arthritis and associated foot/ankle problems will be randomised to receive integrated podiatric care via a new foot care programme, or to receive standard podiatry care. Sixty patients (30 in each arm including children, adolescents and adults diagnosed with juvenile idiopathic arthritis who satisfy the inclusion and exclusion criteria will be recruited from 2 outpatient centres of paediatric and adult rheumatology respectively. Participants will be randomised by process of minimisation using the Minim software package. The primary outcome measure is the foot related impairment measured by the Juvenile Arthritis Disability Index questionnaire's impairment domain at 6 and 12 months, with secondary outcomes including disease activity score, foot deformity score, active/limited foot joint counts, spatio-temporal and plantar-pressure gait parameters, health related quality of life and semi-quantitative ultrasonography score for inflammatory foot lesions. The new foot care programme will comprise rapid assessment and investigation, targeted treatment, with detailed outcome assessment and follow-up at minimum intervals of 3 months. Data will be collected at baseline, 6 months and 12 months from baseline

  15. Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases

    Science.gov (United States)

    2010-06-25

    Rheumatoid Arthritis; Spondyloarthritis; Systemic Lupus Erythematosus (SLE); Dermatomyositis (DM); DMixed Connective Tissue Disease; Systemic Vasculitis; Systemic Sclerosis (SSc); Sjögren's Syndrome; Antiphospholipid Syndrome; Juvenile Idiopathic Arthritis; Juvenile SLE; Juvenile DM

  16. Dental and facial characteristics of patients with juvenile idiopathic arthritis Características dentárias e faciais de pacientes com artrite idiopática juvenil

    Directory of Open Access Journals (Sweden)

    Cynthia Savioli

    2004-01-01

    Full Text Available OBJECTIVE: It has been shown that the temporomandibular joint is frequently affected by juvenile idiopathic arthritis, and this degenerative disease, which may occur during facial growth, results in severe mandibular dysfunction. However, there are no studies that correlate oral health (tooth decay and gingival diseases and temporomandibular joint dysfunction in patients with juvenile idiopathic arthritis. The aim of this study is to evaluate the oral and facial characteristics of the patients with juvenile idiopathic arthritis treated in a large teaching hospital. METHOD: Thirty-six patients with juvenile idiopathic arthritis (26 female and 10 male underwent a systematic clinical evaluation of their dental, oral, and facial structures (DMFT index, plaque and gingival bleeding index, dental relationship, facial profile, and Helkimo's index. The control group was composed of 13 healthy children. RESULTS: The mean age of the patients with juvenile idiopathic arthritis was 10.8 years; convex facial profile was present in 12 juvenile idiopathic arthritis patients, and class II molar relation was present in 12 (P = .032. The indexes of plaque and gingival bleeding were significant in juvenile idiopathic arthritis patients with a higher number of superior limbs joints involved (P = .055. Anterior open bite (5 and temporomandibular joint noise (8 were present in the juvenile idiopathic arthritis group. Of the group in this sample, 94% (P = .017 had temporomandibular joint dysfunction, 80% had decreased mandibular opening (P = 0.0002, and mandibular mobility was severely impaired in 33% (P = .015. CONCLUSION: This study confirms that patients with juvenile idiopathic arthritis a have a high incidence of mandibular dysfunction that can be attributed to the direct effect of the disease in the temporomandibular joint and b have a higher incidence of gingival disease that can be considered a secondary effect of juvenile idiopathic arthritis on oral health

  17. An intra-articular ganglion cyst in a patient with juvenile idiopathic arthritis.

    Science.gov (United States)

    Deng, Donna Y; Yee, Keolamau; Burkhalter, William; Okimoto, Kelley Chinen; Kon, Kevin; Kurahara, David K

    2014-01-01

    We report an intra-articular ganglion cyst (IAGC) presenting as knee pain and a mass in a patient with longstanding Juvenile Idiopathic Arthritis (JIA). We could not find a similar case of an IAGC occurring in the knee of JIA patients in the literature. IAGC may need to be included as a possibility in patients with inflammatory arthritis with new-onset knee pain, especially in those with a palpable mass. MRI was useful in distinguishing IAGC from more worrisome causes of a knee mass. Orthopedic input was helpful in diagnosis and treatment. In addition, methotrexate therapy was effective in bringing about a long-lasting remission.

  18. PReS-FINAL-2148: Rheumates@work a cognitive behavioural internet based intervention promoting physical activity in children with juvenile idiopathic arthritis: Preliminary results of a randomized clinical trail

    NARCIS (Netherlands)

    Bos, J; Armbrust, W.; Geertzen, J.; Sauer, P.; Dijkstra, P.; Van Brussel, M.; Cappon, J.; Lelieveld, O.

    2013-01-01

    Introduction: Juvenile Idiopathic Arthritis (JIA) is a chronic disease in which periods of active inflammation alternate with periods of inactive disease in an unpredictable way. Although impairments are most pronounced in children with disease activity, deficits like fatigue, decreased physical act

  19. EVALUATION OF THICKNESS OF INTIMA-MEDIA COMPLEX OF COMMON CAROTID ARTERIES IN CHILDREN WITH JUVENILE ARTHRITIS AND SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    A.B. Sugak

    2010-01-01

    Full Text Available Rheumatic diseases in adults are associated with accelerated atherosclerosis, and its early signs can be stated by the thickening of intima-media complex of common carotid arteries (CCA. This symptom is detected during ultrasound examination in 49% of children with systemic lupus erythematosus, in 24% of patients with juvenile rheumatoid arthritis and in 13% of children with juvenile spondylarthritis. Besides, 36% of children with systemic lupus erythematosus and 17% — with systemic type of juvenile rheumatoid arthritis had structure changes of CCA wall. A dependence of these disorders on cholesterol and glucose levels in blood serum, overweight and Cushing syndrome, age, duration and activity of a disease, levels of ESR, C-reactive protein and white blood cells was not showed. Authors detected a correlation between the thickness of intima-media complex of CCA and hemostasis parameters.Key words: children, juvenile arthritis, systemic lupus erythematosus, intima-media complex, ultrasound diagnostics.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(2:64-69

  20. Rheumatoid arthritis: Disease or syndrome?

    Science.gov (United States)

    Stanich, Jessica A; Carter, John D; Whittum-Hudson, Judith; Hudson, Alan P

    2009-01-01

    Rheumatoid arthritis (RA) has been described in the medical literature for over two hundred years, but its etiology remains unknown. RA displays phenotypic heterogeneity, and it is a relatively prevalent clinical entity: it affects approximately 1% of the population, resulting in enormous pathologic sequelae. Earlier studies targeting the cause(s) of RA suggested potential infectious involvement, whereas more recent reports have focused on a genetic origin of the disease. However, neither infection nor genetics, nor any other single factor is currently accepted as causative of RA. In this article we review studies relating to the etiology of RA, and those of several related matters, and we conclude that the literature indeed does provide insight into the causes underlying the initiation of RA pathogenesis. Briefly, given the remarkable phenotypic variation of RA, especially in its early stages, as well as a number of other characteristics of the condition, we contend that RA is not a discrete clinical entity with a single etiological source. Rather, we argue that it represents a common clinical endpoint for various starting points, each of which is largely guided by as yet poorly understood aspects of the genetic background of the affected individual. Adoption of this alternative view of the origin of RA will have significant consequences for future research and for development of new therapeutic interventions for this burdensome condition.

  1. Early juvenile arthritis – results of two-year follow up

    Directory of Open Access Journals (Sweden)

    S O Salugina

    2009-01-01

    Full Text Available Objective. To study clinical and laboratory manifestations of different variants of juvenile arthritis (JA at the disease onset and during prospective two-year follow up. Material and methods. The study was performed as a part of Institute of Rheumatology early arthritis examination program RADIKAL. 130 pts with early JA (60,7% - girls with disease duration from 2 weeks to 6 months (mean 2,9±1,6 months aged 1,5- 16 years (mean 7,9±5,0 years were included. 13 (10% pts had systemic, 45 (34,6% – polyarticular and 72 (55,4% – olygoarticular variant of JA. General state, joint status, systemic and organ manifestation as well as immunological parameters (ANF, RF, disease activity, functional class (by Steinbrocker and CHAQ were assessed at baseline, and after 6, 12, 24 months of follow up. Results. Oligoarticular variant prevailed at onset and after 2 years (57,6%-55,6%. Systemic features were noted in reduced form as single manifestations. Morning stiffness was absent in half of children and lasted more than 1 hour in 16,4% of pts. After 2 years number of pts with morning stiffness significantly decreased and its duration diminished. Rheumatoid nodules appeared in 1 pt after 1 year. Uveitis developed in 7 children (5,3% and to the end of follow up it appeared in 2 more pts. Most of pts had minimal or moderate functional disability (FK 1,2 and CHAQ 0,1-1,5 during follow up. Disease activity at onset did not exceed 1 or 2 stage (80,2% and after 2 years the disease was not active in half of pts. To the end of follow up remission was achieved in 59% of pts, more often in those who received disease modifying anti-rheumatic drugs. In 23,2% of pts mostly in those with polyarthritis JA continued to recur independently on treatment. Conclusion. Timely administered complex therapy hampered disease progression, induced remission and improved quality of life in most children with JA. Pts with olygoarthritis had most favorable course of the disease. Pts

  2. Innovations and Challenges by Applying Sublingual Laser Blood Irradiation in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Laura Marinela Ailioaie

    2014-01-01

    Full Text Available Sublingual laser blood irradiation (SLBI was applied into a randomized, single-blind, placebo-controlled study in juvenile idiopathic arthritis (JIA, aimed at inducing disease remission. 105 children with JIA, without an adequate response to classical treatment, were administrated a disease modifying drug (Methotrexate and were randomly assigned to three groups. Group I (36 patients received SLBI with the Weberneedle Lasershower Mouth Applicator with three wavelengths (635 nm, 536 nm, and 405 nm, 5 mW maximum output power each, in continuous mode, simultaneously, for 20 minutes daily, 7 successive sessions per month, repeated every 7 weeks, for three times. Group II (36 patients received placebo SLBI. Group III (33 patients received only treatment with Methotrexate. Evaluation was performed using American College of Rheumatology Pediatric criteria (ACR Pedi at study enrollment and at 8, 16, 24, and 48 weeks. At the end of study, there was an improvement of the ACR Pedi 30 by 86.11% in SLBI group compared to only 61.11% in Group II, respectively, and 60.6% in Group III (P=0.001, with significant statistical differences. SLBI has reduced the pain, lowered the number of articulations with movement limitation, increased the quality of life, and made it possible to avoid the administration of biological agents.

  3. Temporomandibular joint involvement in juvenile idiopathic arthritis: clinical predictors of magnetic resonance imaging signs

    Energy Technology Data Exchange (ETDEWEB)

    Argyropoulou, Maria I.; Margariti, Persefoni N.; Astrakas, Loukas; Kosta, Paraskevi [Medical School University of Ioannina, Department of Radiology, Ioannina (Greece); Karali, Aikaterini; Alfandaki, Sapfo; Siamopoulou, Antigoni [University of Ioannina, Department of Child Health, Medical School, Ioannina (Greece)

    2009-03-15

    The aim of the study was to define clinical predictors of magnetic resonance imaging (MRI) findings of temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA). Forty-six patients, aged 2.08-36.7 years, with JIA (oligoartitular 18, polyarticular 17, systemic type 11) were examined with standard plain and contrast-enhanced sequences. Of 88 TMJs examined, an abnormal condyle was observed in 32%, flattened articular eminence in 27%, flattened articular disk in 17%, intra-articular fluid in 10%, enhancing pannus in 45% and restricted condylar motion in 9%. Logistic regression analysis revealed that for abnormal condyle and flattened articular eminence, independent predictors were type of JIA (P < 0.015), age at onset (P < 0.038), and duration of disease activity (P < 0.001). Plots of the logistic regression models showed that TMJ involvement approached certainty for systemic sooner than for the other JIA types. Pannus was present with probability >0.5 when the disease started before 4 years of age. In conclusion, the systemic type of JIA, young age at onset and long duration of activity are risk factors for TMJ damage. MRI of the TMJ should be performed in patients who are less than 4 years of age at the onset of JIA, and in those with the systemic type, whatever the age of onset. (orig.)

  4. The early magnetic resonance imaging features of the knee in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, Karl; Wittkop, Berndt; Haigh, Fiona; Ryder, Clive; Gardner-Medwin, Janet M

    2002-06-01

    AIMS: Early diagnosis of juvenile idiopathic arthritis (JIA) facilitates earlier more aggressive therapy, and improved outcome. Recognition of the features of early, untreated JIA on magnetic resonance imaging (MRI) will improve disease detection and expedite treatment. This study aims to highlight the relevant MRI features. METHODS: MRI examinations of the knee joint were performed on 11 children with clinically confirmed, early, untreated JIA. The MRI images were obtained at a mean of 2 months after symptom onset and independently evaluated by two consultant paediatric radiologists. RESULTS: Abnormalities were found on all MRI examinations. Synovial hypertrophy, joint effusions, popliteal lymph nodes and soft tissue swelling were present in all patients. Gadolinium DTPA enhancement improved the detection of synovial hyperplasia. Metaphyseal splaying and condylar overgrowth were seen in five cases (41%), oedema of the lateral collateral ligament in two cases (18%) and superficial cartilage thinning in one case. Bony erosions and deep cartilage destruction were not demonstrated. CONCLUSION: MRI of the knee joint identifies early joint changes which are distinct from those in later disease. The presence of these features should alert the radiologist to the possible diagnosis of JIA and post gadolinium DTPA sequences should be performed. Gadolinium DPTA enhancement increases the sensitivity for the detection of inflammatory changes in JIA. Johnson, K. et al. (2002)

  5. Intravenous Laser Blood Irradiation and Tocilizumab in a Patient with Juvenile Arthritis

    Directory of Open Access Journals (Sweden)

    Dragos Andrei Chiran

    2014-01-01

    Full Text Available This study presents effects of intravenous laser blood irradiation (ILBI in a transient immunodeficiency patient with juvenile idiopathic arthritis (JIA treated with an interleukin-6 receptor inhibitor (Tocilizumab. Biological agents induce JIA remission, but some patients do not respond favorably to this final therapeutic line of defense. ILBI was performed in a 16-year-old male patient, with JIA and transient immunodeficiency. When ILBI was introduced, the patient was receiving disease-modifying drugs, steroids, tocilizumab, and physical therapy. Because the disease was not well controlled, ILBI was applied in addition to other ongoing therapies. The patient underwent 1 session daily, and 10 successive sessions per month, repeated every 3 months, for 7 months. Patient evaluation was performed before ILBI was started and at 3, 6, 9, and 12 months after ILBI initiation, using the ACR Pediatric response. The outcome was evaluated using Pediatric 50, 70, and 90 responses and compared to initial status, after 3, 6, 9, and 12 months. At the end of study, the titre of IgA and IgG levels returned to normal. Synergistic anti-inflammatory effect of ILBI was evident, if applied additionally in combination with tocilizumab, in a patient with a therapy-resistant severe form of JIA and related subacute transient immunodeficiency.

  6. Rheumatoid arthritis and periodontal disease.

    Science.gov (United States)

    Berthelot, Jean-Marie; Le Goff, Benoît

    2010-12-01

    The prevalence of periodontal disease has increased two-fold among patients with rheumatoid arthritis (RA) compared to the general population. This increased prevalence is unrelated to secondary Sjögren's syndrome but instead reflects shared pathogenic mechanisms, including an increased prevalence of the shared epitope HLA-DRB1-04; exacerbated T-cell responsiveness with high tissue levels of IL-17; exaggerated B-cell responses, with plasma cells being the predominant cell type found within gingival tissue affected with periodontitis and B cells being twice as numerous as T cells; RANK overexpression; and an increase in the ratio of RANK-L over osteoprotegerin with a high level of RANK-L expression on gingival B cells, most notably those capable of recognizing Porphyromonas gingivalis. Other factors conducive to periodontitis include smoking and infection with the Epstein-Barr virus or cytomegalovirus, which act by promoting the growth of organisms such as P. gingivalis, whose DNA is often found in synovial tissue from RA patients. P. gingivalis produces the enzyme peptidylarginine deiminase that induces citrullination of various autoantigens, and levels of anti-CCP antibodies are considerably higher in RA patients with than without periodontal disease, suggesting that periodontitis may contribute to the pathogenesis of RA. Further support for this hypothesis comes from evidence that other antigens involved in RA, such as HC-gp39, are also present in gingival tissue. TNFα antagonists slow alveolar resorption but may perpetuate infection of periodontal pockets. Therefore, rheumatology patients, including those taking biotherapies, are likely to benefit from increased referral to dental care (e.g., scaling, root planing and, if needed, dental surgery), particularly as periodontitis is also associated with an increased risk of premature atheroma. Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  7. Patterns of compensation of functional deficits of the knee joint in patients with juvenile idiopathic arthritis

    Science.gov (United States)

    Księżopolska-Orłowska, Krystyna

    2015-01-01

    Objectives Juvenile idiopathic arthritis (JIA) is a group of pathological syndromes of unknown aetiology, observed at the developmental age. Their common feature is sustained chronic arthritis with flares and remissions. Clinical signs and symptoms include joint pain, periarticular tissue oedema or articular exudate, frequently associated with hypertrophy of the synovial membrane. The intra- and extra-articular structural damage impairs the motion range and smoothness. The disease process may involve any joint. The knee joint is the most frequently affected in oligo- and polyarthritis. The aim of the study was to determine a direct correlation between disorders of knee joint function and the change in the range of motion of the ankle and hip joints of both lower extremities, and the so-called indirect impact of these changes on patients’ posture. Material and methods The study included 36 JIA patients and 56 healthy controls aged 8–16 years. The evaluation was based on physical examination. Results The results showed differences in the values of quality and range of motion between patients and controls. In the patient group pes planovalgus was more frequently associated with knee joint dysfunction along with the inherent restriction of dorsal flexion of the foot. Shortening of the iliotibial band, increased outward rotation of the right lower extremity with enlarged joint contour and augmented inward rotation of the contralateral healthy extremity all proved significant. Changes in motion range in the joints below and over the knee were associated with alterations of antero-posterior spine curvatures and vertebral rotation along the long spinal axis. Based on the results, the mechanism of the compensation is outlined. Conclusions The observed differences in the range and quality of motion in the ankle, hip and spinal joints between patients and healthy children provide evidence that dysfunction of the knee joint affects the function of the other above

  8. Analysis of juvenile idiopathic arthritis associated uveitis in India over the last 16 years

    Directory of Open Access Journals (Sweden)

    Sudharshan S

    2007-01-01

    Full Text Available Aim: Juvenile idiopathic arthritis (JIA associated uveitis is one of the most common causes of visual morbidity in children. We report the systemic, clinical and investigational features of a cohort of all cases of JIA associated uveitis seen at our referral uveitis clinic between 1988 and 2004. Study Design: Retrospective case series Materials and Methods: All patients of JIA seen at the uveitis clinic of tertiary eye care hospital, between 1988 and 2004 with minimum follow up of 3 months were included. Complete history and ophthalmic evaluation and findings on each visit were noted. Ocular complications were identified and recorded. Results of laboratory investigations and diagnostic as well as therapeutic procedures were analyzed. A rheumatologist managed systemic status. Results: There were 40 patients (64 eyes with JIA. Thirty four patients (85% had pauciarticular type and 6 patients (15% had polyarticular type of JIA. Complicated cataract and band shaped keratopathy were seen in 38 eyes (63% and 37 eyes (62% respectively. Twenty-two patients (17 bilateral and 5 unilateral were treated with immunosuppressives and in 19 of these patients, the disease went into remission. Twenty-three eyes (38% had improvement in visual acuity while in 27 eyes (45%, the vision remained stable and in 10 eyes (17%, vision deteriorated despite therapy. Conclusion: In India, JIA associated uveitis commonly presented in pauciarticular type with preponderance in males. Rheumatoid arthritis factor and anti nuclear antibodies were not as common as compared to the western population. Among long-term treatment options, immunosuppressives are a better choice. Ocular surgery was performed when mandatory for visual rehabilitation.

  9. Long-term treatment with rituximab in severe juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Miserocchi, Elisabetta; Modorati, Giulio; Berchicci, Luigi; Pontikaki, Irene; Meroni, Pierluigi; Gerloni, Valeria

    2016-06-01

    To evaluate retrospectively the long-term efficacy of rituximab in patients with severe juvenile idiopathic arthritis (JIA)-associated uveitis. Eight patients (15 eyes) with severe and longstanding JIA uveitis, who had an inadequate response in controlling uveitis to one or more biologic agents including tumour necrosis factor blockers and abatacept, received rituximab therapy. Rituximab was given at a dose of 1000 mg per infusion on days 1 and 15 and then every 6 months. Clinical responses to treatment, including decrease in uveitis activity, visual acuity changes, reduction of concomitant local and systemic corticosteroid and/or immunosuppressants, and occurrence of adverse events, were assessed. Eight patients with a mean±SD age of 22.8±5.5 years were treated. The mean ocular disease duration was 17.7 years; the mean±SD follow-up time on rituximab was 44.75±4.9 months; and the mean number of rituximab infusions received was 8.75 (range 6-12). All patients achieved complete control of uveitis, but in two patients rituximab was discontinued due to inefficacy in treating arthritis. The decrease in uveitis activity was evident 4-5 months after the first infusion. Systemic corticosteroids and immunosuppressants used in association with rituximab were discontinued in five patients at the end of follow-up. None of the patients experienced visual worsening during the follow-up. No drug-related complications were encountered. Rituximab may be a promising effective treatment option for refractory uveitis associated with JIA leading to long-term quiescence of uveitis, particularly for patients who have not previously responded to other biologic therapies. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  10. Patterns of compensation of functional deficits of the knee joint in patients with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Beata Żuk

    2015-09-01

    Full Text Available Objectives: Juvenile idiopathic arthritis (JIA is a group of pathological syndromes of unknown aetiology, observed at the developmental age. Their common feature is sustained chronic arthritis with flares and remissions. Clinical signs and symptoms include joint pain, periarticular tissue oedema or articular exudate, frequently associated with hypertrophy of the synovial membrane. The intra- and extra-articular structural damage impairs the motion range and smoothness. The disease process may involve any joint. The knee joint is the most frequently affected in oligo- and polyarthritis. The aim of the study was to determine a direct correlation between disorders of knee joint function and the change in the range of motion of the ankle and hip joints of both lower extremities, and the so--called indirect impact of these changes on patients’ posture. Material and methods : The study included 36 JIA patients and 56 healthy controls aged 8–16 years. The evaluation was based on physical examination. Results : The results showed differences in the values of quality and range of motion between patients and controls. In the patient group pes planovalgus was more frequently associated with knee joint dysfunction along with the inherent restriction of dorsal flexion of the foot. Shortening of the iliotibial band, increased outward rotation of the right lower extremity with enlarged joint contour and augmented inward rotation of the contralateral healthy extremity all proved significant. Changes in motion range in the joints below and over the knee were associated with alterations of antero-posterior spine curvatures and vertebral rotation along the long spinal axis. Based on the results, the mechanism of the compensation is outlined. Conclusions : The observed differences in the range and quality of motion in the ankle, hip and spinal joints between patients and healthy children provide evidence that dysfunction of the knee joint affects the function

  11. Superior oblique tendon (Brown’s syndrome as the presenting finding in childhood onset HLA-B27-related enthesitis and juvenile idiopathic oligoarticular arthritis

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    C. Pham

    2014-11-01

    Full Text Available We report two patients who presented with Brown’s syndrome. The first is a 7-year-old boy who at the time of his diagnosis was also found to have enthesitis and HLA-B27 positivity. The second patient was diagnosed with bilateral Brown’s syndrome at 13 months of age. At age 7 she developed a persistent oligoarticular arthritis and unilateral anterior iritis consistent with the oligoarticular Juvenile Idiopatic Arthritis (JIA phenotype. These cases highlight ophthalmologic findings and diagnostic considerations with respect to Brown’s syndrome and associated childhood onset rheumatologic disease.

  12. Clinical presentation of juvenile Huntington disease

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    Ruocco Heloísa H.

    2006-01-01

    Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

  13. Joint functional disability in children of early age with olygoarticular juvenile arthritis

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    T A Shelepina

    2008-01-01

    Full Text Available Objective. To study functional status, character of functional disturbances and possibilities of their correction in children of early age with juvenile arthritis (JA. Material and methods. 42 pts with oligoarticular (at presentation variant of JA with onset before 4 years of age admitted to pediatric department of the Institute of rheumatology of RAMS were included. Mean age of children at the disease onset was 22,4+10 months (from 9 to 46 months, mean disease duration at the first hospitalization — 9,5±5,5 months. Functional status and daily activities limitations (decrease of moving capacity and hand performance were assessed at the beginning of the disease, at admission to the institute and at discharge after drug and rehabilitation treatment. Results. At the disease onset 40 pts (95,2% had knee joint damage (one sided in 28 — 66,6%. 30 from these 40 pts (75% had flexion deformity and daily activities limitations and two pts had anlde joint damage. 12 pts (28,6% received courses of nonsteroidal anti-inflammatory drugs (NSA1D and 5 pts (11,9% — disease modifying anti-rheumatic drugs (DMARD before admission. All pts had active joint syndrome at admission. Daily activities limitations were present in 41 pts (97,6%. DMARD were prescribed to 41 pts (with glucocorticoids (GC in 12 cases. Intra-articular injections of GC were performed in 15 pts. Individual exercise therapy (passive joint movement was applied in all pts, postural treatment — in 41 (97,6%. One pt received exercise therapy in a group. Splints were done for 6 pts. Stepped correction in plaster was performed in 4 from them. In all pts after treatment arthritis subsided and functional status improved. In 18 (42,8% from them function of damaged joint was fully restored and in 19 (45,2% — improved. After several stages of hospital treatment deformation was corrected and function restored in 4 (9,5% pts Conclusion. Клее joints are frequently involved in children of early age

  14. Isolated Arthritis of the Temporomandibular Joint as the Initial Manifestation of Juvenile Idiopathic Arthritis

    DEFF Research Database (Denmark)

    Hügle, Boris; Spiegel, Lynn; Hotte, Julia

    2017-01-01

    . Demographic and clinical data were analyzed using descriptive statistics. RESULTS: Fifty-five patients were identified (65% bilateral presentation). Six patients developed arthritis in other joints (median time 6 mos); 4 patients developed uveitis, all prior to arthritis. At last followup, 9% were still...

  15. What does it mean to grow up with juvenile idiopathic arthritis?

    OpenAIRE

    2010-01-01

    Allied Health Professionals in Rheumatology (AHP) Abstracts 27. Paediatric rheumatology EULAR2010-AHP-2198 WHAT DOES IT MEAN TO GROW UP WITH JUVENILE IDIOPATHIC ARTHRITIS? D. Hilderson 1, 2,*, L. Eyckmans 1, R. Westhovens 3, C. Wouters 4, P. Moons 2 1Department of Paediatrics, 3Rheumatology, Department of Musculoskeletal Sciences, 4Department of Paediatric Rheumatology, University Hospitals Leuven, 2Centre for Health Services and Nursing Research, Katholieke Universiteit ...

  16. Therapeutic exercises in the combination therapy of patients with juvenile chronic arthritis

    Directory of Open Access Journals (Sweden)

    Tatyana Andreyevna Shelepina

    2013-01-01

    Full Text Available The paper analyzes the role of therapeutic exercises in the therapy of patients with juvenile chronic arthritis. The author discusses the time of, indications for, and contraindications to their use and states her belief that it is advisable for a rheumatologist to prescribe this therapy option. The data available in the literature and the author’s data on the efficiency of this therapy are given.

  17. [Two pairs of brothers with juvenile idiopathic arthritis (JIA): case reports].

    Science.gov (United States)

    Robazzi, Teresa Cristina M V; Rios, Gabriela; Castro, Catarina

    2015-01-01

    This is a case report of juvenile idiopathic arthritis (JIA) in two pairs of brothers followed in the department of pediatric rheumatology, Universidade Federal da Bahia. Genetic involvement in JIA pathogenesis is clear and the risk of recurrence among siblings supports this contribution. An important landmark of this discovery involves the acknowledgment of major histocompatibility complex (MHC) polymorphism contribution to JIA development susceptibility. Despite many advances, the numerous available studies cannot explain several implicit mechanisms in JIA pathogenesis yet.

  18. Emotion Regulation Predicts Pain and Functioning in Children With Juvenile Idiopathic Arthritis: An Electronic Diary Study

    OpenAIRE

    Connelly, Mark; Bromberg, Maggie H.; Anthony, Kelly K.; Gil, Karen M.; Franks, Lindsey; Schanberg, Laura E

    2011-01-01

    Objectives This study utilized e-diaries to evaluate whether components of emotion regulation predict daily pain and function in children with juvenile idiopathic arthritis (JIA). Methods 43 children ages 8–17 years and their caregivers provided baseline reports of child emotion regulation. Children then completed thrice daily e-diary assessments of emotion, pain, and activity involvement for 28 days. E-diary ratings of negative and positive emotions were used to calculate emotion variability...

  19. Treatment of knee flexion contracture in patients with chronic juvenile arthritis: A case report

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    Matijević Radmila

    2006-01-01

    Full Text Available Introduction. Knee flexion contractures are common after-effects of juvenile arthritis. Treatment is usually conservative and may include physical therapy and kinesitherapy. Surgical treatment, particularly of the soft parts, indicated for contractures resistant to conservative treatment, helps to correct the deformity, maintain movements, and relieves pain. Intensive postoperative physiotherapy is of special importance. Case report. A 23-year-old female patient with chronic juvenile arthritis since the age of one was admitted for treatment of flexion con­tractures in both knees, muscle hypotrophy, loss of strength and gait disability. The patient underwent arthroscopic synovectomy. The operation was first performed on the right and after 3 mouths on the left knee. The pre operative range of motion in the rigth knee was 30°-70° and in the left 40°-80°. The patient underwent intensive physical therapy to reduce postoperative swelling of knees and firstly passive and then active kinesitherapy. Nine months after the first surgery and six months after the second, the range of motion in the right knee was 0°-100° and in the left 0°-105°. The strength of tested muscles was increased and gait was improved. Conclusion. Management and rehabilitation of patients with chronic juvenile arthritis include maintenance or improvement in position and function of joints that is achieved with synovectomy. The results depend on combined interdisciplinary rehabilitation, well-experienced staff, and pre- and post-operative physiotherapy as well as kinesitherapy. Arthroscopic synovectomy has many advantages and we believe that it was a better solution than open capsulosynovectomy in this patient with chronic juvenile arthritis of the knee. .

  20. Overlapping juvenile idiopathic arthritis and systemic lupus erythematosus: a case report

    OpenAIRE

    Bazsó, Anna; Sevcic, Krisztina; Orbán, Ilonka; Poór, Gyula; Balogh, Zsolt; Kiss, Emese

    2010-01-01

    Abstract Hereby, we report the case of a 12-year-old girl developing oligoarthritis and progressing into a polyarticular form. Rheumatoid factor was positive, and juvenile idiopathic arthritis (JIA) was diagnosed. After a poor response to DMARDs, an anti-TNF agent (infliximab) was initiated, but to be discontinued due to an allergic reaction. The same complication was observed with the fully human derivative, adalimumab. At the age of 22, the patient presented septicemia with sever...

  1. Recommendations for the Use of Ultrasound and Magnetic Resonance in Patients With Spondyloarthritis, Including Psoriatic Arthritis, and Patients With Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Uson, Jacqueline; Loza, Estibaliz; Möller, Ingrid; Acebes, Carlos; Andreu, Jose Luis; Batlle, Enrique; Bueno, Ángel; Collado, Paz; Fernández-Gallardo, Juan Manuel; González, Carlos; Jiménez Palop, Mercedes; Lisbona, María Pilar; Macarrón, Pilar; Maymó, Joan; Narváez, Jose Antonio; Navarro-Compán, Victoria; Sanz, Jesús; Rosario, M Piedad; Vicente, Esther; Naredo, Esperanza

    2016-10-27

    To develop evidence-based recommendations on the use of ultrasound (US) and magnetic resonance imaging in patients with spondyloarthritis, including psoriatic arthritis, and juvenile idiopathic arthritis. Recommendations were generated following a nominal group technique. A panel of experts (15 rheumatologists and 3 radiologists) was established in the first panel meeting to define the scope and purpose of the consensus document, as well as chapters, potential recommendations and systematic literature reviews (we used and updated those from previous EULAR documents). A first draft of recommendations and text was generated. Then, an electronic Delphi process (2 rounds) was carried out. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70% of participants voted≥7. The level of evidence and grade or recommendation was assessed using the Oxford Centre for Evidence Based Medicine levels of evidence. The full text was circulated and reviewed by the panel. The consensus was coordinated by an expert methodologist. A total of 12 recommendations were proposed for each disease. They include, along with explanations of the validity of US and magnetic resonance imaging regarding inflammation and damage detection, diagnosis, prediction (structural damage progression, flare, treatment response, etc.), monitoring and the use of US guided injections/biopsies. These recommendations will help clinicians use US and magnetic resonance imaging in patients with spondyloarthritis and juvenile idiopathic arthritis. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  2. Case Report: Juvenile Tophaceous Gout

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    Seyma Gunes

    2016-01-01

    Full Text Available Gout is a metabolic disease that manifests as recurrent arthritis. Its incidance increases with age. Clinical findings include recurrent acute arthritis, tophus at joints and tissues, uricacid stones and gouty nephropathy. Tophi is a late period complication of arthritis. In this casereport we presented  a patient with early-onset juvenile tophaceous gout.

  3. Juvenile idiopathic arthritis%幼年特发性关节炎分类

    Institute of Scientific and Technical Information of China (English)

    何晓琥

    2003-01-01

    @@ 为了便于对儿童时期关节炎的免疫遗传学、流行病学、转归和治疗方案实施等方面进行国际间协作研究,国际风湿病学联盟儿科常委专家组举行了三次会议进行讨论,将儿童时期不明原因的关节肿胀持续6周以上这类关节炎的分类统一起来,定名为幼年特发性关节炎(juvenile idiopathic arthritis, JIA),从而取代了原有的美国应用的幼年类风湿关节炎(juvenile rheumatoid arthritis, JRA)和欧洲应用的幼年慢性关节炎(juvenile chronic arthritis, JCA)这两个分类标准.现将JIA分类标准介绍如下(此标准于1994年制订,1997年修改,2001年正式通过).

  4. MRI assessment of tenosynovitis in children with juvenile idiopathic arthritis: inter- and intra-observer variability

    Energy Technology Data Exchange (ETDEWEB)

    Lambot, Karen; Brunelle, Francis [Hopital Necker-Enfants Malades, Department of Paediatric Radiology, Paris (France); Boavida, Peter [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Damasio, Maria Beatrice [Ospedale Pediatrico Gaslini, Department of Radiology, Genoa (Italy); Tanturri de Horatio, Laura; Barbuti, Domenico [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Desgranges, Marie; Bader-Meunier, Brigitte; Quartier, Pierre [Hopital Necker-Enfants Malades, Department of Paediatric Immunology, Hematology and Rheumatology, APHP French Reference Center ' ' Arthrites juveniles' ' , Paris (France); Malattia, Clara [University of Genoa, Department of Paediatrics, Genoa (Italy); Bracaglia, Claudia [Ospedale Pediatrico Bambino Gesu, Department of Paediatrics, Rome (Italy); Ording Mueller, Lil-Sofie [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); University Hospital of North Norway, Department of Radiology, Tromsoe (Norway); Elie, Caroline [Paris Descartes University, Department of Biostatistics, Hopital Necker-Enfants Malades, Paris (France); Rosendahl, Karen [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Haukeland University Hospital, Department of Radiology, Bergen (Norway)

    2013-07-15

    There is sparse knowledge about grading tenosynovitis using MRI. The purpose of this study was to assess the reliability of a tenosynovitis MRI scoring system in juvenile idiopathic arthritis. Children with juvenile idiopathic arthritis and wrist involvement were enrolled in two paediatric centres, from October 2006 to January 2010. The extensor (compartments II, IV and VI) and flexor tendons were assessed for the presence of tenosynovitis on T1-weighted postcontrast fat-saturated MR images and were scored from 0 (normal) to 2 (moderate to severe) by two observers independently. Intra- and interobserver agreement was assessed. Ninety children (age range: 5-18.5 years) were included, of whom 34 had tenosynovitis involving extensors and 28 had tenosynovitis involving flexors. A total of 360 tendon areas were analysed, of which 114 had tenosynovitis (86/270 extensors and 28/90 flexors). Intra-reader 1 agreement was excellent for the extensors (k = 0.82-0.91) and for the flexors (k = 0.85); intra-reader 2 agreement was moderate to good for the extensors (k = 0.51-0.72) and good for the flexors (k = 0.64). Inter-reader agreement was good for the extensors (k = 0.69-0.73) and moderate for the flexors (k = 0.49). The proposed MRI scoring system for the assessment of wrist tenosynovitis in juvenile idiopathic arthritis appears feasible with an observer agreement sufficient for clinical use. (orig.)

  5. High rates of unsuccessful transfer to adult care among young adults with juvenile idiopathic arthritis

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    Duffy Ciarán M

    2010-01-01

    Full Text Available Abstract Background This study aimed to describe the proportion of patients with juvenile idiopathic arthritis (JIA who had experienced an unsuccessful transfer from a pediatric rheumatology team to an adult rheumatologist and to compare the characteristics of those who achieved successful transfer to those who did not. Methods We conducted a systematic chart review of all patients with JIA who attended their final Montreal Children's Hospital JIA clinic appointment between 1992 and 2005. We tracked these patients for the two years after transfer to an adult rheumatologist. We then compared characteristics of patients with successful and unsuccessful transfers of care. Variables pertaining to disease characteristics, disease severity and psychosocial factors were examined. Univariate analyses were performed to determine if any single factor was associated with the outcome of unsuccessful transfer of care. Results 52% of patients fulfilled our criteria for unsuccessful transfer. Of the variables tested, an active joint count (AJC of zero at last visit was associated with the outcome of unsuccessful transfer (OR = 2.67 (CI 1.16-6.16; p = 0.0199. Conclusions Despite the presence of a coordinated process of transfer from pediatric to adult health care for the majority of the patients in this study, there was a high rate of unsuccessful transfer and/or sustained follow up which is disheartening. We found that patients with less active disease at the time of transfer, as indicated by a lower AJC, were more likely to be lost to follow up. Recent literature suggests that even in the least severe categories of JIA, 50% of patients persist with active disease into adulthood. Thus educating all JIA patients about the possibility of disease flare in adulthood may improve their adherence to recommendations for sustained follow-up in the adult milieu. This may lead to improvement of longitudinal outcomes for all JIA patients.

  6. A case report of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Anita Choudhary

    2017-09-01

    Full Text Available Huntington disease (HD is a progressive neurodegenerative disorder, characterized by autosomal dominant inheritance, movement disorder, dementia, and behavioural disturbances. It is caused by a mutation in IT15 gene on chromosome 4p16.3, which leads to unstable CAG trinucleotide repeat expansion. The onset of juvenile HD occurs before the 2nd decade of life and comprises approximately 10% of total HD patients. Juvenile HD differs in symptomatology and is usually transmitted from paternal side with genetic anticipation phenomenon. Magnetic resonance imaging (MRI of the brain shows specific changes of early affection of caudate nucleus and putamen. Multidisciplinary approach with symptomatic treatment of specific symptoms is the current available management. Gene editing and gene silencing treatment are under trial. Hereby, we introduce a case of an 8-year-old boy, who presented with typical symptoms of juvenile HD, positive family history with genetic anticipation phenomenon and characteristic MRI findings.

  7. Radiographic temporomandibular joint abnormality in adults with micrognathia and juvenile rheumatoid arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Larheim, T.A.; Haanaes, H.R.; Dale, K. (Oslo Univ. (Norway))

    1981-01-01

    Radiographic findings of the upper and lower jaw bone of 20 adult patients with micrognathia, bird face, and juvenile rheumatoid arthritis are reported. In all patients a symmetrically underdeveloped mandible with the chin posteriorly positioned was found at cephalometry. Arthritic lesion of the temporomandibular joint, mostly symmetric, with limitation of movement and secondary arthrosis, was observed in all patients. Complete absence of the mandibular head was frequent (75%). The fossa was generally flat, probably due to growth disturbance of the tubercle. Abnormal anterior position of the mandibular head occurred in almost half of the patients. The degree of mandibular growth disturbance seemed to be correlated to the severity of the arthritis, indicating the arthritis to be a causal mechanism of micrognathia.

  8. Complications of systemic juvenile idiopathic arthritis: risk factors and management recommendations.

    Science.gov (United States)

    Woerner, Andreas; von Scheven-Gête, Annette; Cimaz, Rolando; Hofer, Michaël

    2015-05-01

    Systemic juvenile idiopathic arthritis (SJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. Systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that SJIA is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of SJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation, continue to be a major issue in SJIA patients' care. Translational research leading to a profound understanding of the cytokine crosstalk in SJIA and the identification of risk factors for SJIA complications will help to improve long-term outcome.

  9. Biological therapies for the treatment of juvenile idiopathic arthritis: Lessons from the adult and pediatric experiences.

    Science.gov (United States)

    Stoll, Matthew L; Gotte, Alisa C

    2008-06-01

    Biologics have advanced the therapy of adult and pediatric arthritis. They have been linked to rare serious adverse outcomes, but the actual risk of these events is controversial in adults, and largely unknown in pediatrics. Because of the paucity of safety and efficacy data in children, pediatric rheumatologists often rely on the adult literature. Herein, we reviewed the adult and pediatric literature on five classes of medicines: Tumor necrosis factor (TNF) inhibitors, anakinra, rituximab, abatacept, and tocilizumab. For efficacy, we reviewed randomized controlled studies in adults, but did include lesser qualities of evidence for pediatrics. For safety, we utilized prospective and retrospective studies, rarely including reports from other inflammatory conditions. The review included studies on rheumatoid arthritis and spondyloarthritis, as well as juvenile idiopathic arthritis. Overall, we found that the TNF inhibitors have generally been found safe and effective in adult and pediatric use, although risks of infections and other adverse events are discussed. Anakinra, rituximab, abatacept, and tocilizumab have also shown positive results in adult trials, but there is minimal pediatric data published with the exception of small studies involving the subgroup of children with systemic onset juvenile idiopathic arthritis, in whom anakinra and tocilizumab may be effective therapies.

  10. Clinical and Serological Findings in Juvenile Patients with Idiopathic Arthritis in Southwestern of Iran

    Directory of Open Access Journals (Sweden)

    Soheila Alyasin

    2014-10-01

    Full Text Available Introduction: The purpose of this study was to describe clinical features and serological findings of children with idiopathic arthritis in south-western Iran.Methods: This descriptive study included 60 patients with juvenile idiopathic arthritis who were referred to a pediatric rheumatology clinic at a university hospital during 6-month period. Initial manifestations, first laboratory tests and clinical course of patients were reviewed.Results: Sixty children (32 boys and 28 girls with idiopathic arthritis ranged in age from 1.5 to 16 years. The mean age at the first presentation was 4.92 years (SD= 3.68. Oligoarthritis was the most common subtype in 27 (45%, followed by systemic- onset in 17 (28.3% and polyarthritis in 16 (26.7% of patients. The most commonly involved joints were knee 53(88.3%, ankle 28(46.6% and wrist 27(45%. Uveitis was detected in two patients, and positivity for ANA titer was revealed in one patient. Conclusions: In this study, the pattern of most clinical features in different subtypes of juvenile idiopathic arthritis resembles to other studies. Positive ANA was less; however, the low numbers of Iranian patients with uveitis was noteworthy.

  11. Subclinical Cardiovascular System Changes in Obese Patients with Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Barbara Głowińska-Olszewska

    2013-01-01

    Full Text Available Objective. We aimed to determine the prevalence of excess body mass in juvenile idiopathic arthritis (JIA children and to investigate the influence of obesity into the early, subclinical changes in cardiovascular system in these patients. Methods. Fifty-eight JIA patients, aged median 13 years, were compared to 36 healthy controls. Traditional cardiovascular risk factors and inflammatory markers (hsCRP, IL-6, TNFα, adiponectin were studied together with IMT (intima-media thickness, FMD (flow mediated dilation, and LVMi (left ventricle mass index as surrogate markers of subclinical atherosclerosis. Results. Thirteen JIA children (22% were obese and had increased systolic blood pressure, cholesterol, triglycerides, insulin, HOMA, hsCRP, and IL-6 compared to nonobese JIA and controls. FMD was decreased compared to nonobese JIA and controls, whereas IMT and LVMi were increased. In multivariate regression analysis, TNFα, SDS-BMI, and systolic blood pressure were independent predictors of early CV changes in JIA. Conclusions. Coincident obesity is common in JIA children and is associated with insulin resistance, dyslipidemia, and increased levels of inflammatory markers leading to early changes in cardiovascular system. Thus, medical care of children with JIA should include strategies preventing cardiovascular disease by maintenance of adequate body weight.

  12. EXPERIENCE OF TREATMENT OF THE TWINS SICK WITH JUVENILE IDIOPATHIC ARTHRITIS BY ABATACEPT

    Directory of Open Access Journals (Sweden)

    V. A. Kel'tsev

    2014-01-01

    Full Text Available The case of clinical observation over twin brothers (boys E. and N suffering juvenile idiopathic arthritis is presented in the article. Boys fell ill with a difference in 1 year and 5 months at the age of 1 year and 9 months and 3 years 2 months, respectively. The disease proceeded wavily with the periods of remissions and exacerbations. Resistant articulate syndrome developed in July and November, 2009, respectively. Both children received NSAID, methotrexate combination in a dose of 10 mg/m2/week and sulphasalazine in a dose of 20 mg/kg of body weight per day. To the boy E. it was also prescripted prednisolon in a dose of 10 mg per day. Duration of antirheumatic therapy made 7 and 3 months, respectively. The therapy with a blocker of T-lymphocytes costimulation, abatacept, was initiated for the twins due to the inefficiency of treatment by immunodepressants and prednisolon at the boy E. In 6 weeks of the treatment 50% improvement, in 24 weeks — 90% improvement by criteria of the American College of Rheumatologists (ACRpedi were registered. The undesirable phenomena weren’t observed.

  13. Differential cytokine profiles in juvenile idiopathic arthritis subtypes revealed by cluster analysis.

    Science.gov (United States)

    van den Ham, Henk-Jan; de Jager, Wilco; Bijlsma, Johannes W J; Prakken, Berent J; de Boer, Rob J

    2009-08-01

    With the introduction of high-throughput biomarker measurements, traditional analysis of these markers is increasingly difficult. Using samples from a diverse group of patients, we tested the applicability of cluster analysis to these data. Using this method, we aim to visualize some of the patterns specific to certain disease groups. In particular, we focus on juvenile idiopathic arthritis (JIA), a multifactorial autoimmune disorder that ultimately leads to chronic inflammation of the joints. Cytokine measurements were performed using multiplex immunoassays. Using heuristic clustering methods, we set out to compare the pattern of 30 cytokines in plasma and SF of JIA, RA, OA, or diabetes type II patients and healthy controls. Analysis shows that oligo- and polyarticular JIA have similar biomarker profiles, both in plasma and SF. Systemic onset JIA (SoJIA) has a profile distinct from other JIA subtypes, suggesting that they involve different inflammatory processes. SoJIA samples do, however, cluster together with RA in SF, suggesting that these two conditions have similar cytokine profiles. Furthermore, we identify several clusters of ILs and chemokines that are co-expressed, suggesting that they are co-regulated. We show that previously undetected clusters of cytokines and patients can be identified by applying cluster analysis to multiplex data. Cytokine clusters identified in plasma and SF samples were quite different, which underscore the differential cytokine signalling in these two compartments, and suggest that plasma samples may not be suitable for estimating joint biomarker profiles and inflammation.

  14. Anti-cyclic citrullinated peptide antibodies in children with Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Hamooda, Mohamed; Fouad, Hala; Galal, Nermeen; Sewelam, Nadia; Megahed, Dina

    2016-09-01

    The purpose of present study was to access the prevalence of anti-cyclic citrullinated peptide (anti-CCP) antibodies in children with Juvenile Idiopathic Arthritis (JIA), and to investigate the clinical significance and diagnostic value of the anti-CCP antibodies in correlation with age, sex & activity. This case-control study was performed on 50 patients with JIA in addition to 40 sex and age-matched children as a control group. The participants were recruited from rheumatology Outpatient Clinic of Cairo University Specialized Pediatric Hospital. Patients were subjected to full history taking, clinical examination, routine laboratory investigations and x-rays on involved joints. Both patients and controls underwent assay of anti-CCP antibodies by AxSYM Anti-CCP IgG Microparticle Enzyme Immunoassay (MEIA) which is a semi-quantitative determination of the IgG class of autoantibodies specific to cyclic citrullinated peptide (CCP) in patients' serum or plasma. Data were analyzed using Mann-Whitney U test, ANOVA, and independent-samples t-test by SPSS version 15. Anti-CCP positivity was identified amongst patients with JIA, particularly those JIA patients experiencing RF positive polyarticular disease onset. Above all, it is important that anti-CCP positivity and bone erosions, degree of joint damage, and ESR levels were significantly correlated. Anti-CCP could be utilized as a valuable marker in the polyarticular form of JIA to direct early, and could be aggressive therapeutic intervention.

  15. SIAE Rare Variants in Juvenile Idiopathic Arthritis and Primary Antibody Deficiencies

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    Eirini Sevdali

    2017-01-01

    Full Text Available Sialic acid acetylesterase (SIAE deficiency was suggested to lower the levels of ligands for sialic acid-binding immunoglobulin-like receptors, decreasing the threshold for B-cell activation. In humans, studies of rare heterozygous loss-of-function mutations in SIAE gene in common autoimmune diseases, including juvenile idiopathic arthritis (JIA, yielded inconsistent results. Considering the distinct pathogenesis of the two main subtypes of JIA, autoinflammatory systemic (sJIA and autoimmune oligo/polyarticular (aJIA, and a predisposition to autoimmunity displayed by patients and families with primary antibody deficiencies (PADs, the aim of our study was to analyze whether SIAE rare variants are associated with both the phenotype of JIA and the autoimmunity risk in families with PADs. A cohort of 69 patients with JIA, 117 healthy children, 54 patients, and family members with PADs were enrolled in the study. Three novel SIAE variants (p.Q343P, p.Y495X, and c.1320+33T>C were found only in patients with aJIA but interestingly also in their healthy relatives without autoimmunity, while none of PAD patients or their relatives carried SIAE defects. Our results show that SIAE rare variants are not causative of autoimmunity as single defects.

  16. Body experiences, emotional competence, and psychosocial functioning in juvenile idiopathic arthritis.

    Science.gov (United States)

    Bomba, Monica; Meini, Antonella; Molinaro, Anna; Cattalini, Marco; Oggiano, Silvia; Fazzi, Elisa; Neri, Francesca; Plebani, Alessandro; Nacinovich, Renata

    2013-08-01

    We investigated self-image, psychological functioning, and quality of life in children and adolescents with juvenile idiopathic arthritis (JIA). Thirty-nine children with JIA were compared with 80 healthy peers. We first administered the Human Figure Drawing Test (HFDT) to all subjects; children also completed standardized questionnaires evaluating health-related quality of life (PEDSQL 4.0 Generic Core Scales) and the main aspects of psychological functioning: anxiety (SAFA-A) and depression (CDI). Parents were asked to complete the Child Behaviour Checklist (CBCL) and the PEDSQL 4.0. For each patient with JIA, clinical notes were gathered and a global disease assessment (visual analog scale--VAS) was performed. Compared to healthy peers, patients with JIA reported reduced maturity quotients at HFDT, more depressive traits, greater anxiety, and lower health-related quality of life. Among the subjects with JIA, HFDT revealed that adolescents had a greater impairment in all areas investigated. Furthermore, there was a significant correlation between the physical well-being rated by VAS and the perception of poorer quality of life in patients, mostly in the psychosocial domains. Children and adolescents with JIA exhibit emotional difficulties and a delay of psychological development leading to low self-esteem, a distorted self-image, more anxiety and depression traits, and a worse quality of life, when compared to healthy subjects.

  17. Gout in a 15-year-old boy with juvenile idiopathic arthritis: a case study.

    Science.gov (United States)

    Morris, Hallie; Grant, Kristen; Khanna, Geetika; White, Andrew J

    2014-01-06

    Joint pain is a common complaint in pediatrics and is most often attributed to overuse or injury. In the face of persistent, severe, or recurrent symptoms, the differential typically expands to include bony or structural causes versus rheumatologic conditions. Rarely, a child has two distinct causes for joint pain. In this case, an obese 15-year-old male was diagnosed with gout, a disease common in adults but virtually ignored in the field of pediatrics. The presence of juvenile idiopathic arthritis (JIA) complicated and delayed the consideration of this second diagnosis. Indeed, the absence of gout from this patient's differential diagnosis resulted in a greater than two-year delay in receiving treatment. The patients' BMI was 47.4, and he was also mis-diagnosed with osteochondritis dissecans and underwent medical treatment for JIA, assorted imaging studies, and multiple surgical procedures before the key history of increased pain with red meat ingestion, noticed by the patient, and a subsequent elevated uric acid confirmed his ultimate diagnosis. With the increased prevalence of obesity in the adolescent population, the diagnosis of gout should be an important consideration in the differential diagnosis for an arthritic joint in an overweight patient, regardless of age.

  18. Anti-cyclic citrullinated peptide antibodies in children with Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Hamooda, Mohamed; Fouad, Hala; Galal, Nermeen; Sewelam, Nadia; Megahed, Dina

    2016-01-01

    Aim The purpose of present study was to access the prevalence of anti-cyclic citrullinated peptide (anti-CCP) antibodies in children with Juvenile Idiopathic Arthritis (JIA), and to investigate the clinical significance and diagnostic value of the anti-CCP antibodies in correlation with age, sex & activity. Methods This case-control study was performed on 50 patients with JIA in addition to 40 sex and age-matched children as a control group. The participants were recruited from rheumatology Outpatient Clinic of Cairo University Specialized Pediatric Hospital. Patients were subjected to full history taking, clinical examination, routine laboratory investigations and x-rays on involved joints. Both patients and controls underwent assay of anti-CCP antibodies by AxSYM Anti-CCP IgG Microparticle Enzyme Immunoassay (MEIA) which is a semi-quantitative determination of the IgG class of autoantibodies specific to cyclic citrullinated peptide (CCP) in patients’ serum or plasma. Data were analyzed using Mann-Whitney U test, ANOVA, and independent-samples t-test by SPSS version 15. Results Anti-CCP positivity was identified amongst patients with JIA, particularly those JIA patients experiencing RF positive polyarticular disease onset. Above all, it is important that anti-CCP positivity and bone erosions, degree of joint damage, and ESR levels were significantly correlated. Conclusion Anti-CCP could be utilized as a valuable marker in the polyarticular form of JIA to direct early, and could be aggressive therapeutic intervention. PMID:27790341

  19. HLA class II genes in Latvian patients with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Rumba, I; Denisova, A; Sochnev, A; Nilsson, B; Sanjeevi, C B

    1997-01-01

    PCR-based HLA genotyping was used to analyze the association of HLA-DR and -DQ genes in 127 juvenile rheumatoid arthritis patients and 111 population-based controls from Latvia. The results show DQA1*03 to be positively associated in overall patients and DRB1*01-DQA1*0101-DQB1*0501 to be negatively associated with JRA in overall patients and in polyarthritis patients compared to controls. These data indicate the immunogenetic heterogeneity in the JRA patients, in the disease subgroups and in different ethnic groups. Rheumatoid factor (RF) was assayed in patients (n = 119) and controls (n = 98). RF was present in patients (7/119, 6%) compared to controls (5/98, 5%). None of the DQA1, DQB1 alleles, DQ and DR-DQ haplotypes was associated in seropositive patients compared to seropositive controls. DR1-DQ5 (DQA1*0101-B*0501) was decreased in seronegative patients (11/111, 10%) compared to seronegative controls (24/105, 23%), but the difference was not significant after correction of the p value.

  20. Land-Jump Performance in Patients with Juvenile Idiopathic Arthritis (JIA): A Comparison to Matched Controls

    Science.gov (United States)

    Ford, Kevin R.; Myer, Gregory D.; Melson, Paula G.; Darnell, Shannon C.; Brunner, Hermine I.; Hewett, Timothy E.

    2009-01-01

    Objective. The purpose of this study was to determine if high functioning children with Juvenile Idiopathic Arthritis (JIA) with minimal disease activity have different biomechanics during high loading tasks compared to controls. Patients were included if they had minimal inflammation documented in one or both knees. Methods. The subject groups consisted of eleven patients with JIA and eleven sex, age, height, and weight matched controls. Sagittal plane kinematic and kinetics were calculated during a drop vertical jump maneuver. The Child Health Assessment Questionnaire (CHAQ) was collected on each patient with JIA. Results. The subjects with JIA had increased knee (P = .011) and hip flexion (P < .001) compared to control subjects. Subjects with JIA also demonstrated decreased knee extensor moments during take-off (P = .028) and ankle plantar flexor moments during landing (P = .024) and take-off (P = .004). In the JIA group, increased hip extensor moments were predictive of increased disability (R2 = .477, SEE = .131). Conclusions. Patients with JIA may demonstrate underlying biomechanical deviations compared to controls. In addition, biomechanical assessment of hip extensor mechanics during dynamic tasks may provide an objective assessment tool to determine overall function in patients with JIA. PMID:20148070

  1. Subclinical cardiovascular system changes in obese patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Głowińska-Olszewska, Barbara; Bossowski, Artur; Dobreńko, Elżbieta; Hryniewicz, Andrzej; Konstantynowicz, Jerzy; Milewski, Robert; Łuczyński, Włodzimierz; Piotrowska-Jastrzębska, Janina; Kowal-Bielecka, Otylia

    2013-01-01

    We aimed to determine the prevalence of excess body mass in juvenile idiopathic arthritis (JIA) children and to investigate the influence of obesity into the early, subclinical changes in cardiovascular system in these patients. Fifty-eight JIA patients, aged median 13 years, were compared to 36 healthy controls. Traditional cardiovascular risk factors and inflammatory markers (hsCRP, IL-6, TNF α, adiponectin) were studied together with IMT (intima-media thickness), FMD (flow mediated dilation), and LVMi (left ventricle mass index) as surrogate markers of subclinical atherosclerosis. Thirteen JIA children (22%) were obese and had increased systolic blood pressure, cholesterol, triglycerides, insulin, HOMA, hsCRP, and IL-6 compared to nonobese JIA and controls. FMD was decreased compared to nonobese JIA and controls, whereas IMT and LVMi were increased. In multivariate regression analysis, TNF α, SDS-BMI, and systolic blood pressure were independent predictors of early CV changes in JIA. Coincident obesity is common in JIA children and is associated with insulin resistance, dyslipidemia, and increased levels of inflammatory markers leading to early changes in cardiovascular system. Thus, medical care of children with JIA should include strategies preventing cardiovascular disease by maintenance of adequate body weight.

  2. Infection-Related Death among Persons with Refractory Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Lane, Jonathan P.; Wood, Mark; Friswell, Mark; Flood, Terence J.; Foster, Helen E.

    2016-01-01

    Severe infections are emerging as major risk factors for death among children with juvenile idiopathic arthritis (JIA). In particular, children with refractory JIA treated with long-term, multiple, and often combined immunosuppressive and antiinflammatory agents, including the new biological disease-modifying antirheumatic drugs (DMARDs), are at increased risk for severe infections and death. We investigated 4 persons with JIA who died during 1994–2013, three of overwhelming central venous catheter–related bacterial sepsis caused by coagulase-negative Staphylococus or α-hemolytic Streptococcus infection and 1 of disseminated adenovirus and Epstein-Barr virus infection). All 4 had active JIA refractory to long-term therapy with multiple and combined conventional and biological DMARDs. Two died while receiving high-dose systemic corticosteroids, methotrexate, and after recent exposure to anti–tumor necrosis factor-α biological DMARDs, and 2 during hematopoietic stem cell transplantation procedure. Reporting all cases of severe infections and especially deaths in these children is of paramount importance for accurate surveillance. PMID:27648582

  3. Decreased antioxidant capacity and increased oxidative stress in patients with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Turkan GUNEY

    2009-11-01

    Full Text Available Purpose: Juvenile idiopathic arthritis (JIA is a common rheumatic disease in children which has three types. Systemic type goes with fever oligoarticular type involving joints are less than five and the polyarticular type more than five joints involved. Reactive oxygen species (ROS have been implicated in its pathogenesis. The ROS generated damage proteins, lipids and serve to amplify the signaling pathways sustaining the synovitis. Enzymes such as superoxide dismutase (SOD and catalase protect cellular systems from ROS. Our hypothesis is; patients with JIA could have defective defense mechanisms against ROS, which can vary from one type to other. Patients and Methods: We investigated antioxidant status including plasma SOD, catalase and serum ceruloplasmin levels in 25 JIA patients. Also, malondialdehyde (MDA, a product generated by the oxygenation of arachidonic acid, levels were measured. Results: Three patients had systemic; 10 with oligoarticular and 12 with polyarticular JİA and control subject number is 20. Plasma SOD and catalase levels were lower, ceruloplasmin and MDA levels were higher were higher in the study group than in controls. There were a negative correlation between catalase, MDA and SOD levels in patients. In between JIA types; the lowest catalase and ceruloplasmin levels were found in oligoarticular type. Conclusively, present study suggested that patients with JIA have decreased antioxidant capacity and defective defense mechanism against ROS and this could be more evident in patients with oligoarticular JIA. In addition, elevated ceruloplasmin levels do not seem to protect against ROS in JIA.

  4. Land-Jump Performance in Patients with Juvenile Idiopathic Arthritis (JIA: A Comparison to Matched Controls

    Directory of Open Access Journals (Sweden)

    Kevin R. Ford

    2009-01-01

    Full Text Available Objective. The purpose of this study was to determine if high functioning children with Juvenile Idiopathic Arthritis (JIA with minimal disease activity have different biomechanics during high loading tasks compared to controls. Patients were included if they had minimal inflammation documented in one or both knees. Methods. The subject groups consisted of eleven patients with JIA and eleven sex, age, height, and weight matched controls. Sagittal plane kinematic and kinetics were calculated during a drop vertical jump maneuver. The Child Health Assessment Questionnaire (CHAQ was collected on each patient with JIA. Results. The subjects with JIA had increased knee (=.011 and hip flexion (<.001 compared to control subjects. Subjects with JIA also demonstrated decreased knee extensor moments during take-off (=.028 and ankle plantar flexor moments during landing (=.024 and take-off (=.004. In the JIA group, increased hip extensor moments were predictive of increased disability (2=.477, =.131. Conclusions. Patients with JIA may demonstrate underlying biomechanical deviations compared to controls. In addition, biomechanical assessment of hip extensor mechanics during dynamic tasks may provide an objective assessment tool to determine overall function in patients with JIA.

  5. Case reports Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome – effective treatment with tocilizumab

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    Małgorzata Kwiatkowska

    2015-08-01

    Full Text Available A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5th year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy’s condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  6. EXPERIENCE OF THE EFFICIENT USING OF METHOTREXATE FOR SUBCUTANEOUS ADMINISTRATION IN PATIENT WITH POLYARTICULAR JUVENILE IDIOPATHIC ARTHRITIS

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    S. A. Ushakova

    2014-01-01

    Full Text Available The article describes successful case of the methotrexate monotherapy through subcutaneous administration (15 mg/m2 of body surface per week for treatment of the polyarticular juvenile idiopathic arthritis in the girl in the age of 4. After 6 and 12 months of the methotrexate therapy the significant reduction of the clinical and laboratory disease activity indices and increase in functional activity of the child according to the pediatric ACR criteria for 50 and 90% respectively were observed. No adverse experience during the monotherapy was recorded. Development of inactive disease phase after 15 months of treatment allows predicting the development of pharmacological remission in patient in the prospective. Timely (immediately after the disease was diagnosed administration of methotrexate allowed preventing the disablement of the child and improving the life quality of both the patient and her family.

  7. Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-up study.

    Science.gov (United States)

    Ekelund, Maria; Aalto, Kristiina; Fasth, Anders; Herlin, Troels; Nielsen, Susan; Nordal, Ellen; Peltoniemi, Suvi; Rygg, Marite; Zak, Marek; Berntson, Lillemor

    2017-02-22

    To study the impact of psoriasis and features associated with psoriasis on classification and outcome in a population-based follow-up cohort of children with juvenile idiopathic arthritis (JIA). In all, 440 children with JIA were followed for a median of 8 years in a prospective Nordic population-based cohort study. Data for remission was available for 427 of these children. The presence of psoriasis, psoriasis-like rash, dactylitis, nail pitting, enthesitis, tenosynovitis and heredity was assessed in relation to ILAR classification and remission. Clinical findings associated with psoriasis developed consecutively during the 8-year period. Six of 14 children with psoriasis were not classified as juvenile psoriatic arthritis according to the ILAR criteria at 8 year follow-up. Dactylitis was more common in children with early onset of JIA. After 8 years we found a cumulative median number of eleven arthritic joints in children with psoriasis or psoriasis-like rash compared with six in the rest of the cohort (p = 0.02). Also, the chance for not being in remission after 8 years increased significantly in patients with psoriasis, psoriasis-like rash or at least two of: 1) first-degree heredity for psoriasis or psoriatic arthritis, 2) dactylitis or 3) nail pitting, compared with the rest of the group (OR 3.32, p = 0.010). Our results indicate a more severe disease over time in psoriasis-associated JIA, as features of psoriasis develop during the disease course. This group is a major challenge to encompass in a future JIA classification in order to facilitate early tailored treatment.

  8. Early detection of temporomandibular joint arthritis in children with juvenile idiopathic arthritis - the role of contrast-enhanced MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kalle, Thekla von; Stuber, Tina; Winkler, Peter [Olgahospital Klinikum Stuttgart, Pediatric Radiology, Radiologisches Institut, Stuttgart (Germany); Maier, Jan; Hospach, Toni [Olgahospital Klinikum Stuttgart, Pediatric Rheumatology, Stuttgart (Germany)

    2015-03-01

    Early treatment of temporomandibular joint (TMJ) arthritis is crucial in children with juvenile idiopathic arthritis (JIA) to prevent permanent functional impairment. As involvement of TMJs is often asymptomatic, contrast-enhanced MRI is regarded as the most sensitive noninvasive diagnostic tool. To evaluate the degree of contrast enhancement in TMJs of children and adolescents with JIA in comparison to normal controls from a previous study. Dynamic contrast-enhanced MRI of 50 children and adolescents with JIA (6.3 to 18 years of age; mean: 12 years) were retrospectively analysed. We assessed morphological abnormalities and postcontrast time-intensity curves of the soft joint tissue and the mandibular condyle. Ratios were calculated to quantify postcontrast signal intensities (SI) in relation to precontrast SI at initial (1 min postcontrast) and maximum (6 min postcontrast) increase. Time-intensity curves followed similar biphasic patterns in normal and pathological joints. In joints with morphological signs of arthritis, mean SI ratios were on average higher than in normal joints of the reference group, but ranges of values widely overlapped. Arthritis: mean initial increase of SI 62% (±2 S.D. 18-105%), mean maximum SI 106% higher than precontrast (±2 S.D. 46-166%). Normal: mean initial increase of SI 49% (±2 S.D. 14- 85%), mean maximum of SI 73% (±2 S.D. 23-123%). Given this considerable overlap of results in dynamic contrast-enhanced MRI, the degree of contrast enhancement alone did not allow differentiation between TMJs with and without signs of inflammation. Thickening of the soft joint tissue seems to remain the earliest sign to reliably indicate TMJ arthritis. (orig.)

  9. Could a Germ Link Gum Disease, Rheumatoid Arthritis?

    Science.gov (United States)

    ... 162571.html Could a Germ Link Gum Disease, Rheumatoid Arthritis? Study may offer new insight into the cause ... the long-noticed connection between gum disease and rheumatoid arthritis, a new study suggests. The discovery might also ...

  10. Síndrome metabólica e artrite idiopática juvenil Metabolic syndrome and juvenile idiopathic arthritis

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    Clarisse de Almeida Zanette

    2010-04-01

    Full Text Available A Artrite Idiopática Juvenil (AIJ é a artropatia crônica mais prevalente na infância e na adolescência. A prevalência da síndrome metabólica, assim como da obesidade, vem apresentando rápido aumento, atingindo todas as faixas etárias, inclusive a infância. A síndrome metabólica é caracterizada por um conjunto de riscos para doença cardiovascular e diabetes melito tipo 2, abrangendo adiposidade abdominal, resistência à insulina, dislipidemias e hipertensão arterial sistêmica. Além desses componentes, a inflamação tem sido reconhecida cada vez mais como um fator importante na síndrome metabólica e na obesidade, e pacientes com doenças caracterizadas por processos inflamatórios crônicos, como a AIJ, poderiam representar grupos de risco especiais. Os glicocorticoides são utilizados rotineiramente no controle da inflamação da AIJ, em doses elevadas e com uso prolongado. O uso crônico do glicocorticoide pode induzir resistência à insulina, hipertensão arterial sistêmica e obesidade, aumentando o risco de desenvolver síndrome metabólica. O objetivo deste artigo é revisar a literatura sobre a prevalência dos diversos componentes da síndrome metabólica em pacientes com AIJ. Observamos que, nesses pacientes, os dados sobre síndrome metabólica e seus componentes são muito escassos e mais estudos se fazem necessários, tendo em vista o potencial impacto no aumento do risco de doença cardiovascular.Juvenile idiopathic arthritis (JIA is the most prevalent chronic arthropathy in childhood and adolescence. The prevalence of metabolic syndrome, as well as obesity, is increasing rapidly in all age groups, including children. Metabolic syndrome is defined as a cluster of risk factors for cardiovascular disease and type 2 diabetes mellitus, including abdominal obesity, insulin resistance, dyslipidemia and hypertension. Besides those components, inflammation has been increasingly considered as a significant component of

  11. Cardiovascular disease in patients with rheumatoid arthritis

    DEFF Research Database (Denmark)

    Naranjo, Antonio; Sokka, Tuulikki; Descalzo, Miguel

    2008-01-01

    ABSTRACT: INTRODUCTION: We analyzed the prevalence of cardiovascular (CV) disease in patients with rheumatoid arthritis (RA) and its association with traditional CV risk factors, clinical features of RA, and the use of disease-modifying antirheumatic drugs (DMARDs) in a multinational cross......-sectional cohort of nonselected consecutive outpatients with RA (The Questionnaires in Standard Monitoring of Patients with Rheumatoid Arthritis Program, or QUEST-RA) who were receiving regular clinical care. METHODS: The study involved a clinical assessment by a rheumatologist and a self-report questionnaire...

  12. EXPERIENCE OF TREATMENT WITH INHIBITOR OF T-LYMPHOCYTES CO-STIMULATION ABATACEPT IN PATIENT WITH POLYARTICULAR TYPE OF JUVENILE RHEUMATOID ARTHRITIS

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    T.M. Bzarova

    2010-01-01

    Full Text Available The article presents a case report of recurrent clinical course of polyarticular type of juvenile rheumatoid arthritis, which is characterized with rapid development of disability, low quality of life, high index of functional insufficiency, torpid flow. The disease developed with intolerance to metotrexate and presence of contra-indications to blockers of tumor necrotizing factor _. Authors describe successful treatment with inhibitor of T-lymphocytes co-stimulation abatacept. The dose of drug was 10 mg/kg of body weight during 24 weeks. In two weeks after the beginning of treatment pain and exudative lesions in joints were lessened, joint range of motions significantly increased. The quality of life of patient and her family increased in 4 weeks of treatment with abatacept. The drug induced clinical and laboratory remission in 24 weeks.Key words: children, juvenile rheumatoid arthritis, abatacept, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. – 2010;9(4:147-154

  13. Ultrasonography and color Doppler of proximal gluteal enthesitis in juvenile idiopathic arthritis: a descriptive study

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    Thomsen Carsten

    2011-08-01

    Full Text Available Abstract Background The presence of enthesitis (insertional inflammation in patients with juvenile idiopathic arthritis (JIA is difficult to establish clinically and may influence classification and treatment of the disease. We used ultrasonography (US and color Doppler (CD imaging to detect enthesitis at the small and deep-seated proximal insertion of the gluteus medius fascia on the posterior iliac crest where clinical diagnosis is difficult. The findings in JIA patients were compared with those obtained in healthy controls and with the patients' MRI results. Methods Seventy-six proximal gluteus medius insertions were studied clinically (tenderness to palpation of the posterior iliac crest and by US and CD (echogenicity, thickness, hyperemia in 38 patients with JIA and in 38 healthy controls, respectively (median age 13 years, range 7-18 years. In addition, an additional MRI examination of the sacroiliac joints and iliac crests was performed in all patients. Results In patients with focal, palpable tenderness, US detected decreased echogenicity of the entheses in 53% of the iliac crests (bilateral in 37% and unilateral in 32%. US also revealed significantly thicker entheses in JIA patients compared to healthy controls (p Conclusions According to US, the gluteus medius insertion was thicker in JIA patients than in controls, and it was hypoechoic (enthesitis in about half of the patients. These findings may represent chronic, inactive disease in some of the patients, because there was only limited Doppler flow and MRI contrast enhancement. The present study indicates that US can be useful as an adjunct to clinical examination for improved assessment of enthesitis in JIA. This may influence disease classification, ambition to treat, and choice of treatment regimen.

  14. Identification of a novel susceptibility locus for juvenile idiopathic arthritis by genome-wide association analysis

    Science.gov (United States)

    Hinks, Anne; Barton, Anne; Shephard, Neil; Eyre, Steve; Bowes, John; Cargill, Michele; Wang, Eric; Ke, Xiayi; Kennedy, Giulia C; John, Sally; Worthington, Jane; Thomson, Wendy

    2009-01-01

    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. PMID:19116933

  15. The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis.

    Science.gov (United States)

    Lin, Yu-Tsan; Wang, Chen-Ti; Gershwin, M Eric; Chiang, Bor-Luen

    2011-06-01

    Juvenile idiopathic arthritis (JIA) has had a long and difficult problem with classification. It is clearly a heterogeneous and multi-factorial autoimmune disease but all too often the distinctions among subtypes were unclear. In fact, there is now increasing evidence of a distinct pathogenesis of oligo/polyarticular JIA compared to systemic JIA. Oligo/polyarticular JIA is an antigen-driven lymphocyte-mediated autoimmune disease with abnormality in the adaptive immune system. Cartilage-derived auto-antigens activate autoreactive T cells including Th1 and Th17 cells with production of pro-inflammatory cytokines IFN-γ and IL-17. On the other hand, the inhibition of regulatory T (Treg) cells including natural Foxp3(+) Treg and self-heat shock protein-induced Treg cells with decreased anti-inflammatory cytokine IL-10 results in the loss of immune tolerance. Imbalance between autoreactive Th1/Th17 and Treg cells leads to the failure of T cell tolerance to self-antigens, which contributes to the synovial inflammation of oligo/polyarticular JIA. By contrast, systemic JIA is an autoinflammatory disease with abnormality in the innate immune system. A loss of control of the alternative secretory pathway leading to aberrant activation of phagocytes including monocytes, macrophages and neutrophils seems to be involved in the release of pro-inflammatory cytokines IL-1, IL-6, IL-18 and pro-inflammatory S100-proteins, which contribute to the multisystem inflammation of systemic JIA. Markedly distinct pathogenesis of oligo/polyarticular JIA and systemic JIA implies that they might need different treatment strategies.

  16. B-cell Lineage Study in Patients with Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Hossein Asgarian-Omran

    2008-12-01

    Full Text Available Objective: Juvenile idiopathic arthritis (JIA is the most common rheumatic disease in children. The exact causes of disease are still poorly understood. It seems that B cells have several functions in JIA, including production of autoantibodies, antigen presentation, production of cytokines, and activation of T cells. Here, we aimed to evaluate B-cell lineage and its precursors in the bone marrow of patients with JIA. Methods: Twenty consecutive patients with JIA were enrolled in this study. JIA is subdivided into three groups of Pauciarticular, Polyarticular, and Systemic JIA. Bone marrow mononuclear cells were separated. Then we analyzed the immunophenotype of the JIA patients by flow cytometry. After separation, the mononuclear cells were stained specific for B cell lineage [CD10, CD19 and CD20], T cell lineage [CD3] and non specific lineage [CD34, HLA-DR and TdT]. Findings: Flow cytometric study of bone marrow showed that JIA patients had low level of CD10, CD19, and CD20. Polyarticular patients had lower level of D10, CD19, and CD20 than pauciarticular JIA patients and systemic onset JIA patients had lower levels than both of them. Conclusion: Decreasing of B cell precursor in bone marrow is one of mechanisms for pathogenesis of JIA and the more decreased B cell precursors in bone marrow are, the worst severity of the disease is. Significant differences in CD10 content of bone marrow were detected between the polyarticular and pauciarticular groups.So, it seems that polyarticular JIA patients had lower percentage of pre B cell stage.

  17. Therapy progress of juvenile idiopathic arthritis%幼年特发性关节炎的治疗进展

    Institute of Scientific and Technical Information of China (English)

    刘光陵; 茅松

    2013-01-01

    幼年特发性关节炎是小儿最常见的慢性风湿免疫性疾病,起病方式不同,临床表现各异,预后较差.有关幼年特发性关节炎的研究已成为近年来结缔组织病的重要研究课题,并且随着医疗水平的提高,对于该病治疗的研究越来越多.%Juvenile idiopathic arthritis (JIA) is the most common chronic imnuro-rheumatic disease in children with different type of onset and varies clinical manifestation as well as poor prognosis.The research on the JIA have become the important subject of the connective disease.This paper has reviewed the different therapeutic methods of JIA.

  18. Rituximab use in young adults diagnosed with juvenile idiopathic arthritis unresponsive to conventional treatment: report of 6 cases.

    Science.gov (United States)

    Sakamoto, Ana Paula; Pinheiro, Marcelo M; Barbosa, Cássia Maria Passarelli Lupoli; Fraga, Melissa Mariti; Len, Claudio Arnaldo; Terreri, Maria Teresa

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Without an effective therapy, patients may progress quickly to functional disability. Recently, depletion of B cells emerged as a new approach for the treatment of autoimmune diseases, including JIA. We describe six cases of JIA patients followed at a referral center for Rheumatology and Pediatric Rheumatology, submitted to treatment with rituximab (RTX) after refractoriness to three anti-TNF agents. Patients received RTX cycles with two infusions every six months. Response to treatment was assessed by DAS28, HAQ/CHAQ, and an overall assessment by the doctor and the patient. Of our six patients, four were girls (mean age at onset of disease: 6.1 years; mean disease evolution time: 15.1 years; mean age upon receiving RTX: 21.6 years). Four patients belonged to polyarticular subtype (1 rheumatoid factor [RF]-negative, 3 FR-positive), a patient with systemic JIA subtype with a polyarticular course and arthritis related to enthesitis. Of our six patients, five responded to treatment; and during the course of 12 months, the clinical response was maintained, although not sustained. However, discontinuation by infusion reactions caused the withdrawal of RTX in two patients. The use of RTX in JIA is restricted to cases refractory to other biological agents and, even considering that this study was held in a small number of advanced patients, RTX proved to be an effective therapeutic option.

  19. Association of the IL2RA/CD25 Gene With Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Hinks, Anne; Ke, Xiayi; Barton, Anne; Eyre, Steve; Bowes, John; Worthington, Jane; Thompson, Susan D; Langefeld, Carl D; Glass, David N; Thomson, Wendy

    2009-01-01

    Objective IL2RA/CD25, the gene for interleukin-2 receptor α, is emerging as a general susceptibility gene for autoimmune diseases because of its role in the development and function of regulatory T cells and the association of single-nucleotide polymorphisms (SNPs) within this gene with type 1 diabetes mellitus (DM), Graves' disease, rheumatoid arthritis (RA), and multiple sclerosis (MS). The aim of this study was to determine whether SNPs within the IL2RA/CD25 gene are associated with juvenile idiopathic arthritis (JIA). Methods Three SNPs within the IL2RA/CD25 gene, that previously showed evidence of an association with either RA, MS, or type 1 DM, were selected for genotyping in UK JIA cases (n = 654) and controls (n = 3,849). Data for 1 SNP (rs2104286) were also available from North American JIA cases (n = 747) and controls (n = 1,161). Association analyses were performed using Plink software. Odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated. Results SNP rs2104286 within the IL2RA/CD25 gene was significantly associated with UK JIA cases (OR for the allele 0.76 [95% CI 0.66–0.88], P for trend = 0.0002). A second SNP (rs41295061) also showed modest evidence for association with JIA (OR 0.80 [95% CI 0.63–1.0], P = 0.05). Association with rs2104286 was convincingly replicated in the North American JIA cohort (OR 0.84 [95% CI 0.65–0.99], P for trend = 0.05). Meta-analysis of the 2 cohorts yielded highly significant evidence of association with JIA (OR 0.76 [95% CI 0.62–0.88], P = 4.9 × 10−5). Conclusion These results provide strong evidence that the IL2RA/CD25 gene represents a JIA susceptibility locus. Further investigation of the gene using both genetic and functional approaches is now required. PMID:19116909

  20. Agreement between physicians and parents in rating functional ability of children with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Buoncompagni Antonella

    2007-12-01

    Full Text Available Abstract Objective To investigate concordance between physicians and parents in rating the degree of functional ability of children with juvenile idiopathic arthritis (JIA. Methods The attending physician and a parent were asked to rate independently the level of physical functioning of 155 patients with disease duration ≥ 5 years on a 6-point scale ranging from 1 = no disability (i.e. the child can do without difficulty all activities that children of his/her age can do to 6 = severe disability (i.e. all activities are difficult for the child. At study visit, measures of JIA activity and damage were assessed. Agreement was evaluated with weighted kappa (0.80 excellent agreement. Physician/parent evaluations were divided in 3 groups: 1 concordance; 2 parent over-rating = parent assessment over-rated relative to physician assessment; 3 physician over-rating = physician assessment over-rated relative to parent assessment. Factors affecting concordance/discordance were evaluated by means of Kruskal-Wallis or Chi-square/Fisher exact test. Results Concordance, parent over-rating and physician over-rating were observed in 107 (69%, 29 (18.7% and 19 (12.3% evaluations, respectively. Kappa value was 0.69. Parent over-rating was associated with greater intensity of pain (p = 0.01 and higher Childhood Health Assessment Questionnaire (C-HAQ score (p = 0.004, whereas physician over-rating was associated with more severe joint disease (p = 0.04 to Conclusion Physicians and parents revealed fair concordance in rating functional ability of children with JIA. Parent over-rating was associated with greater child's pain and worse C-HAQ score, whereas physician over-rating was associated with greater severity of joint inflammation and damage.

  1. Assessment of left ventricular systolic and diastolic function in juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Bharti Bishwa Bhushan

    2004-10-01

    Full Text Available Background and Aims: Recognizing the paucity of data regarding echocardiographic studies of Left ventricular (LV systolic and diastolic function in patients with juvenile rheumatoid arthritis (JRA, a study was carried out to study these parameters in these subjects. Settings, Design and Methods: Thirty-five patients with JRA and an equal number of age- and sex-matched controls were studied by two-dimensional and Doppler echocardiography. Results: Patients with JRA had higher systolic and diastolic blood pressures, resting heart rates, LV systolic (26.9±4.3 vs. 22.4 ± 4.1 mm, p=0.001 and diastolic size (42.3±4.6 vs. 35.4±3.8 mm, p<0.001 and volumes. Though ejection fraction (EF and fractional shortening (FS were normal, they were lower in those with JRA as compared to controls (EF: 62.9±4.47 vs. 67.5±3.63 %, p<0.001; FS: 36.4±4.5 vs. 38.5 ± 6.87, p=0.2. On Doppler analysis the JRA group had lower peak E velocity, higher peak A velocity, higher A VTI and more prolonged IVRT. Male patients had higher A VTI and IVRT as compared to females. Those with longer duration of disease had larger LV systolic (r=0.517, p=0.01 and diastolic dimension (r=0.40, p=0.05 and lower FS (r=-0.506, p=0.01. Patients with polyarticular JRA had higher E and A VTI as compared to those with systemic or oligoarticular types. Conclusion: Despite an asymptomatic cardiac status, significant systolic and diastolic functional abnormalities exist in patients with JRA. The duration of the disease, mode of presentation, patient's age and gender have a significant impact on the left ventricular systolic and diastolic functions in patients with JRA.

  2. Assessment of bone mineral density in adults with a history of juvenile chronic arthritis: a cross-sectional long-term followup study

    DEFF Research Database (Denmark)

    Zak, M; Hassager, C; Lovell, D J

    1999-01-01

    To assess bone mineral density (BMD) and bone turnover in adults with a history of juvenile chronic arthritis (JCA) or persistent JCA, and to identify predictors of reduced BMD.......To assess bone mineral density (BMD) and bone turnover in adults with a history of juvenile chronic arthritis (JCA) or persistent JCA, and to identify predictors of reduced BMD....

  3. Juvenile Spondyloarthritis

    Science.gov (United States)

    Gmuca, Sabrina; Weiss, Pamela F.

    2015-01-01

    Purpose of review To provide a comprehensive update of the pathogenesis, diagnostic imaging, treatments, and disease activity measurements of juvenile spondyloarthritis (JSpA). Recent findings Genetic and microbiome studies have provided new information regarding possible pathogenesis of JSpA. Recent work suggests that children with JSpA have decreased thresholds for pain in comparison to healthy children. Additionally, pain on physical examination and abnormalities on ultrasound of the entheses are not well correlated. Treatment guidelines for juvenile arthritis, including JSpA, were published by the American College of Rheumatology and are based on active joint count and presence of sacroiliitis. Recent studies have established the efficacy of tumor necrosis factor inhibitors in the symptomatic treatment of axial disease, though their efficacy for halting progression of structural damage is less clear. Newly developed disease activity measures for JSpA include the Juvenile Arthritis Disease Activity Score and the JSpA Disease Activity index. In comparison to other categories of juvenile arthritis, children with JSpA are less likely to attain and sustain inactive disease. Summary Further microbiome and genetic research may help elucidate JSpA pathogenesis. More randomized therapeutic trials are needed and the advent of new composite disease activity measurement tools will hopefully allow for the design of these greatly needed trials. PMID:26002028

  4. Juvenile idiopathic arthritis – an update on its diagnosis and ...

    African Journals Online (AJOL)

    2015-12-03

    Dec 3, 2015 ... early diagnosis and treatment have been shown to directly improve .... bowel disease and SLE may increase the risk of these conditions. A family history .... of treatment is complete disease remission and normal physical and.

  5. Intravoxel incoherent motion magnetic resonance imaging of the knee joint in children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hilbert, Fabian; Sauer, Alexander; Koestler, Herbert [University Hospital Wuerzburg, Department of Diagnostic and Interventional Radiology, Wuerzburg (Germany); Holl-Wieden, Annette [University Hospital Wuerzburg, Department of Paediatrics, Wuerzburg (Germany); Neubauer, Henning [University Hospital Wuerzburg, Department of Diagnostic and Interventional Radiology, Wuerzburg (Germany); University Hospital Ulm, Department of Diagnostic and Interventional Radiology, Ulm (Germany)

    2017-05-15

    MRI of synovitis relies on use of a gadolinium-based contrast agent. Diffusion-weighted MRI (DWI) visualises thickened synovium but is of limited use in the presence of joint effusion. To investigate the feasibility and diagnostic accuracy of diffusion-weighted MRI with intravoxel incoherent motion (IVIM) for diagnosing synovitis in the knee joint of children with juvenile idiopathic arthritis. Twelve consecutive children with confirmed or suspected juvenile idiopathic arthritis (10 girls, median age 11 years) underwent MRI with contrast-enhanced T1-weighted imaging and DWI at 1.5 T. Read-out segmented multi-shot DWI was acquired at b values of 0 s/mm{sup 2}, 200 s/mm{sup 2}, 400 s/mm{sup 2} and 800 s/mm{sup 2}. We calculated the IVIM parameters perfusion fraction (f) and tissue diffusion coefficient (D). Diffusion-weighted images at b=800 s/mm{sup 2}, f parameter maps and post-contrast T1-weighted images were retrospectively assessed by two independent readers for synovitis using the Juvenile Arthritis MRI Scoring system. Seven (58%) children showed synovial hypertrophy on contrast-enhanced imaging. Diagnostic ratings for synovitis on DWI and on f maps were fully consistent with contrast-enhanced imaging, the diagnostic reference. Two children had equivocal low-confidence assessments on DWI. Median f was 6.7±2.0% for synovitis, 2.1±1.2% for effusion, 5.0±1.0% for muscle and 10.6±5.7% for popliteal lymph nodes. Diagnostic confidence was higher based on f maps in three (25%) children and lower in one child (8%), as compared to DWI. DWI with IVIM reliably visualises synovitis of the knee joint. Perfusion fraction maps differentiate thickened synovium from joint effusion and hence increase diagnostic confidence. (orig.)

  6. The incidence of juvenile rheumatoid arthritis in Quebec: a population data-based study

    Directory of Open Access Journals (Sweden)

    Houde Michelle

    2009-11-01

    Full Text Available Abstract Objective To determine the population incidence of juvenile rheumatoid arthritis (JRA in Quebec. Methods We obtained data from Quebec's physician claims database. Incident cases were defined as having a visit for JRA in 2000, no visit in the previous 3 years, a confirmed diagnosis by an arthritis specialist, or having ≥ 2 visits to any physician for JRA, ≥ 2 months apart but within 2 years. Results Cumulative incidence of JRA was 17.8/100,000. Mean age at diagnosis was 9.8 ± 4.6 years, 68% were female and more persons were diagnosed in winter. Subjects had a median of 10 medical visits over the first year. Conclusion Our population based incidence estimate was similar to others. Children and adolescents with JRA are heavy users of medical care. Additional study of environmental or climate- related triggers may be warranted.

  7. Association of two functional polymorphisms in the CCR5 gene with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Prahalad, S; Bohnsack, J F; Jorde, L B; Whiting, A; Clifford, B; Dunn, D; Weiss, R; Moroldo, M; Thompson, S D; Glass, D N; Bamshad, M J

    2006-09-01

    Juvenile rheumatoid arthritis (JRA) is mediated by Th1-immune responses. In children with JRA, synovial T cells express high levels of the Th1-chemokine receptor CC chemokine receptor 5 (CCR5), which has been implicated in susceptibility to rheumatoid arthritis. To test the hypothesis that genetic variation in CCR5 is associated with susceptibility to JRA, we analyzed patterns of variation in the 5'cis-regulatory region of CCR5 in 124 multiplex families from a JRA-affected sibpair registry. After sequencing the upstream region of CCR5, variants were tested for association with JRA by transmission disequilibrium testing. A single nucleotide polymorphism, C-1835T, was significantly undertransmitted to children with early-onset JRA (PJRA (PJRA (PJRA (PJRA.

  8. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, Joyce J. F. J.; Cappon, Jeannette; van Rossum, Marion A. J. J.; Sauer, Pieter J. J.; Wulffraat, Nico; van Wijnen, Veera K.; Lelieveld, Otto T. H. M.

    2015-01-01

    Background: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term card

  9. Design and acceptance of Rheumates@Work, a combined internet-based and in person instruction model, an interactive, educational, and cognitive behavioral program for children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Armbrust, Wineke; Bos, Joyce J. F. J.; Cappon, Jeannette; van Rossum, Marion A. J. J.; Sauer, Pieter J. J.; Wulffraat, Nico; van Wijnen, Veera K.; Lelieveld, Otto T. H. M.

    2015-01-01

    BACKGROUND: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease. Patients suffer daily discomforts such as pain, fatigue, stiffness, and mood disturbances. Their exercise capacity is decreased to a variable degree and physical activity levels may be impaired. To prevent long-term card

  10. [The temporomandibular joint in juvenile idiopathic arthritis: what radiologists need to look for on magnetic resonance imaging].

    Science.gov (United States)

    De La Hoz Polo, M; Navallas, M

    2014-01-01

    The term "juvenile idiopathic arthritis" (JIA) encompasses a group of arthritis of unknown cause with onset before the age of 16 years that last for at least 6 weeks. The prevalence of temporomandibular joint involvement in published series ranges from 17% to 87%. Temporomandibular joint involvement is difficult to detect clinically, so imaging plays a key role in diagnosis and monitoring treatment. MRI is the technique of choice for the study of arthritis of the temporomandibular joint because it is the most sensitive technique for detecting acute synovitis and bone edema. Power Doppler ultrasonography can also detect active synovitis by showing the hypervascularization of the inflamed synovial membrane, but it cannot identify bone edema. This article describes the MRI technique for evaluating the temporomandibular joint in patients with juvenile idiopathic arthritis, defines the parameters to look for, and illustrates the main findings. Copyright © 2013 SERAM. Published by Elsevier Espana. All rights reserved.

  11. Lipoprotein cholesterol fractions are related to markers of inflammation in children and adolescents with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Bohr, Anna-Helene; Pedersen, Freddy Karup; Nielsen, Claus Henrik

    2016-01-01

    BACKGROUND: The purpose of the study is to determine levels of total cholesterol (TC), low-density, and high-density lipoprotein fractions of cholesterol (LDLc and HDLc), in patients with juvenile idiopathic arthritis (JIA), and relate those to disease activity, overweight, and physical activity...... in the patients were within the normal range for Danish Children. HDLc was negatively correlated with MRP8/14 (r = -0.343, CI -0.474 to -0.201, p overweight or PA. Neither TC nor LDLc showed any association with inflammation, overweight, or PA. MRP8/14 correlated positively...... (PA), testing the hypothesis that the levels of cholesterol fractions are associated with inflammation as well as with overweight and low PA. METHODS: Two hundred ten patients with JIA were included in this descriptive cross-sectional study. TC, LDLc, HDLc were measured, and associations with clinical...

  12. Relationship of pain coping strategies and pain specific beliefs to pain experience in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Thastum, Mikael; Herlin, Troels; Zachariae, R.

    2005-01-01

    OBJECTIVE: To examine whether pain-specific beliefs and coping strategies of patients with juvenile idiopathic arthritis (JIA) independently predict their reported pain, while controlling for relevant demographic variables, disease activity, and parent-rated disability. To compare use of pain...... contribution to the prediction of pain after controlling for other variables. Significant differences were found between the scores of high pain patients and the rest of the group for the health belief subscale of disability (mean +/- SD 2.0 +/- 0.6 and 1.2 +/- 0.7, respectively), and for the health belief...... subscale of harm (mean +/- SD 2.7 +/- 0.6 and 1.8 +/- 0.7, respectively). Significant correlations were obtained between the pain measure and the pain-coping subscale of catastrophizing, the pain belief subscales of disability, harm, solicitude (inverse), control, and medical cure. CONCLUSION...

  13. Late sequelae of juvenile rheumatoid arthritis of the hip: A follow-up study into adulthood

    Energy Technology Data Exchange (ETDEWEB)

    Patriquin, H.B.; Camerlain, M.; Trias, A.

    1984-03-01

    A 4-13 year follow up study of 29 patients with juvenile rheumatoid arthritis into adulthood revealed several structural deformities of the hips: coxa magna, short femoral neck, subluxations associated with large cyst-like erosions of femur and acetabulum near the ligamentum teres, flattened, wide femoral head (without steroid treatment). The deformities evoke the possibility of ischemia of the femoral head in the presence of active, compressive synovial proliferation during the growth period in a joint with largely intra-articular nutrient vessels.

  14. A radiographic classification system in juvenile rheumatoid arthritis applied to the knee

    Energy Technology Data Exchange (ETDEWEB)

    Dale, K. [Dept. of Radiology, Oslo Sanitetsforening Rheumatism Hospital (Norway); Paus, A.C. [Dept. of Surgery, Oslo Sanitetsforening Rheumatism Hospital (Norway); Laires, K. [Dept. of Radiology, Oslo Sanitetsforening Rheumatism Hospital (Norway)

    1994-02-01

    A new radiographic grading system for evaluation of juvenile rheumatoid arthritis (JRA) for the knee is presented. The classification is based on known arthritic criteria in childhood. Joints with erosion are given a higher score than growth disturbances alone. Signs of osteoarthrosis including joint space narrowing were excluded from the classification. The femorotibial and patello-femoral joints are assessed together. Verbal definitions are used for the classification, but, regarding the erosions, standard reference films are used. The intra- and inter-observer variations of the method were low. (P < 0.01) (orig.)

  15. Antioxidant status in children with juvenile rheumatoid arthritis (JRA) living in Cairo, Egypt.

    Science.gov (United States)

    Ashour, M; Salem, S; Hassaneen, H; el-Gadban, H; Elwan, N; Awad, A; Basu, T K

    2000-03-01

    The aim of this study was to examine both enzymatic and non-enzymatic antioxidant status in a select group of children with juvenile rheumatoid arthritis (JRA), living in Cairo, Egypt. The plasma concentrations of albumin, ceruloplasmin, vitamin C, vitamin E as well as erythrocyte superoxide dismutase and whole blood glutathione peroxidase activities were all significantly decreased in the presence of JRA compared to those without JRA. Unlike these antioxidant factors, vitamin A and its carrier (e.g. retinol binding protein), which have very little or no antioxidant property, remained unaffected by JRA. These results suggest that the children with JRA are subject to oxidative stress.

  16. Premature subclinical atherosclerosis in children and young adults with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Bohr, Anna-Helene; Fuhlbrigge, Robert C; Karup Pedersen, Freddy

    2016-01-01

    Many studies show that Juvenile Idiopathic Arthritis (JIA) is associated with early subclinical signs of atherosclerosis. Chronic inflammation per se may be an important driver but other known risk factors, such as dyslipidemia, hypertension, insulin insensitivity, a physically inactive lifestyle......, were considered.We found 13 descriptive cross sectional studies with healthy controls, one intervention study and two studies on adults diagnosed with JIA. Only one study addressed obesity, and physical activity (PA) has only been assessed in one study on adults with JIA and only by self...

  17. Etanercept In the treatment of AA-amyloidosis in juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    H Mihels

    2004-01-01

    Full Text Available Objective. To study effect of etanercept, an antagonist of serum A amyloid production in the AA amyloidosis treatment in juvenile idiopathic arthritis (JIA. Material and methods. Etanercept was administered to all pts with AA amyloidosis admitted to Garmisch-Paterkirchen pediatric rheumatological clinic beginning with 2000. C-reactive protein (CRP, degree of proteinuria and serum creatinin were used as preliminary outcome measures. Results. 11 pts with seronegative JIA (6 boys and 5 girls were included in the study. Mean follow up duration was l,9±l.01 years. 8 children had systemic, 2 - olygoarticular and one - polyarticular disease onset. Before the study all pts had CRP level elevation (1,03-8,29 mg/dl, mean 4,53 mg/dl. 8 from 11 had marked proteinuria (364-7400 mg/24 hours, mean 1186 mg/24 hours. 2 from 11 had serum creatinin elevation. During etanercept treatment CRP level normalized in 2 and significantly decreased in 4 pts. Proteinuria decreased in 4 from 8 pts. Significant change of creatinin level was not achieved. One girl who did not have improvement during etanercept treatment showed CRP normalization and decrease of proteinuria when the drug was changed to infliximab. Conclusion. Treatment with etanercept provided improvement in almost 2/3 from 11 pts with JIA and AA amyloidosis. Etanercept may be the drug of choice in pts with normal creatinine level in the absence of proteinuria. When it fails another tumor necrosis factor antagonist such as infliximab should be used. It is necessary to extend volume and duration of the study to get more reliable data on etanercept efficacy in JIA pts with AA amyloidosis.

  18. The diagnostic accuracy of unenhanced MRI in the assessment of joint abnormalities in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hemke, Robert [University of Amsterdam, Department of Radiology (G1-235), Academic Medical Center, Amsterdam (Netherlands); University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Kuijpers, Taco W.; Veenendaal, Mira van [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Berg, J.M. van den; Rossum, Marion A.J. van [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Reade, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Dolman, Koert M. [Reade, Department of Pediatric Rheumatology, Amsterdam (Netherlands); St. Lucas Andreas Hospital, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Maas, Mario [University of Amsterdam, Department of Radiology (G1-235), Academic Medical Center, Amsterdam (Netherlands)

    2013-07-15

    To assess the diagnostic accuracy and reliability of MRI without contrast enhancement in the evaluation of JIA knee joint abnormalities. JIA patients with clinically active knee involvement were prospectively studied using an 1-T open-bore magnet. MRI features were independently evaluated by two readers using the JAMRIS system. The first reading included unenhanced images, whereas complete image sets were available for the second reading. Imaging findings from 73 patients were analysed. Agreement between Gd-enhanced (+Gd) and Gd-unenhanced (-Gd) MRI scores of bone marrow changes, cartilage lesions and bone erosions was good concerning sensitivity, specificity, negative predictive value and positive predictive value. Inter-observer agreement was good for both -Gd and +Gd scores (ICC = 0.91-1.00, 0.93-1.00, respectively). Regarding the assessment of synovial hypertrophy, specificity of -Gd was high (0.97), but the sensitivity of unenhanced MRI was only 0.62. Inter-reader agreement for +Gd MRI was ICC = 0.94; however, omitting post-Gd acquisitions increased inter-reader variation (ICC = 0.86). If Gd-enhanced MRI is the reference standard, omitting Gd contrast medium is irrelevant for the assessment of bone marrow changes, cartilage lesions and bone erosions as joint abnormalities in JIA. Omitting intravenous Gd in the MRI assessment of joints in JIA is inadvisable, because it decreases the reliability of detecting synovial disease. circle Magnetic resonance imaging is increasingly used to assess juvenile idiopathic arthritis. circle Synovial hypertrophy, a marker of JIA activity, is well shown by MRI. (orig.)

  19. Sexual maturation in Egyptian boys and girls with juvenile rheumatoid arthritis.

    Science.gov (United States)

    Maher, Sheren Esam; Ali, Faten Ismael

    2013-08-01

    Children with juvenile rheumatoid arthritis (JRA) exhibit a compromised growth status while information concerning the pattern of their sexual maturity is scant. The aim of the current work was to study sexual maturity in boys and girls with JRA. The study included eighty JRA patients they were 45 male and 35 female and eighty age- and sex-matched normal children served as controls. Development of genitalia was evaluated as per sexual maturity rating criteria given by Tanner score. Development of hair (pubic, axillary and facial) and age of monarch to JRA females were noted The mean (±SD) age of appearance of genitalia stage G-2 in boys with systemic onset JRA (12.0 ± 0.3 years) was earlier when compared with pauciarticular (12.60 ± 0.93 years) and polyarticular (13.39 ± 0.93 years) JRA but delayed for all types of JRA when compared with controls (10.06 ± 0.63 years). In comparison with female groups, the mean (±SD) age of appearance of genitalia stage G-2 with systemic onset JRA (12.0 ± 0.4 years) was also earlier when compared with pauciarticular (12.68 ± 1.09 years) and polyarticular (13.72 ± 0.39 years). Age of menarche delayed in all JRA female patients. None of the study group reach stage G-5 of genitalia development. The timing of initiation of sexual maturity in boys and girls with JRA delayed and this delay variable according to disease subtype.

  20. [Cytokine profile changes in children with juvenile idiopathic arthritis-associated uveitis].

    Science.gov (United States)

    Drozdova, E A; Yadykina, E V; Mezentseva, E A; Nikushkina, K V

    to identify the differences between serum cytokine profiles in juvenile idiopathic arthritis (JIA) with or without uveal tract inflammation. Serum cytokine profiles were studied in two groups of patients: 20 children with JIA and JIA-associated uveitis and 33 children, who had no signs of uveitis under basic therapy for their JIA. All the patients showed drug remission of articular syndrome. Inflammation of the choroid took the form of chronic anterior uveitis. The process was active in 95% of cases. The control group consisted of 35 children without rheumatic disease or other acute condition at the time of examination. Groups were comparable in terms of age and sex. Serum levels of TNF-α, IFN-γ, IL-17, IL-10 were measured by the enzyme multiplied immunoassay technique. A statistically significant increase in TNF-α, IFN-γ, IL-17, and IL-10 levels was found in all patients as compared to the control group. A comparison drawn between serum cytokine levels of JIA patients and those, who also suffered from JIA-associated uveitis, revealed a decrease in IFN-γ and an increase in IL-10 in the latter group. There was also a statistically significant positive correlation between TNF-α and IFN-γ serum levels in patients with JIA-associated uveitis. Development of uveitis in patients with drug remission of JIA occurs on the background of cytokine imbalance in the serum, in particular, increased concentrations of proinflammatory TNF-α and IL-17 cytokines along with reduced IFN-γ and increased IL-10 levels. This may be regarded as risk factors for ocular inflammation and should be taken into account when making treatment decisions.

  1. Exposure to animals and risk of oligoarticular juvenile idiopathic arthritis: a multicenter case-control study

    Directory of Open Access Journals (Sweden)

    Michels Hartmut

    2010-04-01

    Full Text Available Abstract Background An inverse association between early contact with microbial compounds and respiratory allergies is well established. The protective effect of infant contact with animals was also shown for inflammatory bowel disease (IBD and systemic lupus erythematosus (SLE. We aimed to test the association between animal contact in infancy and oligoarticular juvenile idiopathic arthritis (OA JIA. Methods Parents of children with OA JIA registered at the Hospital for Pediatric Rheumatology in Garmisch-Partenkirchen were asked to complete a questionnaire. Children who underwent strabismus surgery at six referral centers for ophthalmology served as controls. Children age 6 to 18 years born in Germany without malformations were included (238 cases; response 89% and 832 controls; response 86%. Data were analyzed using logistic regression models after adjusting for potential confounders. Results Neither place of living (urban vs. rural area, living on a farm, nor regular farm animal (adjusted odds ratio 0.79; 95% confidence interval 0.42-1.47 or pet contact (0.79; 0.55-1.14 during infancy were clearly related to case status. Allergic rhinitis was inversely related to OA JIA (0.57; 0.34-0.95. Neither place of living (urban vs. rural area, living on a farm, nor regular farm animal (adjusted odds ratio 0.79; 95% confidence interval 0.42-1.47 or pet contact (0.79; 0.55-1.14 during infancy were related to case status. Allergic rhinitis was inversely related to OA JIA (0.57; 0.34-0.95. Conclusions Contact with farm environments in infancy might not be associated with OA JIA. This finding is consistent with previous findings for diabetes mellitus type 1 but contradicts results for IBD and SLE.

  2. Rheumatic patients at work : a study of labour force participations and its determinants in rheumatoid arthritis, ankylosing spondylitis, and juvenile chronic arthritis

    NARCIS (Netherlands)

    Chorus, A.M.J.

    2004-01-01

    This thesis at the University of Maastricht, defended at May 7, 2004, yields several important and new findings with regard to work related quality of life, participation in the labour force and its determinants of patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS) and juvenile chr

  3. Anti-type II collagen antibodies detection and avidity in patients with oligoarticular and polyarticular forms of juvenile idiopathic arthritis.

    Science.gov (United States)

    Araujo, Galber R; Fonseca, João E; Fujimura, Patricia T; Cunha-Junior, Jair P; Silva, Carlos H M; Mourão, Ana F; Canhão, Helena; Goulart, Luiz R; Gonçalves, João; Ueira-Vieira, Carlos

    2015-05-01

    Juvenile idiopathic arthritis (JIA) refers to a heterogeneous group of illnesses that have in common the occurrence of chronic joint inflammation in children younger than 16 years of age. The diagnosis is made only on clinical assessment. The identification of antibody markers could improve the early diagnosis, optimizing the clinical management of patients. Type II collagen is one potential autoantigen that has been implicated in the process of arthritis development. The aims of our study were to investigate the occurrence of anti-type II collagen antibodies and also to determine the avidity of the antibody-antigen binding. Ninety-six patients with oligoarticular or polyarticular JIA, 13 patients with ankylosing spondylitis (AS) and 61 healthy controls (HC) were tested for anti-type II collagen antibodies by ELISA and avidity ELISA. Sensitivity and specificity were determined by the receiver operating characteristic (ROC) curve analysis. Forty-two JIA patients (44%) were positive for antibodies against type II collagen. Its detection was significantly higher in JIA patients than in AS patients (p=0.006) and HCs (ppolyarticular JIA, being its presence more prevalent in patients with early disease. It also demonstrates that JIA patients with active disease present antibodies with high avidity against type II collagen.

  4. Distinct Effects of Methotrexate and Etanercept on the B Cell Compartment in Patients With Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Glaesener, Stephanie; Quách, Tâm D; Onken, Nils; Weller-Heinemann, Frank; Dressler, Frank; Huppertz, Hans-Iko; Thon, Angelika; Meyer-Bahlburg, Almut

    2014-01-01

    Objective B cells have been shown to play an important role in the pathogenesis of rheumatoid arthritis and juvenile idiopathic arthritis (JIA). Current treatments include the disease-modifying antirheumatic drugs methotrexate (MTX) and tumor necrosis factor α inhibition with etanercept. This study was undertaken to determine how these drugs influence the B cell compartment in patients with JIA. Methods B cell subpopulations and follicular helper T (Tfh) cells in the peripheral blood of JIA patients were investigated by multicolor flow cytometry. Serum immunoglobulin and BAFF levels were determined by enzyme-linked immunosorbent assay. Results There was a significant decrease in transitional B cells and significantly lower serum immunoglobulin levels in patients receiving MTX than in untreated patients and those receiving etanercept. In contrast, etanercept treatment had no effect on most of the B cell subpopulations, but resulted in significantly lower BAFF levels and increased numbers of Tfh cells. Thus, our findings indicate an unexpected and previously unknown direct effect of low-dose MTX on B cells, whereas etanercept had a more indirect influence. Conclusion Our results contribute to a better understanding of the potency of MTX in autoantibody-mediated autoimmune disease and present a possible mechanism of prevention of the development of drug-induced antibodies to biologic agents. The finding that MTX and etanercept affect the B cell compartment differently supports the notion that combination therapy with etanercept and MTX is more effective than monotherapy. PMID:24909567

  5. Modern methods of early diagnostics of juvenile arthritis

    Directory of Open Access Journals (Sweden)

    Chernenkov Y.V.

    2013-06-01

    Full Text Available The problem of inflammatory diseases of joints is one of the most important issues in the pediatrics. Nowadays the significant attention in this sphere is paid to the search of new accurate criteria of diagnostics. It will help estimate the severity of disease, determine the prognosis, choose the method of treatment and monitoring and evaluate the efficacy of the therapy.

  6. The comparisons between thermography and ultrasonography with physical examination for wrist joint assessment in juvenile idiopathic arthritis.

    Science.gov (United States)

    Lerkvaleekul, Butsabong; Jaovisidha, Suphaneewan; Sungkarat, Witaya; Chitrapazt, Niyata; Fuangfa, Praman; Ruangchaijatuporn, Thumanoon; Vilaiyuk, Soamarat

    2017-03-01

    This study aimed to assess infrared thermography (IRT) and ultrasonography (US) for detecting wrist arthritis in juvenile idiopathic arthritis (JIA) patients. Although IRT could help us detecting joint inflammation, IRT studies in JIA patients with wrist arthritis are still limited. Currently, no validated US criteria exist for detecting arthritis, and the most useful parameters between Gray-scale ultrasound (GSUS) or Power Doppler ultrasound (PDUS) remain unclear. Therefore, this study focused on detecting wrist arthritis in varying degrees using IRT and US compared with physical examination. Of 46 JIA patients, 16 had previous wrist arthritis but currently inactive, 30 still had wrist arthritis, and the median ages (IQR) were 7.7 (4.3) and 10.2 (4.8) years respectively. Fifteen healthy participants were included, with a median age (IQR) of 9.2 (2.0) years. Using IRT, mean temperature (Tmean) and maximum temperature (Tmax) at skin surface in the region of interest (ROI) in the arthritis group were higher than in the inactive group and the healthy controls with p examination, the moderate to severe arthritis had Tmean and Tmax higher than the mild arthritis group with statistical significance. The Heat Distribution Index (HDI), two standard deviations of all pixel temperature values in the ROI, in the moderate to severe arthritis group was higher than in the healthy controls (p = 0.027). The receiver operating characteristic analysis in arthritis detection revealed diagnostic sensitivity of 85.7% and 71.4% and specificity of 80.0% and 93.3% at a cut-off points of Tmean ≥ 31.0 C and Tmax ≥ 32.3 C respectively. For US, GSUS and PDUS are useful in detecting arthritis, providing high sensitivity (83.3%) and specificity (81.3%). Our study demonstrated that both IRT and US were applicable tools for detecting wrist arthritis.

  7. Benefit of fluoroscopically guided intraarticular, long-acting corticosteroid injection for subtalar arthritis in juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Cahill, Anne M.; Cho, Sandy S. [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Baskin, Kevin M. [Children' s Hospital of Philadelphia, Department of Pediatrics, Division of Rheumatology, Philadelphia, PA (United States); Beukelman, Timothy; Cron, Randy Q. [Children' s Hospital of Pittsburgh, Department of Radiology, Pittsburgh, PA (United States); Kaye, Robin D. [Children' s Hospital of Wisconsin, Department of Radiology, Milwaukee, WI (United States); Towbin, Richard B.

    2007-06-15

    Children with arthritis may endure a lifetime of disfigurement, dysfunction, and pain if acute inflammation progresses to chronic changes in the joint cartilage and underlying bone. Intraarticular steroids have become an integral component of treatment, but at times are difficult to deliver to joints, such as the subtalar joint, that have complex anatomies. We describe our technique and outcomes using fluoroscopically guided intraarticular subtalar steroid injection in patients with active symptoms of juvenile idiopathic arthritis (JIA). Fluoroscopically guided subtalar joint injections were performed in 38 children (mean age 6.7 years). Medical records were reviewed retrospectively and improvement was evaluated clinically by the degree of foot movement in eversion and inversion. Subtalar joint injection was technically successful in 100% of the JIA patients with improvement in physical symptoms in 34/38 (89%). Of the 38 children, 32 were followed up within 13 weeks of the initial injection and, therefore, satisfied the eligibility criteria for resolution of arthritis. Of these 32 children, 14 showed clinical resolution (44%). The mean duration of improvement was 1.2 {+-} 0.9 years. Children with a longer interval (>1 year) from diagnosis to treatment had significantly less resolution (P = 0.04). Local subcutaneous atrophy or hypopigmentation were observed in 53% of the children after steroid injection (20/38). These minor complications were associated with a greater volume of steroid injected into the site per child (P = 0.02). Fluoroscopically guided subtalar joint injection is an effective treatment for subtalar arthropathy. Prompt referral for intraarticular steroid treatment in the acute phase improves response. Skin changes often occur at the injection site, and specific precautions should be employed to reduce this risk. Prospective study is indicated to determine the most effective treatment strategy to prevent long-term pain and disability. (orig.)

  8. Evidence for chronic, peripheral activation of neutrophils in polyarticular juvenile rheumatoid arthritis.

    Science.gov (United States)

    Jarvis, James N; Petty, Howard R; Tang, Yuhong; Frank, Mark Barton; Tessier, Philippe A; Dozmorov, Igor; Jiang, Kaiyu; Kindzelski, Andrei; Chen, Yanmin; Cadwell, Craig; Turner, Mary; Szodoray, Peter; McGhee, Julie L; Centola, Michael

    2006-01-01

    Although strong epidemiologic evidence suggests an important role for adaptive immunity in the pathogenesis of polyarticular juvenile rheumatoid arthritis (JRA), there remain many aspects of the disease that suggest equally important contributions of the innate immune system. We used gene expression arrays and computer modeling to examine the function in neutrophils of 25 children with polyarticular JRA. Computer analysis identified 712 genes that were differentially expressed between patients and healthy controls. Computer-assisted analysis of the differentially expressed genes demonstrated functional connections linked to both interleukin (IL)-8- and interferon-gamma (IFN-gamma)-regulated processes. Of special note is that the gene expression fingerprint of children with active JRA remained essentially unchanged even after they had responded to therapy. This result differed markedly from our previously reported work, in which gene expression profiles in buffy coats of children with polyarticular JRA reverted to normal after disease control was achieved pharmacologically. These findings suggest that JRA neutrophils remain in an activated state even during disease quiescence. Computer modeling of array data further demonstrated disruption of gene regulatory networks in clusters of genes modulated by IFN-gamma and IL-8. These cytokines have previously been shown to independently regulate the frequency (IFN-gamma) and amplitude (IL-8) of the oscillations of key metabolites in neutrophils, including nicotinamide adenine dinucleotide (phosphate) (NAD(P)H) and superoxide ion. Using real-time, high-speed, single-cell photoimaging, we observed that 6/6 JRA patients displayed a characteristic defect in 12% to 23% of the neutrophils tested. Reagents known to induce only frequency fluctuations of NAD(P)H and superoxide ion induced both frequency and amplitude fluctuations in JRA neutrophils. This is a novel finding that was observed in children with both active (n = 4) and

  9. Managing juvenile Huntington’s disease

    OpenAIRE

    Quarrell, Oliver W. J.; Nance, Martha A.; Nopoulos, Peggy; Paulsen, Jane S.; Smith, Jonathan A.; Squitieri, Ferdinando

    2013-01-01

    Huntington’s disease (HD) is a well-recognized progressive neurodegenerative disorder that follows an autosomal dominant pattern of inheritance. Onset is insidious and can occur at almost any age, but most commonly the diagnosis is made between the ages of 35 and 55 years. Onset ≤20 years of age is classified as juvenile HD (JHD). This age-based definition is arbitrary but remains convenient. There is overlap between the clinical pathological and genetic features seen in JHD and more traditio...

  10. Ultrasonography and color Doppler in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Nielsen, Susan

    2011-01-01

    hypertrophy, effusion) and by color Doppler (synovial hyperemia) before and 4 weeks after US-guided steroid injection. Results US detected 121 compartments with active disease (joints, tendon sheaths and 1 ganglion cyst). Multiple compartments were involved in 80% of the ankle regions. The talo-crural joint...... inflammation in the ankle region of JIA patients. The talo-crural joint was not always involved. Disease was frequently found in compartments difficult to evaluate clinically. US enabled exact guidance of steroid injections, gave a low rate of subcutaneous atrophy and was proved valuable for follow...

  11. Vertebral Osteomyelitis and Septic Arthritis Associated With Staphylococcus hyicus in a Juvenile Peregrine Falcon ( Falco peregrinus ).

    Science.gov (United States)

    Maier, Kristina; Fischer, Dominik; Hartmann, Antje; Kershaw, Olivia; Prenger-Berninghoff, Ellen; Pendl, Helene; Schmidt, Martin J; Lierz, Michael

    2015-09-01

    A 6-week-old, parent-reared peregrine falcon ( Falco peregrinus ) was presented with spastic hypertonus of its hind limbs of unknown origin and duration. Radiologic examination revealed smooth periosteal reactions ventrally at thoracic vertebrae 5 to 7. Contrast-enhanced computed tomography identified the swelling as inflammation; antibiotic, antimycotic, anti-inflammatory, and analgesic treatments were initiated, and vitamins and minerals were supplemented. Because the bird's condition did not improve after 10 days, it was euthanatized and submitted for postmortem examination. On histopathologic examination, chronic, active osteomyelitis was diagnosed in thoracic vertebrae 5 to 7, and chronic, active arthritis was present in both the right shoulder and left elbow joints. Staphylococcus hyicus was isolated from these 3 locations, as well as from lungs and liver, indicating a chronic septic staphylococcosis. Although infections with Staphylococcus species are occasional causes of vertebral osteomyelitis in juvenile poultry with active growth plates, it is only sporadically reported in raptors and companion birds. This case report is the first description of the clinical features and diagnostic and pathologic findings in a juvenile peregrine falcon with hematogenous osteomyelitis and arthritis associated with septicemia caused by S hyicus.

  12. [Change in condylar and mandibular morphology in juvenile idiopathic arthritis: cone beam volumetric imaging].

    Science.gov (United States)

    Garagiola, Umberto; Mercatali, Lorenzo; Bellintani, Claudio; Fodor, Attila; Farronato, Giampietro; Lőrincz, Adám

    2013-03-01

    The aim of this study is to show the importance of Cone Beam Computerized Tomography to volumetrically quantify TMJ damage in patients with JIA, measuring condylar and mandibular real volumes. 34 children with temporomandibular involvement by Juvenile Idiopathic Arthritis were observed by Cone Beam Computerized Tomography. 4 were excluded because of several imaging noises. The mandible was isolated from others craniofacial structures; the whole mandibular volume and its components' volumes (condyle, ramus, hemibody, hemisymphysis on right side and on left side) has been calculated by a 3D volume rendering technique. The results show a highly significant statistical difference between affected side volumetric values versus normal side volumetric values above all on condyle region (P < 0.01), while they don't show any statistical differences between right side versus left side. The Cone Beam Computerized Tomography represents a huge improvement in understanding of the condyle and mandibular morphological changes, even in the early stages of the Juvenile Idiopathic Arthritis. The JIA can lead in children to temporomandibular joint damage with facial development and growth alterations.

  13. Anti-TNF therapy for juvenile idiopathic arthritis-related uveitis

    Directory of Open Access Journals (Sweden)

    Semeraro F

    2014-03-01

    Full Text Available Francesco Semeraro,1 Barbara Arcidiacono,2 Giuseppe Nascimbeni,1 Martina Angi,1 Barbara Parolini,2 Ciro Costagliola31Eye Clinic, Department of Neurological Sciences and Vision, University of Brescia, Brescia, Italy; 2Department of Ophthalmology, S. Anna Hospital, Brescia, Italy; 3Eye Clinic, Department of Health Sciences, University of Molise, Campobasso, ItalyAbstract: Juvenile idiopathic arthritis-related uveitis is the most common type of uveitis in childhood and one of the main causes of visual impairment in children. The introduction of biological treatment has widened the range of therapeutic options for children with uveitis refractory to standard nonbiologic immunosuppressants. Data from clinical trials suggest that both adalimumab and infliximab have demonstrated effectiveness and safety in open-label studies, although no large, randomized, controlled trials have been reported so far. The role of etanercept in treating juvenile idiopathic arthritis-related uveitis is not yet well defined. In our experience, anti-tumor necrosis factor therapy has been shown to be more effective than steroids and/or methotrexate in treating uveitis. Up to now, tumor necrosis factor blocking compounds have been reserved for the treatment of the most severe cases of refractory uveitis, and larger prospective clinical trials are required in order to better assess the safety of these new compounds.Keywords: adalimumab, etanercept, infliximab

  14. Recognition systemic juvenile idiopathic arthritis%再认识全身型幼年特发性关节炎

    Institute of Scientific and Technical Information of China (English)

    吴凤岐

    2014-01-01

    全身型幼年特发性关节(sJIA)是幼年特发性关节炎(JIA)的一个亚型,不同于JIA的其他亚型,sJIA的临床表现除关节炎外,全身表现特别突出,如弛张热、皮疹、肝脾大、浆膜炎等,与巨噬细胞活化综合征强相关.sJIA发病机制上主要与固有免疫异常有关,细胞因子白细胞介素(IL)-1、IL-6、IL-18及中性粒细胞、单核细胞、巨噬细胞等起重要作用.目前认为sJIA不是自身免疫性疾病,而是一个自身炎症性疾病.对sJIA发病机制的新认识带来治疗方法的进步,近年来,IL-1和IL-6拮抗剂靶向治疗取得了良好效果,为sJIA患儿远期预后带来了新希望.%Systemic juvenile idiopathic arthritis (sJIA) is systemic inflammatory disease classified as a subtype of juvenile idiopathic arthritis (JIA).Besides arthritis,it is characterised by systemic features such as spiking fever,skin rash,hepatosplenomegaly or serositis.It is becoming clear now that abnormalities in the innate immunity [cytokines such as interleukin (IL)-1,IL-6 and IL-18,and neutrophils and monocytes/macrophages rather than lymphocytes] play a major role in the pathogenesis of sJIA,distinguishing it from other JIA subtypes.Another distinctive feature of sJIA is its strong association with macrophage activation syndrome (MAS).Based on this,consensus is emerging that sJIA should be viewed as an autoinflammatory syndrome rather than a classic auto-immune disease.As a consequence of the progression in understanding the underlying mechanisms of sJIA,major changes in the management are evolving.Recently,remarkable improvement has been observed with IL-1 and IL-6 targeted therapies.These therapies might also change the long-term outcome of this disease.

  15. Evaluation of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis: single center experience over a one-year period

    Science.gov (United States)

    Barut, Kenan; Yücel, Gözde; Sinoplu, Ada Bulut; Şahin, Sezgin; Adroviç, Amra; Kasapçopur, Özgür

    2015-01-01

    Aim: This study aimed to evaluate the demographic, clinical, laboratory properties of patients with macrophage activation syndrome and treatment outcomes. Material and Methods: The data of the patients who were diagnosed with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis between June 2013–May 2014 were evaluated by screening patient records. Results: Ten patients with macrophage activation syndrome were followed up in one year. The mean age at the time of diagnosis was found to be 7.6±4.5 years. The most common clinical finding at presentation (80%) was increased body temperature. Hepatosplenomegaly was found in half of the patients. The most common hematological finding (90%) was anemia. The mean erythrocyte sedimentation rate was found to be 71.8±36.2 mm/h, whereas it was measured to be lower (31.2±25.2 mm/h) at the time of the diagnosis of macrophage activation syndrome. Increased ferritin level was found in all of our patients (the mean ferritin level was found to be 23 957±15 525 ng/mL). Hypertriglyceridemia was found in nine patients (90%). The mean triglyceride level was found to be 397±332 mg/dL. Systemic steroid treatment was administered to all patients. Cyclosporine A was given to eight patients (80%), canakinumab was given to four patients (40%) and anakinra was given to five patients (50%). Plasmapheresis was performed in two patients. Improvement was found in all patients except for one patient. The patient in whom no improvement was observed showed a chronic course. Conclusions: The diagnosis of macrophage activation syndrome should be considered in presence of sudden disturbance in general condition, resistant high fever and systemic inflammation findings in children with active rheumatic disease. Complete recovery can be provided with early and efficient treatment in macrophage activation syndrome which develops secondary to systemic juvenil idiopathic arthritis. PMID:26884689

  16. Biologic agents for the treatment of juvenile rheumatoid arthritis: current status.

    Science.gov (United States)

    Carrasco, Ruy; Smith, Judith A; Lovell, Daniel

    2004-01-01

    Biologic therapies, primarily anticytokine therapies, are being increasingly used in patients with juvenile rheumatoid arthritis (JRA). Levels of a variety of proinflammatory cytokines have been shown to be elevated in the peripheral blood and synovial fluid and tissue in children with JRA. In a blinded, randomized, controlled trial in children with severe, long-standing, polyarticular-course JRA not responsive to standard therapies, etanercept showed a statistically significantly greater response rate than placebo. Approximately 75% of these children responded to etanercept. Etanercept has been efficacious in 50-60% of children with active systemic JRA in open clinical trials with acceptable tolerance. Adverse events seen in children treated with etanercept have been similar in type and frequency to those reported in adults. Infliximab has been studied in several open clinical trials in both polyarticular and systemic JRA and found to, overall, have demonstrated efficacy in approximately 60% of patients. Approximately 3-5% of patients have demonstrated infusion reactions or frank allergic reactions and 9% developed new autoantibodies. Anakinra has been studied in children with polyarticular JRA. Approximately 65% of patients developed injection-site reactions and 68% demonstrated a response to the medication. Anakinra may have increased efficacy in systemic JRA. Interleukin (IL)-6 is highly related to the systemic disease manifestations in systemic JRA and two patients treated with a monoclonal antibody to the IL-6 receptor have demonstrated significant improvement with prolonged clinical control with continued treatment. A particular pediatric concern is the effect of immunosuppressive biologics in children who are exposed to or develop varicella. These children should be treated, both in terms of prophylaxis and aggressive antivaricella treatment, as for other immunosuppressed children. Anticytokine biologics have demonstrated great promise in the treatment of

  17. MRI assessment of bone marrow in children with juvenile idiopathic arthritis: intra- and inter-observer variability

    Energy Technology Data Exchange (ETDEWEB)

    Tanturri de Horatio, Laura; Barbuti, Domenico; Toma, Paolo [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Damasio, Maria Beatrice [Ospedale G. Gaslini, Department of Radiology, Genoa (Italy); Bracaglia, Claudia [Ospedale Pediatrico Bambino Gesu, Department of Paediatrics, Rome (Italy); Lambot-Juhan, Karen [Hopital Necker-Enfants Malades, Department of Radiology, Paris (France); Boavida, Peter [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Ording Mueller, Lil-Sofie [University Hospital North Norway, Department of Radiology, Tromsoe (Norway); Malattia, Clara [Ospedale G. Gaslini, Department of Pediatrics, Genoa (Italy); Rava, Lucilla [Ospedale Pediatrico Bambino Gesu, Department of Epidemiology, Rome (Italy); Rosendahl, Karen [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Haukeland University Hospital, Department of Pediatric Radiology, Bergen (Norway)

    2012-06-15

    Bone marrow oedema (BMO) is included in MRI-based scoring systems of disease activity in adults with rheumatoid arthritis. Similar systems in juvenile idiopathic arthritis (JIA) are lacking. To assess the reproducibility in a multi-centre setting of an MRI BMO scoring system in children with JIA. Seventy-six wrist MRIs were read twice, independently, by two experienced paediatric radiologists. BMO was defined as ill-defined lesions within the trabecular bone, returning high and low signal on T2- and T1-weighted images respectively, with or without contrast enhancement. BMO extension was scored for each of 14 bones at the wrist from 0 (none) to 3 (extensive). The intra-observer agreement was moderate to excellent, with weighted kappa ranging from 0.85 to 1.0 and 0.49 to 1.0 (readers 1 and 2 respectively), while the inter-observer agreement ranged from 0.41 to 0.79. The intra- and inter-observer intraclass correlation coefficients were excellent and satisfactory, respectively. The scoring system was reliable and may be used for grading bone marrow abnormality in JIA. The relatively large variability in aggregate scores, particularly between readers, underscores the need for thorough standardisation. (orig.)

  18. EFFECTIVENESS AND SAFETY OF INFLIXIMAB IN PATIENTS WITH EARLY AND LATE JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    Е.I. Alexeeva

    2010-01-01

    Full Text Available The article presents results of a study of effectiveness and safety of infliximab — monoclonal antibodies to the tumor necrotizing factor (TNF in treatment of 100 patients11 months — 17 years old with early and late articular types of juvenile rheumatoid arthritis. The duration of treatment was 3 months — 2 years. Infliximap was delivered intravenously by scheme: infusion on 0, 2nd, 6th weeks and then every 8th week. The single dose of infliximab in patients with early rheumatoid arthritis was 6.7 (5.5; 9.0 mg/kg, with late type — 6.0 (5.0; 7.0 mg/kg of body weight. 102 weeks of treatment with anti-TNF-agent provided development of clinical remission, decrease and normalization of laboratory tests of disease’s activity, total restoration of joint’s function, increase of quality of life (on 97% in patients with early type, and 72% 0 in ones with late type. The drug was abolished in 39 (39% of patients, 23% — due to the development of secondary inefficiency, and 11% — due to the development of unfavorable effects.Key words: children, early and late rheumatoid arthritis, treatment, infliximab.(Voprosy sovremennoi pediatrii — Current Pediatrics. – 2010;9(3:30-42

  19. Juvenile idiopathic arthritis: clinically relevant imaging in diagnosis and monitoring

    Energy Technology Data Exchange (ETDEWEB)

    Southwood, Tauny [Birmingham Children' s Hospital NHS Foundation Trust, Department of Rheumatology, Birmingham (United Kingdom)

    2008-06-15

    The role of plain radiographs in monitoring the disease process in JIA is being increasingly superseded by MRI. The use of ultrasound by clinicians is contentious, but has the potential to corroborate and supplement the clinical impression of individual joint inflammation and it can be very useful for localising intra-articular treatment at the bedside. There are exciting developments in MRS technology which may eventually allow in vivo evaluation of the acute inflammatory process, measurement of early responses to treatment and detection of residual inflammation. The aim of this article is to describe a clinician's view of the current role of imaging in the diagnosis and monitoring of JIA. (orig.)

  20. Temporomandibular joint involvement in Juvenile Idiopathic Arthritis : reliability and validity of a screening protocol for the rheumatologist

    NARCIS (Netherlands)

    Steenks, Michel H.; Giancane, G; de Leeuw, Rob R. J.; Bronkhorst, Ewald M.; van Es, Robert J. J.; Koole, Ron; van Bruggen, H. Willemijn; Wulffraat, NM

    2015-01-01

    Background: In Juvenile Idiopathic Arthritis (JIA) the temporomandibular joint (TMJ) can be involved leading to pain, dysfunction and growth disturbances of the mandible and associated structures. There may be value to a three minute screening protocol allowing the rheumatologist to detect TMJ invol

  1. High prevalence of methotrexate intolerance in juvenile idiopathic arthritis: development and validation of a methotrexate intolerance severity score

    NARCIS (Netherlands)

    Bulatovic, M.; Heijstek, M.W.; Verkaaik, M.; Dijkhuizen, E.H. van; Armbrust, W.; Hoppenreijs, E.P.A.H.; Kamphuis, S.; Kuis, W.; Egberts, T.C.; Sinnema, G.; Rademaker, C.M.A.; Wulffraat, N.M.

    2011-01-01

    OBJECTIVE: To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. METHODS: The MTX Intolerance Severity Score (MISS) que

  2. High prevalence of methotrexate intolerance in juvenile idiopathic arthritis : development and validation of a methotrexate intolerance severity score

    NARCIS (Netherlands)

    Bulatović, Maja; Heijstek, Marloes W; Verkaaik, Marleen; van Dijkhuizen, E H Pieter; Armbrust, Wineke; Hoppenreijs, Esther P A; Kamphuis, Sylvia; Kuis, Wietse; Egberts, Toine C G; Sinnema, Gerben; Rademaker, Carin M A; Wulffraat, Nico M

    2011-01-01

    OBJECTIVE: To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. METHODS: The MTX Intolerance Severity Score (MISS) que

  3. Facial morphology in children and adolescents with juvenile idiopathic arthritis and moderate to severe temporomandibular joint involvement

    DEFF Research Database (Denmark)

    Hsieh, Yuh-Jia; Darvann, Tron Andre; Hermann, Nuno V.

    2016-01-01

    Introduction: The aims of this study were to (1) assess lateral facial morphology in children and adolescents with juvenile idiopathic arthritis and moderate to severe temporomandibular joint (TMJ) involvement, (2) compare the lateral facial morphology of these subjects with and without TMJ...

  4. Sulfasalazine in the treatment of juvenile chronic arthritis - A randomized, double-blind, placebo-controlled, multicenter study

    NARCIS (Netherlands)

    Fiselier, TJW; Franssen, MJAM; Zwinderman, AH; ten Cate, R; van Suijlekom-Smit, LWA; van Luijk, WHJ; van Soesbergen, RM; Wulffraat, NM; Oostveen, JCM; Kuis, W; Dijkstra, PF; van Ede, CFP; Dijkmans, BAC

    1998-01-01

    Objective. To assess the efficacy, tolerability, and safety of sulfasalazine (SSZ) in the treatment of juvenile chronic arthritis (JCA). Methods. we conducted a 24-week randomized, placebo-controlled, double-blind, multicenter study of patients with active JCA of both oligoarticular and polyarticula

  5. Radiologic features in juvenile idiopathic arthritis - A first step in the development of a standardized assessment method

    NARCIS (Netherlands)

    van Rossum, MAJ; Zwinderman, AH; Dijkmans, BAC; van Soesbergen, RM; Fiselier, TJW; Franssen, MJAM; ten Cate, R; van Suijlekom-Smit, LWA; Wulffraat, NM; Kuis, W; van Luijk, WHJ; Oostveen, JCM; Dijkstra, PF

    2003-01-01

    Objective. To describe radiologic features of patients with juvenile idiopathic arthritis (JIA) in a standardized manner, to test the reliability and feasibility of this description, and to correlate these features with clinical signs as a first step in the development of a standardized assessment m

  6. Interleukin-1 receptor antagonist in neonates, children and adults, and in patients with pauci- and polyarticular onset juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Müller, K; Zak, M; Nielsen, S;

    2011-01-01

    patients with juvenile chronic arthritis (JCA) of the pauci- or polyarticular onset type. RESULTS: IL-1ra serum levels were found to differ significantly between the three age groups, being higher in neonates (569 pg/ml) than in children (70 pg/ml) and adults (177 pg/ml). IL-1ra production in E. coli...

  7. Autoantibodies to IL-1 alpha in sera from umbilical cords, children, and adults, and from patients with juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Müller, K; Hansen, M B; Zak, M;

    1996-01-01

    umbilical cords (n = 11), children (n = 45), and adults (n = 20), as well as in 51 patients with juvenile chronic arthritis (JCA) of pauciarticular (n = 34), polyarticular (n = 8), or systemic onset type (n = 9). RESULTS. The frequency of positive sera was significantly lower in children than in cord blood...

  8. Facial Asymmetry Evaluation in Juvenile Idiopathic Arthritis Patients Based On Cone-Beam Computed Tomography And 3D Photography

    DEFF Research Database (Denmark)

    Economou, Stalo; Stoustrup, Peter Bangsgaard; Kristensen, Kasper Dahl

    AIMS: The aim of the study was to assess the degree of and correlation between facial hard and soft tissue asymmetry in patients with juvenile idiopathic arthritis, identify valid soft tissue points for clinical examination and assess the smallest clinical detectable level of dentofacial asymmetr...

  9. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Ravelli, Angelo; Minoia, Francesca; Davì, Sergio; Horne, AnnaCarin; Bovis, Francesca; Pistorio, Angela; Aricò, Maurizio; Avcin, Tadej; Behrens, Edward M; De Benedetti, Fabrizio; Filipovic, Alexandra; Grom, Alexei A; Henter, Jan-Inge; Ilowite, Norman T; Jordan, Michael B; Khubchandani, Raju; Kitoh, Toshiyuki; Lehmberg, Kai; Lovell, Daniel J; Miettunen, Paivi; Nichols, Kim E; Ozen, Seza; Pachlopnik Schmid, Jana; Ramanan, Athimalaipet V; Russo, Ricardo; Schneider, Rayfel; Sterba, Gary; Uziel, Yosef; Wallace, Carol; Wouters, Carine; Wulffraat, Nico; Demirkaya, Erkan; Brunner, Hermine I; Martini, Alberto; Ruperto, Nicolino; Cron, Randy Q

    2016-01-01

    OBJECTIVE: To identify which laboratory tests that change over time are most valuable for the timely diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA). METHODS: A multistep process, based on a combination of expert consensus and analysis of

  10. Long-term ocular complications in aphakic versus pseudophakic eyes of children with juvenile idiopathic arthritis-associated uveitis

    NARCIS (Netherlands)

    Sijssens, K. M.; Los, L. I.; Rothova, A.; Schellekens, P. A. W. J. F.; van de Does, P.; Stilma, J. S.; de Boer, H. J.

    2010-01-01

    Aim To evaluate the long-term follow-up of aphakic and pseudophakic eyes of children with juvenile idiopathic arthritis (JIA)-associated uveitis with a special interest in whether intraocular lens implantation increases the risk of developing ocular complications. Methods Data were obtained from the

  11. Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis and systemic lupus erythematosus.

    Science.gov (United States)

    Aytaç, Selin; Batu, Ezgi Deniz; Ünal, Şule; Bilginer, Yelda; Çetin, Mualla; Tuncer, Murat; Gümrük, Fatma; Özen, Seza

    2016-10-01

    Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years. More SJIA patients had MAS at disease onset than SLE patients (53.6 vs. 16.7 %). Fever, high C-reactive protein and hyperferritinemia were present in all MAS episodes. Rash was less (p = 0.03), and fatigue was more frequent (p = 0.042) in SLE than SJIA patients. All received corticosteroids. Cyclosporine was given in 74.2 % of SJIA-MAS; 66.7 % of SLE-MAS episodes. Intravenous immunoglobulin, anakinra, or etoposide was administered during 67.7; 41.9; 32.3 % of SJIA-MAS and 33.3; 33.3; 50 % of SLE-MAS episodes, respectively. Plasmapheresis was performed during 41.9 % of SJIA-MAS and 33.3 % of SLE-MAS episodes. The mortality rate was 11.8 % (n = 4;3 SJIA, 1 SLE). Hepatosplenomegaly was more frequent (p = 0.005), and plasmapheresis was performed more frequently (p = 0.021) in the patients who died compared to the cured patients. The median duration between symptom onset and admission to our hospital was longer among the patients who died (16.5 vs. 7 days; p = 0.049). Our patients' characteristics were similar to the reported cases, but our mortality rate is slightly higher probably due to late referral to our center. Early diagnosis and effective treatment are crucial to prevent mortality.

  12. Rheumatoid arthritis associated interstitial lung disease: a review

    Directory of Open Access Journals (Sweden)

    Deborah Assayag

    2014-04-01

    Full Text Available Rheumatoid arthritis is a common inflammatory disease affecting about 1% of the population. Interstitial lung disease is a serious and frequent complication of rheumatoid arthritis. Rheumatoid arthritis associated interstitial lung disease (RA-ILD is characterized by several histopathologic subtypes. This article reviews the proposed pathogenesis and risk factors for RA-ILD. We also outline the important steps involved in the work-up of RA-ILD and review the evidence for treatment and prognosis.

  13. Rheumatoid Arthritis and Periodontal Disease. An Update.

    Science.gov (United States)

    Venkataraman, Archana; Almas, Khalid

    2015-01-01

    A review of the epidemiological, pathological and immunological relationships between two chronic inflammatory diseases: rheumatoid arthritis (RA) and periodontal disease (PD). RA is a chronic inflammatory disease of the joints, characterized by loss of connective tissue and mineralized structures, the so-called "synovial membrane." Periodontitis is the inflammatory destruction of the periodontal attachment and alveolar bone. While the etiology of these two diseases may differ, the underlying pathogenic mechanisms are similar. And it is possible that individuals manifesting both PD and RA may suffer from a unifying underlying systemic deregulation of the inflammatory response. There is an overproduction of a variety of cytokines and MMPs that appears to be common in both diseases. Oral health parameters should be more closely monitored in patients with RA, an autoimmune disease. Data suggest that periodontal therapies combined with routine RA treatments further improve RA status. Interventions to prevent, minimize or treat periodontitis in arthritis patients will definitely promise a better quality of life for these patients.

  14. Linkage Between Periodontal Disease and Rheumatoid Arthritis

    DEFF Research Database (Denmark)

    Holmstrup, Palle; Nielsen, Claus Henrik

    2016-01-01

    The past decades have significantly widened the perspectives of the chronic oral infectious disease known as periodontitis. The disease is regarded as a bacterial infection resulting in low-grade inflammation of the periodontal tissues, and both the associated release of pro-inflammatory mediators...... and the presence of bacteria in the periodontal pockets, which, as the result of daily procedures, may spread after penetration of the vasculature, are possible mediators of systemic consequences. The present chapter deals with the possible association of periodontitis with rheumatoid arthritis, which may possess...

  15. Contrast-enhanced MRI of the knee in children unaffected by clinical arthritis compared to clinically active juvenile idiopathic arthritis patients

    Energy Technology Data Exchange (ETDEWEB)

    Nusman, Charlotte M.; Hemke, Robert [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands); University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Benninga, Marc A.; Kindermann, Angelika [University of Amsterdam, Department of Pediatric Gastroenterology, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Schonenberg-Meinema, Dieneke; Berg, J.M. van den; Kuijpers, Taco W. [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Rossum, Marion A.J. van [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Reade, Department of Pediatric Rheumatology, Amsterdam (Netherlands); Maas, Mario [University of Amsterdam, Department of Radiology, Academic Medical Center, Amsterdam (Netherlands)

    2016-04-15

    To evaluate enhancing synovial thickness upon contrast-enhanced magnetic resonance imaging (MRI) of the knee in children unaffected by clinical arthritis compared with clinically active juvenile idiopathic arthritis (JIA) patients. A secondary objective was optimization of the scoring method based on maximizing differences on MRI between these groups. Twenty-five children without history of joint complaints nor any clinical signs of joint inflammation were age/sex-matched with 25 clinically active JIA patients with arthritis of at least one knee. Two trained radiologists, blinded for clinical status, independently evaluated location and extent of enhancing synovial thickness with the validated Juvenile Arthritis MRI Scoring system (JAMRIS) on contrast-enhanced axial fat-saturated T1-weighted MRI of the knee. Enhancing synovium (≥2 mm) was present in 13 (52 %) unaffected children. Using the total JAMRIS score for synovial thickening, no significant difference was found between unaffected children and active JIA patients (p = 0.091). Additional weighting of synovial thickening at the JIA-specific locations enabled more sensitive discrimination (p = 0.011). Mild synovial thickening is commonly present in the knee of children unaffected by clinical arthritis. The infrapatellar and cruciate ligament synovial involvement were specific for JIA, which - in a revised JAMRIS - increases the ability to discriminate between JIA and unaffected children. (orig.)

  16. Abatacept in the treatment of severe, longstanding, and refractory uveitis associated with juvenile idiopathic arthritis.

    Science.gov (United States)

    Tappeiner, Christoph; Miserocchi, Elisabetta; Bodaghi, Bahram; Kotaniemi, Kaisu; Mackensen, Friederike; Gerloni, Valeria; Quartier, Pierre; Lutz, Thomas; Heiligenhaus, Arnd

    2015-04-01

    Abatacept (ABA), a selective T cell costimulation modulator that binds to CD80 and CD86 on antigen-presenting cells, was investigated for its antiinflammatory effect in treating severe chronic uveitis associated with juvenile idiopathic arthritis (JIA). Our retrospective study was conducted by members of the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC). Patients with JIA who are receiving ABA treatment for active uveitis were included. In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, immunosuppressives, and at least 1 tumor necrosis factor-α inhibitor. A standardized protocol was used to document uveitis (MIWGUC) and arthritis. Baseline visit and visits at 3, 6, 9, and 12 months before and after ABA start were evaluated. Primary outcome measure was defined as achievement of uveitis inactivity; secondary outcome measures were tapering of corticosteroid and/or immunosuppressive treatment, and occurrence of complications. In all, 21 patients (16 female) with active uveitis (n = 21) and arthritis (n = 18) were included (mean age 11.8 ± 3.6 yrs). In 7 of 18 patients with active arthritis at baseline, inactivity was achieved following ABA treatment. Uveitis inactivity was achieved in 11 patients, but recurred later in 8 of them, and remained active in another 10 cases. Systemic corticosteroids or immunosuppression were tapered in 3 patients, but uveitis recurred in all of them during further followup. Ocular complications secondary to uveitis were present in 17 patients at baseline, while 3 patients developed new ocular complications during followup. A sustained response to ABA was uncommon in patients with severe and refractory uveitis.

  17. 77 FR 39714 - National Institute of Arthritis and Musculoskeletal and Skin Diseases; Notice of Closed Meeting

    Science.gov (United States)

    2012-07-05

    ... Institute of Arthritis and Musculoskeletal and Skin Diseases; Notice of Closed Meeting Pursuant to section... Institute of Arthritis and Musculoskeletal and Skin Diseases, Special Emphasis Panel, Clinical Trials... of Arthritis, Musculoskeletal and Skin Diseases, National Institutes of Health, 6701 Democracy...

  18. Chronic Lyme disease arthritis: review of the literature and report of a case of wrist arthritis.

    Science.gov (United States)

    Scerpella, T A; Engber, W D

    1992-05-01

    A case of Lyme arthritis with advanced degenerative changes localized to the midcarpal joint was treated with a limited wrist arthrodesis with relief of pain and improved function. Chronic Lyme arthritis occurs as the third stage of Lyme disease. Serologic testing and a history of a characteristic rash may be helpful in the diagnosis. Radiographic and histopathologic findings are nonspecific, with both degenerative and inflammatory characteristics. Intravenous antibiotics provide an effective treatment of chronic Lyme arthritis.

  19. Intravenous immunoglobulin therapy leading to dramatic improvement in a patient with systemic juvenile idiopathic arthritis and severe pericarditis resistant to steroid pulse therapy.

    Science.gov (United States)

    Aizawa-Yashiro, Tomomi; Oki, Eishin; Tsuruga, Kazushi; Nakahata, Tohru; Ito, Etsuro; Tanaka, Hiroshi

    2012-05-01

    A 7-year-old Japanese boy with a 4-month history of systemic juvenile idiopathic arthritis (s-JIA) experienced disease flare with spiking fever, exanthema and arthralgia. He then developed progressive dyspnea due to severe pericarditis, and proinflammatory hypercytokinemia was suspected. Methylprednisolone pulse therapy was ineffective and echocardiography showed massive pericardial effusion had persisted. Alternatively, subsequent intravenous immunoglobulin (IVIG) therapy resulted in dramatic resolution of the pericardial effusion, and his general condition significantly improved within a few days. This case report may lend further support the use of IVIG for selected patients with s-JIA and severe pericarditis.

  20. Juvenile chronic arthritis. Dentofacial morphology, growth, mandibular function and orthodontic treatment.

    Science.gov (United States)

    Kjellberg, H

    1995-01-01

    In children with Juvenile Chronic arthritis (JCA), temporomandibular joint involvement may lead to disturbances in dentofacial growth and mandibular function. The aim of this thesis was to study the dentofacial morphology, temporomandibular joint destruction and mandibular function in JCA children, and the relation between these factors. The intention was also to make a longitudinal study of the changes in facial morphology during growth and during treatment with functional appliances. Thirty-five JCA children, 12 boys and 23 girls, aged 7-16 years (mean 11.2 years), and the control groups, with either normal or distal occlusion, were studied by means of panoramic radiographs, lateral cephalograms, study casts, recordings of signs and symptoms of temporomandibular disorders (TMD), bite force and chewing characteristics. A method to evaluate the condylar height on panoramic radiographs was developed. Panoramic radiographs are found to be reliable for evaluation of the condylar height, provided the same panoramic machine is used. The dentofacial morphology in JCA children is characterized by a smaller, more retrognathic and steeper inclined mandible compared to that of healthy children with ideal occlusion. Compared to healthy children with distal occlusion, no difference in mandibular retrognathia could be demonstrated but the JCA children showed a smaller, more steeply inclined mandible. The presence and extent of condylar lesions play a significant role in the development of the facial morphology and also contribute to the facial heterogeneity among JCA children. During growth the JCA children without radiographically visible condylar lesions showed a growth pattern resembling that of healthy children with normal occlusion, while children with condylar lesions showed aggravation of the mandibular retrognathia and a tendency towards a backward-rotating growth pattern. The chewing movements in JCA children are restricted by the disease and by the presence of

  1. Juvenile Arthritis

    Science.gov (United States)

    ... ophthalmologist or orthopedic surgeon), and other health professionals (dentist, nutritionist or psychologist) as well as reach out ... American College of Rheumatology Committee on Communications and Marketing. This information is provided for general education only. ...

  2. Síndrome da ativação do macrófago em paciente com artrite idiopática juvenil poliarticular Macrophage activation syndrome in a patient with polyarticular juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Acir Rachid

    2004-10-01

    Full Text Available A linfohistiocitose hemofagocítica caracteriza-se por ativação e proliferação excessiva de linfócitos e macrófagos. Quando associada à artrite idiopática juvenil é também conhecida por "síndrome de ativação do macrófago", sendo uma complicação potencialmente fatal desta doença. Apresentamos o caso de uma mulher de 26 anos portadora de artrite idiopática juvenil (poliartrite, fator reumatóide negativo, com diagnóstico aos 13 anos, em uso de antiinflamatórios não esteroidais (diclofenaco, nimesulide. Admitida com quadro de resposta inflamatória sistêmica, febre, linfonodomegalia, esplenomegalia, anemia, trombocitopenia, hipofibrinogenemia, hiperferritinemia, hipertrigliceridemia e achados de hematofagocitose na medula óssea. Os autores discutem aspectos relacionados com a patogênese, diagnóstico e tratamento desta doença pouco conhecida.Hemophagocytic lymphohistiocytosis is characterized by massive lymphocyte and macrophage activation and proliferation. When observed in association with juvenile idiopathic arthritis it is also called "macrophage activation syndrome" being a potentially lethal complication of this disease. We report the case of a 26 years old woman with juvenile idiopathic arthritis (polyarthritis, rheumatoid factor negative since 13 years old, receiving nonsteroidal anti-inflammatory drugs (diclofenac, nimesulide. She was admitted with systemic inflammatory response, fever, lymph node enlargement, splenomegaly, anemia, thrombocytopenia, hypofibrinogenemia, hyperferritinemia, hypertriglyceridemia and bone marrow hemophagocytosis. Aspects related to pathogenesis, diagnosis and treatment of this little known disease are discussed.

  3. Preclinical lung disease in early rheumatoid arthritis.

    Science.gov (United States)

    Robles-Perez, Alejandro; Luburich, Patricio; Rodriguez-Sanchon, Benigno; Dorca, Jordi; Nolla, Joan Miquel; Molina-Molina, Maria; Narvaez-Garcia, Javier

    2016-02-01

    Early detection and treatment of lung disease in patients with rheumatoid arthritis (RA) may ameliorate disease progression. The objectives of this study were to investigate the frequency of asymptomatic lung abnormalities in early RA patients and the potential association of positive RA blood reactive biomolecules with lung involvement. A prospective observational study was performed in a cohort of patients with early RA (joint symptoms disease with a baseline chest radiograph (CR) and complete pulmonary function tests (PFTs). In those patients with lung abnormalities on the CR or PFTs, a high-resolution chest computed tomography scan (HRCT) was performed. We included 40 patients (30 women). Altered PFTs were detected in 18 (45%) of these patients. These cases had a diffusion lung transfer capacity of carbon monoxide (DLCO) of disease is present in up to 45% of early RA patients and can be determined by PFTs and ACPA levels.

  4. Increased Fas and Bcl-2 Expression on Peripheral Blood T and B Lymphocytes from Juvenile-Onset Systemic Lupus Erythematosus, but not from Juvenile Rheumatoid Arthritis and Juvenile Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Bernadete L. Liphaus

    2006-01-01

    Full Text Available Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE, juvenile rheumatoid arthritis (JRA and juvenile dermatomyositis (JDM. Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal–Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

  5. Increased Fas and Bcl-2 expression on peripheral blood T and B lymphocytes from juvenile-onset systemic lupus erythematosus, but not from juvenile rheumatoid arthritis and juvenile dermatomyositis.

    Science.gov (United States)

    Liphaus, Bernadete L; Kiss, Maria H B; Carrasco, Solange; Goldenstein-Schainberg, Claudia

    2006-01-01

    Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE), juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDM). Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC) were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal-Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

  6. Acute-onset opioid-induced hyperalgesia in a child with juvenile idiopathic arthritis.

    Science.gov (United States)

    Vijayan, Vini; Moran, Ryan; Elder, Melissa E; Sukumaran, Sukesh

    2012-10-01

    We describe a child with polyarticular juvenile idiopathic arthritis (JIA) presenting with severe diffuse pain refractory to nonsteroidal anti-inflammatory agents and high-dose opioids. Her JIA involved her knees and ankles and was mildly active on etanercept and nonsteroidal anti-inflammatory agents. At presentation, she complained of hip pain progressing to severe diffuse pain and allodynia involving her extremities. No abnormalities were seen in her laboratory parameters and imaging of her lower extremities. After appreciating no substantial benefit by increasing her opioids, her opioids were tapered and discontinued, and this was followed by significant alleviation in her pain, and a diagnosis of opioid-induced hyperalgesia (OIH) was made. Despite reports in adults, the phenomenon of OIH has been reported infrequently in children. To our knowledge, OIH has not been described in children with rheumatologic conditions. We recommend investigating the possibility of OIH when treating a child with JIA and severe refractory pain.

  7. [A case of pseudomembranous colitis in a juvenile rheumatoid arthritis patient taking methotrexate].

    Science.gov (United States)

    Yu, Ji Han; Kim, Na Young; Lee, Hae Min; Lee, Ha Ni; Ahn, Hyo Jun; Kim, Sang Woo; Choi, Kyu Yong

    2010-12-01

    Pseudomembranous colitis is mainly caused by antibiotics and Clostridium difficile infection. But conditions such as gastrointestinal surgery, antacid medication, anti-neoplastic agent or immunosuppressive agent which influences the normal flora of colon can induce colitis without the administration of any antibiotics. We experienced a 13 year-old male who was taking low-dose methotrexate for juvenile rheumatoid arthritis complained diarrhea and abdominal pain for 3 weeks. Sigmoidoscopic findings revealed diffuse patch yellowish pseudomembranes on the rectum. Histologic finding was compatible to pseudomembranous colitis. His symptom was improved after stop taking methotrexate and the administration of metronidazole. If a patient treated with immunosuppressive agents or antineoplastic agents complains diarrhea, fever or abdominal pain and has not improved with conservative care, pseudomembranous colitis should be taken into account as a differential diagnosis and prompt treatment is required for better prognosis.

  8. Cytokine balance and cytokine-driven natural killer cell dysfunction in systemic juvenile idiopathic arthritis.

    Science.gov (United States)

    Avau, Anneleen; Put, Karen; Wouters, Carine H; Matthys, Patrick

    2015-02-01

    Systemic juvenile idiopathic arthritis (sJIA) is a severe inflammatory childhood disorder, characterized by a specific pattern of systemic features and a typical cytokine profile. Patients are at risk to develop macrophage activation syndrome (MAS), an acute life-threatening condition defined by excessive proliferation and activation of macrophages and T cells. Defects of unknown cause in the natural killer (NK) cell cytotoxic capacity are presumed to underlie the pathogenesis of MAS and have been detected in sJIA patients. Here, we provide an overview of the cytokine profiles in sJIA and related mouse models. We discuss the influence of cytokines on NK cell function, and hypothesize that NK cell dysfunction in sJIA is caused by altered cytokine profiles.

  9. The Role of Gender in Juvenile Idiopathic Arthritis-Associated Uveitis

    Directory of Open Access Journals (Sweden)

    Ahmadreza Moradi

    2014-01-01

    Full Text Available Uveitis is a common complication of juvenile idiopathic arthritis (JIA affecting up to 30% of patients with JIA. Although the typical bilateral chronic anterior uveitis associated with the persistent and extended oligoarticular and polyarticular, rheumatoid factor negative variants of JIA occurs predominantly in girls, boys may be more commonly affected in the HLA-B27 positive, enthesitis variant of JIA. While female gender has been associated with the development of the chronic anterior uveitis in children with JIA, the clinical course of JIA-associated uveitis may be worse in boys than in girls. The purpose of this paper is to review the available published literature to determine the role of gender in the clinical presentation and outcomes of patients with JIA-associated uveitis.

  10. Usefulness of Adalimumab in the Treatment of Refractory Uveitis Associated with Juvenile Idiopathic Arthritis

    Science.gov (United States)

    García-De-Vicuña, Carmen; Díaz-Llopis, Manuel; Salom, David; Bou, Rosa; Díaz-Cascajosa, Jesus; Cordero-Coma, Miguel; Ortega, Gabriela; Ortego-Centeno, Norberto; Suarez-De-Figueroa, Marta; Cruz-Martínez, Juan; Fonollosa, Alex; Blanco, Ricardo; García-Aparicio, Ángel María; Benítez-Del-Castillo, Jose M.; Antón, Jordi

    2013-01-01

    Purpose. To assess the efficacy and safety of adalimumab in patients with juvenile idiopathic arthritis (JIA) and associated refractory uveitis. Design. Multicenter, prospective case series. Methods. Thirty-nine patients (mean [SD] age of 11.5 [7.9] years) with JIA-associated uveitis who were either not responsive to standard immunosuppressive therapy or intolerant to it were enrolled. Patients aged 13–17 years were treated with 40 mg of adalimumab every other week for 6 months and those aged 4–12 years received 24 mg/m2 body surface. Results. Inflammation of the anterior chamber (2.02 [1.16] versus 0.42 [0.62]) and of the posterior segment (2.38 [2.97] versus 0.35 [0.71] decreased significantly between baseline and the final visit (P uveitis and may reduce steroid requirement. PMID:24489444

  11. Determination of salicylic acid by HPLC in plasma and saliva from children with juvenile chronic arthritis.

    Science.gov (United States)

    Legaz, M E; Acitores, E; Valverde, F

    1992-12-01

    A high performance liquid chromatography (HPLC) method has been developed for measuring salicylic acid in the plasma and saliva of children with juvenile chronic arthritis (JCA). Samples were extracted with diethyl ether and, after drying, redissolved in methanol to be chromatographed. Quantitation of salicylic acid was performed by reverse phase HPLC on a spherisorb ODS-2 column, using methanol: water: acetic acid as mobile phase. Phenolic was monitored by absorbance at 237 nm. Linearity between the amount of mass injected and the response in the detector was determined. This method was applied to compare concentrations of salivary and plasma salicylic acid. The method also permitted the quantitation of salivary salicylate as a non-invasive, indirect method for monitoring the concentration of plasma salicylate in patients with JCA.

  12. Síndrome CINCA: um diagnóstico diferencial da artrite idiopática juvenil CINCA syndrome: a differential diagnosis of the juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Erica Naomi Naka

    2007-08-01

    Full Text Available A síndrome CINCA (crônico-infantil-neurológica-cutâneaarticular é uma enfermidade inflamatória multissistêmica rara, de início no período neonatal e caracterizada por febre, exantema cutâneo, envolvimento articular e do sistema nervoso central. É também conhecida pela literatura médica norte-americana como NOMID (doença multissistêmica inflamatória de início neonatal. Relatamos o caso de uma criança de 3 anos de idade admitida em nosso serviço com história de febre e exantema cutâneo desde o período neonatal. Apresentou crises convulsivas no sexto mês de vida e artrite simétrica de joelhos desde o nono mês. Na admissão, mostrava-se toxemiada, pálida, com um exantema maculopapular generalizado e artrite de joelhos e tornozelos. Apresentava ainda retardo de crescimento e desenvolvimento. Achados laboratoriais incluíram anemia, leucocitose, trombocitose, níveis elevados de proteína C reativa e meningite asséptica no exame do liquor. Os outros exames foram negativos. Os achados radiográficos dos joelhos, quadris e tornozelos foram anormais. A criança recebeu tratamento com antiinflamatório não hormonal, corticosteróide e metotrexato, com melhora apenas da dor e da febre. A etiologia da síndrome CINCA permanece desconhecida e nenhum tratamento tem se mostrado eficaz. Essa doença deve ser distinguida da forma sistêmica da artrite idiopática juvenil (AIJ, o principal diagnóstico diferencial.CINCA syndrome (chronic-infantile-neurological-cutaneousarticular is a rare multisystemic inflammatory disease with neonatal onset characterized by fever, skin rash, articular, and central nervous system involvement. This syndrome is known in the North American medical literature as infantile onset multisystem inflammatory disease (NOMID. We describe the case of a 3-yearold child admitted in our service with fever and skin rash since the neonatal period. She presented seizures at 6 months-old and bilateral arthritis of the

  13. [Interstitial lung disease in rheumatoid arthritis].

    Science.gov (United States)

    Froidevaux-Janin, Sylvie; Dudler, Jean; Nicod, Laurent P; Lazor, Romain

    2011-11-23

    Interstitial lung disease (ILD) is found in up to 30% of patients with rheumatoid arthritis (RA) and is clinically manifest in 5 to 10%, resulting in significant morbidity and mortality. The most frequent histopathological forms are usual interstitial pneumonia and nonspecific interstitial pneumonia. Another recently described presentation is combined pulmonary fibrosis and emphysema. Similarly to idiopathic pulmonary fibrosis, acute exacerbation of ILD may occur in RA and is associated with severe prognosis. Smoking is a known risk factor of RA and may also play a role in the pathogenesis of RA-associated ILD, in combination with genetic and immunologic mechanisms. Several treatments of RA may also lead to drug-induced ILD.

  14. Blood gene expression profiling in pediatric systemic lupus erythematosus and systemic juvenile idiopathic arthritis: from bench to bedside.

    Science.gov (United States)

    Gilbert, Mileka; Punaro, Marilynn

    2014-01-01

    Blood gene expression profiling has led to major advances in the field of rheumatology over the last few decades. Specifically, DNA microarray technology has been integral in increasing our knowledge of key players in the pathogenesis of some rare pediatric rheumatic diseases. Our group, using microarray analysis, identified the interferon (IFN) gene signature in pediatric systemic lupus erythematosus (SLE) and has published data that suggest high doses of intravenous corticosteroid treatment may have benefit over strictly oral regimens. Additionally, DNA microarray technology led to our discovery that the interleukin (IL)-1 gene signature is associated with systemic juvenile idiopathic arthritis (sJIA) and to the use of IL-1 blockade with anakinra in this disease. We also reported the biologic rationale for use of anakinra early in the disease course. Anakinra is now being used as first-line treatment in sJIA in multiple centers. Herein, we review how information obtained from blood gene expression profiling has changed our clinical practice.

  15. Carpal erosions in children with juvenile idiopathic arthritis: repeatability of a newly devised MR-scoring system

    Energy Technology Data Exchange (ETDEWEB)

    Boavida, Peter [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); Lambot-Juhan, Karen [Hospital Necker Enfants Malades, Department of Radiology, Paris (France); Ording Mueller, Lil-Sofie [Oslo University Hospital, Department of Radiology, Oslo (Norway); Damasio, Beatrice; Malattia, Clara [Ospedale Pediatrico Gaslini, Department of Rheumatology, Genoa (Italy); Tanturri de Horatio, Laura [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Owens, Catherine M. [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); UCL, Institute of Child Health, London (United Kingdom); Rosendahl, Karen [Haukeland University Hospital, Department of Radiology, Bergen (Norway); University of Bergen, Department of Clinical Medicine, Bergen (Norway)

    2015-12-15

    Juvenile idiopathic arthritis (JIA) is characterized by synovial inflammation, with potential risk of developing progressive joint destruction. Personalized state-of-the-art treatment depends on valid markers for disease activity to monitor response; however, no such markers exist. To evaluate the reliability of scoring of carpal bone erosions on MR in children with JIA using two semi-quantitative scoring systems. A total of 1,236 carpal bones (91 MR wrist examinations) were scored twice by two independent pediatric musculoskeletal radiologists. Bony erosions were scored according to estimated bone volume loss using a 0-4 scale and a 0-10 scale. An aggregate erosion score comprising the sum total carpal bone volume loss was calculated for each examination. The 0-4 scoring system resulted in good intra-reader agreement and moderate to good inter-observer agreement in the assessment of individual bones. Fair and moderate agreement were achieved for inter-reader and intra-reader agreement, respectively, using the 0-10 scale. Intra- and particularly inter-reader aggregate score variability were much less favorable, with wide limits of agreement. Further analysis of erosive disease patterns compared with normal subjects is required, and to facilitate the development of an alternative means of quantifying disease. (orig.)

  16. Oral health and orthodontic considerations in children with juvenile idiopathic arthritis: review of the literature and report of a case.

    Science.gov (United States)

    Synodinos, Philippos N; Polyzois, Ioannis

    2008-01-01

    Juvenile idiopathic arthritis (JIA) is a severe disease of childhood, which comprises a diverse group of distinct clinical entities of unclear aetiology. Some abnormality of the immune system is present in all JIA cases. In its most severe clinical form, JIA may show localised and/or systemic complications, including functional impairment of the affected sites. This may result in variable growth and developmental anomalies. In many JIA cases, where the temporomandibular joint (TMJ) is affected, mandibular growth may be restricted, thus leading to the development of mandibular hypoplasia and/or retrognathism. As a result, it is not uncommon for JIA patients to present with skeletal Class II and open bite malocclusions. Furthermore, in JIA cases with unilateral TMJ involvement, craniofacial asymmetry may occur. In such cases, early orthodontic intervention facilitates both the skeletal and the occlusal rehabilitation. Increased prevalence of dental caries and periodontal disease in JIA cases may be attributed to a combination of aetiological factors, including difficulties in executing good oral hygiene, unfavourable dietary practices and side effects from the long-term administration of medication. In addition, an association between periodontal disease and JIA has been reported based on their similar pattern of clinical disregulation of the inflammatory process. This paper presents a brief description of JIA, with special reference to dental health and orthodontic treatment considerations. In addition, a case is presented where the appropriate orthodontic intervention led to the establishment of a normally functioning, as well as an aesthetically pleasing, occlusion.

  17. Ultrasound in Arthritis.

    Science.gov (United States)

    Sudoł-Szopińska, Iwona; Schueller-Weidekamm, Claudia; Plagou, Athena; Teh, James

    2017-09-01

    Ultrasound is currently performed in everyday rheumatologic practice. It is used for early diagnosis, to monitor treatment results, and to diagnose remission. The spectrum of pathologies seen in arthritis with ultrasound includes early inflammatory features and associated complications. This article discusses the spectrum of ultrasound features of arthritides seen in rheumatoid arthritis and other connective tissue diseases in adults, such as Sjögren syndrome, lupus erythematosus, dermatomyositis, polymyositis, and juvenile idiopathic arthritis. Ultrasound findings in spondyloarthritis, osteoarthritis, and crystal-induced diseases are presented. Ultrasound-guided interventions in patients with arthritis are listed, and the advantages and disadvantages of ultrasound are discussed. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Profiling anti-cyclic citrullinated peptide antibodies in patients with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Tebo Anne E

    2012-08-01

    Full Text Available Abstract Background Anti-citrullinated protein/peptide antibodies (ACPA, have high specificity for rheumatoid arthritis (RA. Some children with juvenile idiopathic arthritis (JIA, phenotypically resemble RA and test positive for rheumatoid factor (RF a characteristic biomarker of RA. We investigated the prevalence of ACPA and its relationship to other serologic markers associated with RA in a well-characterized JIA cohort. Methods Cases were 334 children with JIA, 30 of whom had RF + polyarticular JIA. Sera from all cases and 50 healthy pediatric controls were investigated by ELISA at a single time point for anti-cyclic citrullinated peptide (anti-CCP IgG, RF IgM, IgA and IgG, anti-RA33 IgG, and antinuclear antibodies (ANA. Comparisons between cases and controls were made using Chi-square or Fisher exact tests and T-tests. Results The prevalence of RF was 8% among controls, and 12% among cases (ns. The prevalence of ACPA was 2% in controls and 14.3% in cases (OR 8.2, p Conclusions ACPAs are detectable in 14% of children with JIA. Children with positive ACPA but negative RF are frequent, and may define a distinct subset of children with JIA. ACPA testing should be included in the classification of JIA.

  19. Assessment of the Body Composition and Parameters of the Cardiovascular Risk in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2015-01-01

    Full Text Available The study was aimed to evaluate cardiovascular risk parameters, body mass index (BMI centiles for sex and age, and body fat percentage using the electric bioimpedance method in children with juvenile idiopathic arthritis (JIA. 30 children with JIA participated in the study. A control group included 20 children. Patients were well matched for the age and sex. The body mass and body fat percentage were determined using the segmental body composition analyser; the BMI centiles were determined. All patients had the following parameters determined: lipid profile, hsCRP, homocysteine, and IL-6. The intima media thickness (IMT was measured. Patients with JIA had significantly lower body weight, BMI, and the BMI centile compared to the control group. The IL-6 levels were significantly higher in patients with JIA compared to the control group. There were no differences between two groups with regard to the lipid profile, % content of the fat tissue, homocysteine levels, hsCRP, and IMT. Further studies are necessary to search for reasons for lower BMI and BMI centile in children with JIA and to attempt to answer the question of whether lower BMI increases the cardiovascular risk in these patients, similarly as in patients with rheumatoid arthritis (RA.

  20. Methotrexate for the treatment of juvenile idiopathic arthritis: process to approval for JIA indication in Japan.

    Science.gov (United States)

    Mori, Masaaki; Naruto, Takuya; Imagawa, Tomoyuki; Murata, Takuji; Takei, Syuji; Tomiita, Minako; Itoh, Yasuhiko; Fujikawa, Satoshi; Yokota, Shumpei

    2009-01-01

    Methotrexate (MTX), the primary treatment for the articular-type juvenile idiopathic arthritis (JIA), is effective and brings about radiological improvement. Patient compliance is good, and it is recognized that its known side effects, namely, disruption of liver function and induction of pulmonary lesions, are unlikely to be severe at the low MTX doses that are administered. In Japan, MTX was granted approval in 1999 by the then Ministry of Health and Welfare specifically for treating rheumatoid arthritis in adult patients, allowing it be generally used in medical institutions for patients having National Health Insurance. However, in the pediatric field, its use outside the indications has so far been unavoidable, and has been left to the discretion of the physician. Finally, at the present conference, expansion of the indications of MTX for JIA was approved in Japan. It is noteworthy that this expansion of indications was achieved without requiring clinical trials on children sponsored by the pharmaceutical company: it was achieved rather by collecting necessary information through ongoing efforts (including collection and analysis of information about approval status in foreign countries, adequate evidence from the literature, implementation of a clinical use survey in Japan, etc.). It also merits attention that the maximum dose (10 mg/m2) was set on the basis of pharmacokinetic data from children, rather than relying on the dosing method and dose for adults.