Juvenile Idiopathic Arthritis Research Papers (original) (raw)

This collaboration between the American College of Rheumatology and the American Association of Hip and Knee Surgeons developed an evidence-based guideline for the perioperative management of antirheumatic drug therapy for adults with rheumatoid arthritis (RA), spondyloarthritis (SpA) including ankylosing spondylitis and psoriatic arthritis, juvenile idiopathic arthritis (JIA), or systemic lupus erythematosus (SLE) undergoing elective total hip (THA) or total knee arthroplasty (TKA). A panel of rheumatologists, orthopedic surgeons specializing in hip and knee arthroplasty, and methodologists was convened to construct the key clinical questions to be answered in the guideline. A multi-step systematic literature review was then conducted, from which evidence was synthesized for continuing versus withholding antirheumatic drug therapy and for optimal glucocorticoid management in the perioperative period. A Patient Panel was convened to determine patient values and preferences, and the ...

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- Psychology, Rheumatology, Biomedical Engineering, Juvenile Idiopathic Arthritis

Background: Methotrexate has been implicated in a variety of lung complications, one of which is hypersensitivity pneumonitis. Hypersensitivity pneumonitis most often occurs within the first year of starting low-dose orally administered methotrexate. We present a case of methotrexate-induced hypersensitivity pneumonitis after 30 years of methotrexate use, which is the first case to be reported so far. Case presentation: A 77-year-old African American woman with a history of rheumatoid arthritis presented with progressively worsening shortness of breath and nonproductive cough. She was on a daily dose of 2.5 mg of methotrexate that had been orally administered for the last 30 years. A physical examination was significant for fever of 38.2°C (100.8°F), tachycardia, bilateral basal crackles, and oxygen saturation of 88% on room air. A laboratory work up was significant for normal white blood cell count, increased eosinophil count of 18.3%, and erythrocyte sedimentation rate of 111 mm/hour. Sputum cultures were negative for any bacterial pathogens including acid-fast bacilli. Influenza and respiratory syncytial viral infection were ruled out. A (1-3)-B-D-glucan assay (Fungitell®) was within normal limits. Pulmonary embolism was ruled out and echocardiography was normal. A chest X-ray showed hazy opacity with prominent reticulation within the upper lung fields bilaterally, right greater than the left with no pleural effusion. Lung computed tomography revealed nonspecific bilateral upper lung opacification. A pulmonary function test was significant for no obstruction, normal maximum voluntary ventilation, and no restriction, with mildly decreased diffusion. Methotrexate was stopped, and our patient was started on prednisone 60 mg orally administered daily with dramatic clinical and radiologic improvement. Conclusions: Methotrexate-induced hypersensitivity pneumonitis usually occurs in the initial few weeks to months of starting treatment with methotrexate; however, it can occur late during therapy too, and prompt diagnosis is crucial as it is a reversible condition when diagnosed early.

- by myint khaing
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- Pediatrics, Juvenile Idiopathic Arthritis, Hp, Child

Evidence-based medicine is critically dependent on three sources of information: a medical knowledge base, the patient's medical record and knowledge of available resources, including, where appropriate, clinical protocols. Patient data is often scattered in a variety of databases and may, in a distributed model, be held across several disparate repositories. Consequently addressing the needs of an evidence-based medicine community presents issues of biomedical data integration, clinical interpretation and knowledge management. This paper outlines how the Health-e-Child project has approached the challenge of requirements specification for (bio-) medical data integration, from the level of cellular data, through disease to that of patient and population. The approach is illuminated through the requirements elicitation and analysis of Juvenile Idiopathic Arthritis (JIA), one of three diseases being studied in the EC-funded Health-e-Child project.

The aims of this longitudinal study were to perform a comprehensive clinical evaluation of temporomandibular joint (TMJ) and to investigate the association between the clinical and magnetic resonance imaging (MRI) findings in the TMJs of patients with juvenile idiopathic arthritis (JIA). Seventy-five patients with JIA participated in this study. All patients underwent a rheumatological examination performed by a paediatric rheumatologist, a TMJ examination performed by a single dentist and an MRI with contrast of the TMJs. These examinations were scheduled on the same date. The patients were examined again 1 year later. Twenty-eight (37.3 %) patients reported symptoms at the first evaluation and 11 (14.7 %) patients at the second evaluation. In relation to signs, 35 (46.7 %) of the patients presented at least one sign at the first evaluation and 29 (38.7 %) at the second. Intense contrast enhancement of TMJ was significantly associated with disease activity (p < 0.001) at the fir...

To find the incidence of juvenile arthritis according to the ILAR and EULAR criteria within defined areas in the Nordic countries, and to study the validity of the ILAR and EULAR criteria from this perspective. A longitudinal, prospective, population based study with patients enrolled according to the ILAR and EULAR criteria. Twenty doctors in Iceland, Norway, Sweden, Denmark, and Finland collected data from the incidence cases within their catchment areas over a period of 1.5 years, beginning July 1, 1997. Clinical and serological data from the first year of the disease were collected. In the whole group of 315 patients, the incidence rate was 15 per 100,000 children/year (95% CI 13-17) according to the ILAR criteria, varying from 7 (1-13) in Iceland, 19 (7-31) and 23 (10-36) from 2 different regions in Norway, and 9 (5-12) and 16 (9-23) from 2 different areas in Denmark, to 15 (12-18) in Sweden and 21/100,000/year (15-26) in the Helsinki region in Finland. An early peak in distrib...

- by Lillemor Berntson
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- Rheumatology, Immunology, Juvenile Idiopathic Arthritis, Adolescent
- by P. Journeau
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- Juvenile Idiopathic Arthritis, Tumor necrosis factor-alpha

Background: Fatigue is common in patients with JIA and affects daily life negatively. We assessed the presence and severity of fatigue in patients with JIA, including factors presumed associated with fatigue (e.g., disease activity, disability, pain, physical activity, exercise capacity, and self-efficacy), and whether fatigue is related to participation in physical education classes, school attendance, and sports frequency. Methods: The current study used baseline data of 80 patients with JIA (age 8-13) who participated in an intervention aimed at promoting physical activity. Primary outcome measurements were fatigue, assessed using the Pediatric-Quality-of-Life-Inventory (PedsQl)-Fatigue-scale and energy level assessed using a VAS scale. Other outcome measurements were disease activity (VAS Physician Global Assessment Scale), disability (Childhood Health Assessment Questionnaire), physical activity (accelerometer), exercise capacity (Bruce treadmill test), self-efficacy (Childhood Arthritis Self-Efficacy Scale), and participation (self-report). Results: Sixty percent of patients with JIA suffered from daily low-energy levels; 27% suffered from very low-energy levels more than half the week. Low energy levels were best predicted by disability and low physical activity. Fatigue measured with the PEDsQL was higher compared to the control-population. Disability and low self-efficacy were main predictors of fatigue. Self-efficacy was a predictor of fatigue but did not act as moderator. Fatigue was a predictor for sports frequency but not for school attendance. Conclusion: Fatigue is a significant problem for JIA patients. Interventions aimed at reducing perceived disability, stimulating physical activity, and enhancing self-efficacy might reduce fatigue and thereby enhance participation.

- by Pieter Sauer
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- Perception, Quality of life, Juvenile Idiopathic Arthritis, Adolescent

The aim of this systematic review is to describe participation in social and physical leisure activities among children and adolescents with JIA, as well as identify potential determinants of leisure participation. Electronic databases were systematically searched for articles published up until June 2013 pertaining to participation in leisure activities among youth with JIA and other rheumatic diseases. Studies were included if they measured involvement in either social or physical leisure activities. Selection and quality appraisal of articles were completed independently by two authors. Eight hundred and ninety-three articles were found through electronic and reference search. One hundred and nine full articles were reviewed to assess for eligibility. Twelve articles met inclusion criteria and findings were reviewed. Most focused on describing participation in physical rather than social activities. Results suggest that youth with JIA participated less in both social and physical...

- by Sabrina Cavallo
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- Juvenile Idiopathic Arthritis, Adolescent, Multidisciplinary, Child

To develop and test the Parental PELICAN Questionnaire, an instrument to retrospectively assess parental experiences and needs during their child's end-of-life care. To offer appropriate care for dying children, healthcare professionals need to understand the illness experience from the family perspective. A questionnaire specific to the end-of-life experiences and needs of parents losing a child is needed to evaluate the perceived quality of paediatric end-of-life care. This is an instrument development study applying mixed methods based on recommendations for questionnaire design and validation. The Parental PELICAN Questionnaire was developed in four phases between August 2012-March 2014: phase 1: item generation; phase 2: validity testing; phase 3: translation; phase 4: pilot testing. Psychometric properties were assessed after applying the Parental PELICAN Questionnaire in a sample of 224 bereaved parents in April 2014. Validity testing covered the evidence based on tests o...

We report herein the results of the cross-cultural adaptation and validation into the Dutch language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Dutch CHAQ was fully validated with 3 forward and 3 backward translations while the CHQ was already published and therefore it was revalidated. A total of 180 subjects were enrolled: 100 patients with JIA (17% systemic onset, 31% polyarticular onset, 18% extended oligoarticular subtype, and 34% persistent oligoarticular subtype) and 80 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA...

- by Janjaap van der Net
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- Psychometrics, Quality of life, Juvenile Idiopathic Arthritis, Language

Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Early diagnosis and effective therapy of SJIA is desirable, so that complications are avoided. The PRO-KIND initiative of the German Society for Pediatric Rheumatology (GKJR) aims to define consensus-based strategies to harmonize diagnostic and therapeutic approaches in Germany. We analyzed data on patients diagnosed with SJIA from 3 national registries in Germany. Subsequently, via online surveys and teleconferences among pediatric rheumatologists with a special expertise in the treatment of SJIA, we identified current diagnostic and treatment approaches in Germany. Those were harmonized via the formulation of statements and, supported by findings from a literature search. Finally, an in-person consensus conference using nominal group technique was held to further modify and consent the statements. Up to 50% of patients diagnosed with SJIA in Germany do not fulfill the In...

- by Elke Lainka
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- Juvenile Idiopathic Arthritis, Germany, Child, Consensus

Objective. To report on referral patterns of primary physicians for children subsequently diagnosed with juvenile idiopathic arthritis (JIA) and to identify predictors of delayed referral to a pediatric rheumatology center. Methods. A retrospective cohort study of consecutive patients with JIA referred to a pediatric rheumatology center over a 15-year period was performed. Variables included age, sex, JIA subtype, the physician's subspecialty, and distance to the pediatric rheumatology center. Outcome parameters were the time to first presentation to a primary physician, the time to the first rheumatology visit, and the total time to referral. Putative predictors were evaluated by analysis of variance, resulting in regression models.

- by Susanne Benseler
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- Psychology, Pediatrics, Rheumatology, Immunology

- by Antonia Chen
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- Psychology, Rheumatology, Biomedical Engineering, Juvenile Idiopathic Arthritis

We report the results of the cross-cultural adaptation and validation into the German language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The German CHAQ was fully validated with 3 forward and 3 backward translations, while the CHQ has already been published and therefore it was revalidated. A total of 197 subjects were enrolled: 142 patients with JIA (5% systemic onset, 13% polyarticular onset, 8% extended oligoarticular subtype, and 74% persistent oligoarticular subtype) and 55 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA ...

- by Ivan Foeldvari
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- Psychometrics, Quality of life, Juvenile Idiopathic Arthritis, Language

Objective. To look for oligosaccharide structural variants of IgG that may be unique to specific rheumatic diseases.

Reçu le 22 juin 2010 ; accepté le 14 février 2011 Disponible sur Internet le 1 septembre 2011 MOTS CLÉS Enfant ; Uvéite ; Arthrite juvénile idiopathique Résumé But. -Analyser les particularités épidémiologiques, cliniques, étiologiques et évolutives des uvéites de l'enfant. Patients et méthodes. -Étude rétrospective de 49 enfants atteints d'uvéite (soit 8,65 % de l'ensemble des uvéites) entre janvier 2000 et décembre 2009. Tous les enfants ont bénéficié d'un bilan ophtalmologique complet et d'un bilan étiologique avec un recul allant de six mois à sept ans.

- by Nabil Youssef
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- Juvenile Idiopathic Arthritis, Adolescent, Visual acuity, Child
- by Cinzia Maspero
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- Dentistry, Juvenile Idiopathic Arthritis, Child, Follow-up studies
- by Alberto Martini
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- Inflammation, Treatment Outcome, Juvenile Idiopathic Arthritis, Adolescent
- by S. Peltoniemi and +1
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- Immunology, Juvenile Idiopathic Arthritis, Adolescent, Child

Arthritis is the leading cause of chronic illness in the United States. Seventy-two percent of the adults aged 55 years and older with arthritis report sleep difficulties. This review discusses sleep disorders associated with rheumatoid arthritis, juvenile rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, scleroderma, Behcet's disease, seronegative spondyloarthropathies, osteoarthritis, sarcoidosis, and fibromyalgia. We describe the inter-relationship between sleep complaints, disease activity, depression, sleep deprivation, and cytokines. An algorithm for evaluation and treatment of sleep disorders associated with rheumatologic diseases is proposed.

- by Vivien Abad
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- Algorithms, Pain, Treatment, Cytokines

Air pollution consists of a heterogeneous mixture of gasses and particles that include carbon monoxide, nitrates, sulfur dioxide, ozone, lead, toxic by-product of tobacco smoke and particulate matter. Oxidative stress and inflammation induced by inhaled pollutants may result in acute and chronic disorders in the respiratory system, as well as contribute to a state of systemic inflammation and autoimmunity. This paper reviews the mechanisms of air contaminants influencing the immune response and autoimmunity, and it focuses on studies of inhaled pollutants triggering and/or exacerbating rheumatic diseases in cities around the world. Remarkably, environmental factors contribute to the onset of autoimmune diseases, especially smoking and occupational exposure to silica in rheumatoid arthritis and systemic lupus erythematosus. Other diseases such as scleroderma may be triggered by the inhalation of chemical solvents, herbicides and silica. Likewise, primary vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) may be triggered by silica exposure. Only few studies showed that air pollutants could trigger or exacerbate juvenile idiopathic arthritis and systemic lupus erythematosus. In contrast, no studies of tropospheric pollution triggering inflammatory myopathies and spondyloarthropathies were carried out. In conclusion, air pollution is one of the environmental factors involved in systemic inflammation and autoimmunity. Further studies are needed in order to evaluate air pollutants and their potentially serious effects on autoimmune rheumatic diseases and the mechanisms involved in the onset and the exacerbation of these diseases.

- by Alfésio Braga
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- Immunology, Immune response, Inflammation, Air pollution

A generation ago, children with arthritis faced a lifetime of pain and disability. Today, there are a multitude of treatment options, including a variety of biologics targeting key cytokines and other inflammatory mediators. While non-steroidal anti-inflammatory drugs and corticosteroids were once the mainstay of therapy, they are now largely used as bridge or adjunctive therapies. Among the conventional disease-modifying anti-rheumatic drugs, methotrexate remains first-line therapy for most children with juvenile idiopathic arthritis (JIA) due to its long track record of safety and effectiveness in the management of peripheral arthritis. Sulfasalazine and leflunomide may also have a secondary role. The tumor necrosis factor inhibitors (TNFi) have shown tremendous benefit in children with polyarticular JIA and likely in enthesitis-related arthritis and psoriatic JIA as well. There may be additional benefit in combining TNFi with methotrexate. Abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear. For the treatment of systemic JIA, while the TNFi are of less benefit, blockade of interleukin-1 or interleukin-6 is highly effective. Additionally, interleukin-1 blockade appears to be effective treatment of macrophage activation syndrome, one of the most dangerous complications of JIA; specifically, anakinra in combination with cyclosporine and corticosteroids may obviate the need for cytotoxic approaches. In contrast, methotrexate along with the TNFi and abatacept are effective agents for the management of uveitis, another complication of JIA. Overall, the biologics have demonstrated an impressive safety record in children with JIA, although children do need to be monitored for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of malignancy. Finally, there may be a window of opportunity during which children with JIA will demonstrate most optimal responses to aggressive therapy, underscoring the need for rapid diagnosis and initiation of treatment.

- by Randy Cron
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- Treatment Outcome, Juvenile Idiopathic Arthritis, Child, Clinical Sciences

Background-Autologous hematopoietic cell transplantation (HCT) improves survival in patients with multiple myeloma, but disease progression remains a challenge. Allogeneic HCT (alloHCT) has the potential to reduce disease progression through graft-versus-myeloma effects. The aim of the BMT CTN 0102 trial was to compare outcomes of autologous HCT (autoHCT) Krishnan et al.

- by Marc B
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- Treatment Outcome, Juvenile Idiopathic Arthritis, Leukemia, Risk assessment

Juvenile rheumatoid arthritis is a common chronic inflammatory disease in the childhood and it can differentiate rarely into spondiloarthropaties. It is one of the important causes of chronic pain and disability. Some of the drugs used for the treatment have immunosupressive activity. One of the serious side-effects of immunosupressive treatment is activation of opportunistic pathogens. Hepatitis B virus (HBV) is one of these pathogens, and the rate of carriers in the population is considerably high. It can cause liver damage and death if reactivated. Thus, the management of oppotunistic pathogens becomes a complex issue when treating rheumatic diseases with immunosupressive drugs. In this case report, we present a juvenile rheumatoid arthritis patient whose liver enzymes raised while he was under treatment and afterwards HBV reactivation was determined as the cause. When reactivation was detected, we started controlled antiviral therapy. We achieved successful clinical and laboratory results after adding biological agents to the treatment. Careful evaluation of the patients who have indication for immunosuppressive agents and regular follow-up in case of infection may be protective from severe morbidity and/or mortality.

A study of 56 patients (55 female, one male) with 100 reconstructed temporomandibular joints (TMJ) using the Techmedica custom-made total joint system (Techmedica Inc, Camarillo, CA) is presented. The patients ranged in age from 15 to 61 years (average, 39 years) and had 16 to 46 months' follow-up (average 30 months). Outcome groups were categorized as good, fair, or poor, based on clinical assessment. Results show that 35 patients (63%) with 58 joints (58%) had a good outcome, and 13 patients (23%) with 26 joints (26%) had a fair outcome, and 8 patients (14%) with 16 joints (16%) had a poor outcome. Patients with one or no previous temporomandibular joint surgeries had 86% in the good group, 14% in the fair group, and no patients in the poor group. In patients with two or more previous surgeries, the success rate decreased to 55% with good results, 26% with fair results and 19% with poor results. Long-term morbidity included five ramus prostheses that were removed or revised. Seventeen patients (30%) received further operations because of heterotopic bone formation, fibrosis, calcification, inflammation, and/or pain which occurred mostly in patients with previous Proplast/Teflon (Vitek, Inc, Houston, TX) implants. Continued pain has been associated with the poor group, which may be related to problems such as cervical neuropathy, sympathetic dystrophy, a residual inflammatory or immunologic reaction to Proplast/Teflon or silastic particles, fibrosis, calcification, heterotopic bone, or other unidentified factors.

- by Larry Wolford
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- Dentistry, Titanium, Treatment Outcome, Juvenile Idiopathic Arthritis

- by David Vesole
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- Treatment Outcome, Juvenile Idiopathic Arthritis, Leukemia, Risk assessment

Objectives To compare the persistence of measles, mumps, rubella, diphtheria and tetanus antibodies between patients with juvenile idiopathic arthritis (JIA) and healthy controls. Methods Measles, mumps, rubella (MMR) and diphtheria-tetanus toxoid (DT)-specifi c immunoglobulin G antibody concentrations were compared between 400 patients with JIA and 2176 healthy controls aged 1-19 years. Stored patient samples from the period 1997-2006 were obtained from one Dutch centre for paediatric rheumatology. Healthy control samples had been evaluated previously in a nationwide cohort. Participants had been vaccinated according to the Dutch immunisation programme. Antibody concentrations were measured by ELISA (MMR) or multiplex immunoassay (DT). Results Corrected for age and the number of vaccinations, lower vaccine-specifi c geometric mean antibody concentrations (GMC) were found in patients with JIA against mumps, rubella, diphtheria and tetanus (p≤0.001). Measles-specifi c GMC were higher (p<0.001) compared with healthy controls. The prevalence of protective antibody concentrations was signifi cantly lower in patients for mumps (OR 0.4; 95% CI 0.3 to 0.6), rubella (OR 0.4; 0.3 to 0.7), diphtheria (OR 0.1; 0.06 to 0.2) and tetanus (OR 0.1; 0.05 to 0.3). Seroprotection rates against measles did not differ between patients and healthy controls (OR 1.4; 0.8 to 2.5). Methotrexate and glucocorticosteroid use did not affect pathogen-specifi c GMC or seroprotection rates. Conclusions Patients with JIA had lower antibody concentrations and seroprotection rates than healthy controls against mumps, rubella, diphtheria and tetanus, but not measles. In these patients, regular assessment of antibody concentrations and further research on responses to other (booster) vaccines are warranted.

- by Pieter Van Gageldonk
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- Epidemiology, Immunology, Juvenile Idiopathic Arthritis, Adolescent

Antoni Gaudí i Cornet, Catalan architect and one of the most important visual artists of the 19th and 20th centuries, suffered from a recurrent and often persistent arthritis since he was 6 years old. His diagnosis is uncertain but juvenile idiopathic arthritis is most likely. He coped successfully with his rheumatic illness during his life. It is proposed that his arthritis may have influenced him to the development of 2 of his major skills: observation power and analysis of nature.

- by César Díaz-torné
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- Art, Juvenile Idiopathic Arthritis, Spain, Clinical Sciences

Established criteria for diagnosis of juvenile rheumatoid arthritis require consideration of a number of other joint arthropathies and arthritides. In this pictorial essay, we present an approach to those common and uncommon disorders... more

- by Paul Babyn
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- Juvenile Idiopathic Arthritis, Rheumatoid Arthritis, Adolescent, Child

The aim of the study was to assess possible correlations between the clinical parameters of temporomandibular joint (TMJ) arthritis and pathologic MRI findings of the TMJ in patients affected by juvenile idiopathic arthritis (JIA), and the effect of a functional orthodontic therapy on the evolution of TMJ disorders. A prospective clinical and nuclear magnetic resonance (NMR) investigation was conducted on a sample of 53 patients (41 female, 12 male) with JIA, treated for 24 months with an Andresen appliance. The involvement of TMJ was defined by clinical and radiological signs. NMR assessments were performed in closed and maximum opening mouth position before (T0) and at the end of functional orthodontic therapy (T1). Fifteen patients showed physical and radiologic TMJ abnormalities. Changes were not uniformly distributed among the different JIA subtypes. Patients with poliarticular JIA (≥5 peripheral joints affected) showed more destructive bony changes. No correlation existed betw...

- by Joel Kovarsky
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- Juvenile Idiopathic Arthritis, Rheumatoid Arthritis, Adolescent, Sarcoidosis

Pediatric rheumatic diseases with predominant musculoskeletal involvement such as juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis(JDM) can cause considerable physical functional impairment and significantly affect the children's quality of life (QOL). Physical function, QOL, health-related QOL (HRQOL) and health status are personal constructs used as outcomes to estimate the impact of these diseases and often used as proxies for each other. The chronic, fluctuating nature of these diseases differs within and between patients, and complicates the measurement of these outcomes. In children, their growing needs and expectations, limited use of age-specific questionnaires, and the use of proxy respondents further influences this evaluation. This article will briefly review the different constructs inclusive of and related to physical function, and the scales used for measuring them. An understanding of these instruments will enable assessment of functional outcome in clinical studies of children with rheumatic diseases, measure the impact of the disease and treatments on their lives, and guide us in formulating appropriate interventions.

Methods. A total of 136 patients with systemiconset JIA were studied, including 98 patients from the British Paediatric Rheumatology Study Group's National Repository for JIA and 38 patients who were followed up at the IRCCS Policlinico San Matteo (Pavia, Italy) and the IRCCS G. Gaslini (Genoa, Italy). The MIF-173 polymorphism was genotyped using SnaPshot ddNTP primer extension and capillary electrophoresis. MIF levels were measured by enzyme-linked immunosorbent assay. The evaluation of the association of the MIF-173 polymorphism with outcome was performed only in Italian patients who were followed up for >5 years, by analyzing retrospectively 1) the number of joints with active arthritis and the number of joints with limited range of motion; 2) the score, at the last visit, on the Italian version of the Childhood Health Assessment Questionnaire (C-HAQ); and 3) data concerning the treatment regimens during the disease course.

- by L. Holt
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- Immunology, Polymorphism, Treatment Outcome, Juvenile Idiopathic Arthritis

Park-Harris growth arrest lines; Systemic-onset juvenile idiopathic arthritis

- by Alina Boteanu and +1
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- Radiology, Juvenile Idiopathic Arthritis

Objective. To localize the gene for familial recurrent arthritis via a genome-wide linkage scan in an extended kindred with the disease. Methods. A 3-generation family in which 9 members were diagnosed with juvenile idiopathic arthritis (JIA) was ascertained. In this family the disease was of very early onset and included episodic inflammation leading to eventual destruction of joints, muscle, and skin. We treated this disorder as a distinct clinical entity that we have named "familial recurrent arthritis." A genome-wide linkage scan with polymorphic microsatellites at 10-15-cM resolution was initiated.

- by Anne Bowcock
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- Immunology, Juvenile Idiopathic Arthritis, Child, Pedigree

The objective of this report is to explore the balance between serum and synovial fluid levels of interleukin (IL)-18 in children with juvenile idiopathic arthritis (JIA). Blood samples were obtained from 81 children with JIA and 18 control children. Synovial fluid samples were collected from 16 children with oligoarticular JIA. Concentrations of IL-18 were determined using commercial kit. Patients with systemic JIA had higher serum levels of IL-18 than patients with other forms of JIA or control children, both during the active (median, range: 6,240, 1,600-78,750 pg/ml) and inactive (1,615, 513-3,270 pg/ml) phase of disease [analysis of variance (ANOVA), P<0.05). Levels of IL-18 in sera of children with oligoarticular JIA (255, 89-4,342 pg/ml) were similar to the respective synovial fluid levels (217, 89-1,245 pg/ml). Serum levels of IL-18 were proportional to the erythrocyte sedimentation rate and levels of C-reactive protein, but inversely proportional to the haemoglobin levels. IL-18 appears to be an important mediator of systemic JIA, while it seems of a lesser relevance in pathogenesis of other JIA forms. Therefore, inhibition of IL-18 might be a base for a successful biological therapy for systemic JIA.

- by Ivan Malčić and +2
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- Juvenile Idiopathic Arthritis, Adolescent, Child, Clinical

Baydogan SN, Tarakci E, Kasapcopur O: Effect of strengthening versus balanceproprioceptive exercises on lower extremity function in patients with juvenile idiopathic arthritis: a randomized, single-blind clinical trial. Am J Phys Med... more

- by Ozgur Kasapcopur
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- Juvenile Idiopathic Arthritis, Adolescent, Exercise therapy, Muscle strength

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and adolescents. Immunomodulatory drugs are used frequently in its treatment. Using the nominal group technique (NGT) and Delphi method, we created a multidisciplinary, evidence-and consensus-based treatment guideline for JIA based on a systematic literature analysis and three consensus conferences. Conferences were headed by a professional moderator and were attended by representatives who had been nominated by their scientific societies or organizations. 15 statements regarding drug therapy, symptomatic and surgical management were generated. It is recommended that initially JIA is treated with NSAID followed by local glucocorticoids and/or methotrexate if unresponsive. Complementing literature evidence with long-standing experience of caregivers allows creating guidelines that may potentially improve the quality of care for children and adolescents with JIA.

- by Ivan Foeldvari
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- Immunology, Evidence Based Medicine, Juvenile Idiopathic Arthritis, Adolescent

This review summarizes the immunological consequences of biological therapies used in juvenile idiopathic arthritis (JIA). For every frequently used biological agent the characteristics are clearly specifi ed (molecular target, isotype, registered indication for JIA, route of administration, half-life, contraindication, very common side eff ects, expected time of response and average cost in the fi rst year). The emphasis of this review is on the immunological side eff ects that have been encountered for every separate agent in JIA populations. For each agent these adverse events have been calculated as incidence per 100 patientyears for the following categories: serious infections, tuberculosis, malignancies, response to vaccination, new-onset autoimmune diseases and development of anti-drug antibodies. There are large diff erences in side eff ects between various agents and there is a clear need for an international and standardized collection of post-marketing surveillance data of biologicals in the vulnerable group of JIA patients. Such an international pharmacovigilance database, called Pharmachild, has now been started.

- by Joost Swart
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- Immunology, Juvenile Idiopathic Arthritis, Child, Clinical Sciences

Uveitis is an inflammatory disorder involving inflammation of the uveal tract. It is classified as anterior, intermediate, posterior or panuveitis, depending on the part of eye affected by the inflammatory process. In children, non-infectious, chronic uveitis is a relatively uncommon but serious disease, with the potential for significant long-term complications and possible blindness. Although frequently associated with an underlying systemic disease, e.g. juvenile idiopathic arthritis (JIA), a significant number of cases in children show no associated signs or symptoms, and are labelled as idiopathic. Taking into account this evidence, an anti-inflammatory therapy based on an immuno-modulatory approach seems a reasonable strategy for non-infectious chronic uveitis, in children as well as in adults. Due to a lack of controlled studies regarding uveitis in children, immunosuppressive drugs are supported only at evidence level III. The aim of this review is to report currently available medical strategies for treatment of childhood sight-threatening chronic uveitis; in addition, a step-by-step approach to the use of immunosuppressants in this context is suggested.

- by Luca Cantarini
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- Immunology, Blindness, Autoimmunity, Juvenile Idiopathic Arthritis
- by Tim Rapley
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- Pediatrics, Rheumatology, Immunology, Juvenile Idiopathic Arthritis

1 O'Brien C, Duvall-Young J, Brown M, Short C, Bone M. Electrophysiology of type II mesangiocapillary glomerulonephritis with associated fundus abnormalities. Br J Ophthalmol 1993; 77:778-80. 2 Duvall-Young J, Short CD, Raines MF, Gokal R, Lawler W. Fundus changes in mesangiocapillary glomerulonephritis type II: clinical and fluorescein angiographic features. Br J Ophthalmol 1989;73:900-6. 3 Duvall-Young J, MacDonald MK, McKechnie NM. Fundus changes in (type II) mesangiocapillary glomerulonephritis simulating drusen: a histopathological report. Br J Ophthalmol 1989; 73:297-302. 4 Puliafito CA, Hee MR, Lin CP, et al. Imaging of macular diseases with optical coherence tomography. Ophthalmology 1995;102:217-29. 5 Azzolini C, Pierro L, Codenotti M, Brancato R. OCT images and surgery of juvenile macular retinoschisis. Eur J Ophthalmol 1997;81:196-200.

- by Susan Lightman
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- Juvenile Idiopathic Arthritis, Adolescent, Chronic Disease, Prednisolone

Given the generally poor outcome of advanced B cell chronic lymphocytic leukemia, experimental approaches are warranted, especially for younger patients in whom classical treatments have failed. We therefore conducted a prospective single-center study, using polychemotherapy (ESHAP) to prepare patients for hematopoietic stem cell collection and autologous stem cell transplantation as consolidation therapy. Twenty patients entered the study. An adequate response

- by Humberto González
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- Treatment Outcome, Juvenile Idiopathic Arthritis, Leukemia, Risk assessment

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 metabolic syndrome and obesity, and patients with diseases characterized by the presence of chronic inflammation, such as JIA, could represent special risk groups. Glucocorticoids are used routinely in the management of the inflammation of JIA, in high doses and long-term. Long-term use of the glucocorticoids can cause to insulin resistance, hypertension, and obesity, increasing the risk of metabolic syndrome. The aim of this study is to review the literature on the prevalence of different components of metabolic syndrome in patients with JIA. We observed that the data on metabolic syndrome and its components in those patients are very scarce and more studies needed, in view of the potential increased risk of cardiovascular disease.