Bibliography August 2006

1. Anonymous, Tocilizumab in juvenile chronic arthritis|, 2006
   Organism:

2. AGGARWAL P, AGGARWAL A, GUPTA Set MISRA R: Osteopenia is common in adult male patients with active juvenile idiopathic arthritis, J.Rheumatol., Vol. 33(8), 1642-1645., 2006
   Organism:Department of Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaFAU - Aggarwal, Parshant
   Abstract:OBJECTIVE: To assess the prevalence of osteopenia in Southeast Asian men with active juvenile idiopathic arthritis (JIA) and to identify predictors of reduced bone mineral density (BMD). METHODS: BMD of 30 men with active JIA and 23 healthy men was assessed by dual energy x-ray absorptiometry scans. Clinical variables that influence bone mass were also analyzed. T scores were calculated based on Caucasian normative data. RESULTS: Absolute BMD (g/cm(2)) was significantly lower in men with active JIA compared to controls at all measured sites, i.e., lumbar spine (p = 0.018), hip (p = 0.018), and distal third of forearm (p = 0.044). More subjects in the JIA group had low BMD (T score <or= -1.0) than controls at hip (22/30 vs 9/23; p < 0.05) and distal third of forearm (27/30 vs 10/23; p < 0.001), while at lumbar spine region the difference was not statistically significant (22/30 vs 13/23). A significant negative correlation of BMD was found with joint deformities, limitation of joint movement, Health Assessment Questionnaire score, and erythrocyte sedimentation rate. BMD at the hip and distal third of forearm was significantly lower in patients having arthritis at these sites. A positive correlation of BMD was found with body mass index. CONCLUSION: A majority of Southeast Asian men with active JIA have reduced BMD. More patients in our cohort had low BMD compared to reports from Western countries. This finding may be attributed to use of Caucasian normative data, uncontrolled disease activity, severity of disease, poor nutritional status, or an ethnic variation

3. ARABSHAHI Bet CRON RQ: Temporomandibular joint arthritis in juvenile idiopathic arthritis: the forgotten joint, Curr.Opin.Rheumatol., Vol. 18(5), 490-495., 2006
   Organism:aDivision of Rheumatology, Children's Hospital of Philadelphia, USA bDepartment of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
   Abstract:PURPOSE OF REVIEW: This review explores the prevalence, clinical and radiographic signs, and treatment of temporomandibular joint arthritis in children with juvenile idiopathic arthritis. RECENT FINDINGS: Temporomandibular joint arthritis seems to be a more frequent manifestation in patients with juvenile idiopathic arthritis than previously believed, in part due to the paucity of clinical symptoms and poor sensitivity of conventional radiographs used for diagnosis. Antinuclear antibody positivity, early onset of disease, and presence of systemic or polyarticular disease are all risk factors for temporomandibular joint arthritis but may underpredict temporomandibular joint involvement in juvenile idiopathic arthritis. Magnetic resonance imaging enhanced with gadolinium is currently the gold standard in detection of temporomandibular joint arthritis, and treatment with intra-articular corticosteroids has been shown to be effective and safe, with minimal side effects. SUMMARY: Given the paucity of clinical symptoms in temporomandibular joint arthritis, detection of temporomandibular joint inflammation using contrast-enhanced magnetic resonance imaging is essential for instituting appropriate therapy in a timely fashion. The use of intra-articular corticosteroids holds promise for control of temporomandibular joint inflammation and prevention of associated morbidities

4. BARRY C, I, RASHIDI HH, BIFULCO CB, DEHORATIUS DM, HEALD PW, HOWE JG et COWPER SE: Epstein Barr virus related cutaneous T-cell lymphoma associated with juvenile rheumatoid arthritis and methotrexate therapy, 66, 20-10-2001

5. BELY Met APATHY A: [Lethal complications and associated diseases of rheumatoid arthritis--a retrospective clinicopathologic study of 234 autopsy patients], Orv.Hetil., Vol. 147(23), 1063-1076., 2006
   Organism:Budai Irgalmasrendi Korhaz, Patologiai Osztaly drbelymiklos@axelerohuFAU - Bely, Miklos
   Abstract:OBJECTIVE: Complications and/or associated diseases in rheumatoid arthritis can present atypical clinical manifestations which may lead to an incorrect or delayed diagnosis. The aim of this study was to determine: (1) the complications of rheumatoid arthritis, the accompanying diseases, and the mortality of these, (2) the clinically missed diagnoses of complications and/or associated diseases, (3) the possible links between coexistent complications of rheumatoid arthritis and/or diseases associated with it, furthermore the possible role of these in the mortality of rheumatoid arthritis patients. METHODS: Between 1970 and 1999 10,860 patients died at the National Institute of Rheumatology, and among them 234 with rheumatoid arthritis (diagnosed clinically according to the criteria of the American College of Rheumatology). The associated and basic disease, complication(s), and causes of death were determined on the basis of clinical records and in each case the autopsy findings were confirmed by a review of extensive histological material (50-100 tissue blocks from each patient). RESULTS: The complications of rheumatoid arthritis led to death in 152 (65%) of 234 patients. The complications of RA were clinically recognized in 109 (46.6%, 71.7 rel%) and missed in 43 (18.4% 28.3 rel%) of 152 patients. More than two thirds of lethal complications related to rheumatoid arthritis were diagnosed clinically. The remaining 82 (35%) of 234 rheumatoid arthritis patients died of associated diseases; the cause of death was clinically recognized in 78 (33.3%, 95.1 rel%) of 82 cases. There was a significant and positive correlation (1) between vasculitis and cardiac insufficiency (chi2 = 6.37, p <0.01), vasculitis and tuberculosis (chi2 = 4.18, p <0.04), or miliary tuberculosis (chi2 = 3.86, p <0.04); (2) between tuberculosis and miliary tuberculosis (chi2 = 54.84, p <0.001); and (3) between septic infection and purulent arthritis (chi2 = 97.04, p <0.001). There was a significant and inverse correlation between atherosclerosis and vasculitis (chi2 = 5.10, p <0.02), atherosclerosis and amyloidosis (chi2 = 14.58, p <0.001), or atherosclerosis and septic infection (chi2 = 3.81, p <0.05). There was a significant and inverse correlation between atherosclerosis and lethal cases of vasculitis (chi2 = 9.31, p <0.002), of amyloidosis (chi2 = 6.82, p <0.009), of sepsis (chi2 = 3.81, p <0.05) furthermore, between atherosclerosis with lethal outcome (n = 60 of 106) and vasculitis (chi2 = 12.06, p <0.001), or amyloidosis (chi2 = 13.22, p<0.002), or sepsis (chi2 = 10.82, p <0.001), or purulent arthritis (chi2 = 4.18, p <0.04). CONCLUSION: The most important life threatening complications of rheumatoid arthritis (vasculitis, AA amyloidosis and sepsis) are present and lead to death with higher probability in the younger age group (without atherosclerosis), while in older patients who have atherosclerosis these represent a lower risk of death

6. BERDELI G, OEZYUEREK AR, UELGER Z, GUERSES D, LEVENT E, SALAR Ket GUERPINAR AR: Association of macrophage migration inhibitory factor gene-173 G/C polymorphism with prognosis in turkish children with juvenile rheumatoid arthritis, Rheumatol.Int. Berlin, Vol. 26, 726-731., 2006
   Organism:Ege Univ, Sch Med, Dept Pediat, Mol Med Lab, TR-35100 Izmir, Turkey Turkey
   Abstract:The objectives of this study were to determine genotypic and allelic frequencies of macrophage migration inhibitory factor (MIF) gene -173 G/C polymorphism in patients with juvenile rheumatoid arthritis (JRA) and to evaluate the association of the MIF -173 C allele with the outcome of JRA. Genomic DNA was collected from 67 JRA patients and 153 healthy individuals. To evaluate the association of the MIF -173 polymorphism with the outcome, we analyzed the data concerning the treatment regimen, duration of glucocorticoid treatment, score on the childhood health assessment questionnaire (C-HAQ) and the number of joints with active arthritis. Nonsignificant differences were observed between the study and control groups in the distribution of genotype and allele frequencies of the MIF gene -173 G/C polymorphism. In JRA patients, carrying a MIF -173 C allele, the number of disease modifying antirheumatic drugs required for the treatment was more, the duration of glucocorticoid treatment was significantly longer, and at the last visits the C-HAQ scores and the number of joints with active arthritis were significantly higher. MIF gene -173 C allele frequency did not differ between the controls and JRA patients. MIF -173 C allele did not confer increased susceptibility to JRA in our study group. Carriage of the MIF -173 C allele was found to be a strong predictor of poor outcome in all types of JRA

7. BLEYER AJet HART TC: Genetic factors associated with gout and hyperuricemia, Adv.Chronic.Kidney Dis., Vol. 13(2), 124-130., 2006
   Organism:Department of Internal Medicine, Section on Nephrology, The Wake Forest University School of Medicine, Winston-Salem, NC 27157-1053, USA ableyer@wfubmceduFAU - Bleyer, Anthony J
   Abstract:Hyperuricemia and gout are common conditions that have long been known to have a heritable component. Obesity, diabetes, and chronic kidney failure are conditions with multifactorial inheritance that are associated with gout. In addition, social factors such as protein and alcohol intake affect serum uric acid levels. The current review discusses basic uric acid metabolism and the multigenetic inheritance of hyperuricemia. Several monogenic disorders affecting uric acid metabolism are reviewed. The genetics, pathophysiology, diagnosis, and treatment of familial juvenile hyperuricemic nephropathy/medullary cystic kidney disease, autosomal dominant disorders associated with hyperuricemia and progressive kidney failure, are described

8. BORCHERS AT, SELMI C, CHEEMA G, KEEN CL, SHOENFELD Yet GERSHWIN ME: Juvenile idiopathic arthritis, Autoimmun.Rev., Vol. 5(4), 279-298., 2006
   Organism:Department of Nutrition, USAFAU - Borchers, Andrea T
   Abstract:One of the most enigmatic problems in rheumatology has been juvenile idiopathic arthritis (JIA). Firstly, the classification has often depended on clinical features that have variations between patients. Secondly, there are different classification schemes in usage and there are few objective serologic tests that help to resolve the differences between the criteria sets. Thirdly, only recently have significant advances been made in understanding the immunology and immunopathology of JIA and, in particular, new treatment options. In this review, we will define the historical basis of JIA and emphasize not only the clinical features, but also the immunological characteristics, the pathogenesis, and treatment options. We will also discuss, in particular, quality of life, psychosocial functioning, socioeconomic outcomes and the difficult area of mortality. Finally, this review will attempt to bridge genetic observations with clinical presentation. JIA represents a relatively common syndrome of pediatric onset rheumatologic disease and a better understanding of the clinical definition, the relationship to autoimmunity, and novel treatments with biologic agents are critical for improved patient care

9. BOUAYED K, BRIK H, ZAKARI A, MIKOU Net DEHBI F: Hemophagocytic syndrome occuring in polyarticular juvenile arthritis treated by salazopyrine|, 2006
   Organism:

10. BRITISH SCO PA, CHILDREN'S CHRONIC ARTHRIT ASSOC_, LADY HOARE TPet ARTHRIT C: Health-related quality of life in adolescents with juvenile idiopathic arthritis, Arthritis &amp; Rheumatism, Vol. 55, 199-207., 2006
    Organism:Diana Princess Of Wales Childrens Hosp, Inst Child Hlth, Steelhouse Lane, Birmingham B4 6NH, W Midlands, UK UK
    Abstract:Objective. To describe the health-related quality of life (HRQOL) of adolescents with juvenile idiopathic arthritis (JIA), and to examine the usefulness of the juvenile Arthritis Quality of Life Questionnaire (JAQQ) in a UK context. It was hypothesized that HRQOL would decrease with worsening disease and disability.Methods. Patients with JIA ages 11, 14, and 17 years were recruited from 10 major rheumatology centers. HRQOL was measured using the JAQQ. Other data were core outcome variables including the Childhood Health Assessment Questionnaire, demographic characteristics, arthritis-related k nowledge, and satisfaction with health care.Results. Questionnaires were completed by 308 adolescents. One-fifth had persistent oligoarthritis. Median disease duration was 5.7 years (range &lt; 1-16 years). The JAQQ was shown to have good psychometric properties when used in the UK, but was not without limitations. HRQOL of adolescents with JIA was less than optimal, particularly in the domains of gross motor and systemic functioning. Items most frequently rated as adolescents' biggest psychological problems were "felt frustrated" and "felt depressed," rated by 30.2% and 23.4%, respectively. These were particularly problematic for the 17-year-olds, with 39% reporting frustration as one of their biggest problems and 63.6% reporting depression. Variation in the adolescent JAQQ scores was explained by functional disability, pain, and disease activity.Conclusion. JIA can have a significant adverse effect on the HRQOL of adolescents. The JAQQ is a useful tool to assess the HRQOL of UK adolescents with JIA, but there is need for improved measures that incorporate developmentally appropriate issues

11. BRITISH SOC PA, CHILDREN CHRONIC AA, LADY HOARE TRUST PHYSICALLY DISA_et ARTHRIT C: Growing up and moving on in rheumatology: Parents as proxies of adolescents with juvenile idiopathic arthritis, Arthritis &amp; Rheumatism, Vol. 55, 189-198., 2006
    Organism:Diana Princess of Wales Childrens Hosp, Inst Child Hlth, Steelhouse Lane, Birmingham B4 6NH, W Midlands, UK UK
    Abstract:Objective. To examine agreement about physical health, functional ability, and health-related quality of life (HRQOL) between adolescents with juvenile idiopathic arthritis (JIA) and their parents.Methods. The study group comprised 303 adolescent-parent dyads who completed individual questionnaires, including the Childhood Health Assessment Questionnaire with visual analog scales for pain and general well-being, and the juvenile Arthritis Quality of Life Questionnaire. Agreement was determined using the Bland and Altman method.Results. Approximately half of the adolescent-parent dyads showed clinically acceptable agreement for pain, general well-being, functional disability, and HRQOL. Where discrepancies occurred, there were similar numbers of parental overestimation and underestimation, with the exception that parents rated functional ability worse than did adolescents. Parents were also consistent with respect to overestimation or underestimation, irrespective of the health domain in question. Agreement was associated with better disease-related outcome variables, but was not significantly influenced by demographic factors. Agreement between adolescents and parents was dependent on the level of disease outcome and the health domain under scrutiny, and was less for moderate disease outcomes (as compared with mild or severe) and less visible phenomena (e.g., pain, global well-being).Conclusion. There is a wide variation in agreement between adolescents with JIA and their parents that is dependent on which health-related variable is under scrutiny. Proxy report is likely to be valid for adolescents with JIA at either the mild or severe end of the spectrum and/or for the visible manifestations of the disease. Consideration of both adolescent and parent-proxy reports is therefore important in future research

12. BRUCATO A, BRAMBILLA G, MOREO A, ALBERTI A, MUNFORTI C, GHIRARDELLO A, DORIA A, SHINAR Y, LIVNEH A, ADLER Y, SHOENFELD Y, MAURI F, PALMIERI Get SPODICK DH: Long-term outcomes in difficult-to-treat patients with recurrent pericarditis, Am.J.Cardiol., Vol. 98(2), 267-271., 2006
    Organism:Department of Internal Medicine, Ospedale Niguarda, Milano, Department of Rheumatology, University of Padua, Italy antoniobrucato@ospedaleniguardaitFAU - Brucato, Antonio
    Abstract:Patients with many recurrences of acute pericarditis are commonly alarmed by the fear of constriction. We studied their long-term outcome and the possible presence of systemic diseases. Sixty-one Italian patients (36 men) were followed for an average of 8.3 years according to a predefined protocol, including testing for autoimmune diseases and familial Mediterranean fever. Symptomatic pericarditis lasted from 1 to 43 years (mean 5.4 years). Fifty-two patients had been referred to us after failure of previous therapies, including steroids. We observed 378 attacks with a mean of 1.6 per patient per year and 156 hospital admissions. Thirteen patients had a post-cardiac injury syndrome. In 43 (70.5%), the pericarditis remained idiopathic, whereas we made a new diagnosis of rheumatoid arthritis in 1 and of Sjogren's syndrome in 4 patients, but in these patients pericarditis represented the dominant clinical manifestation. Cardiac tamponade occurred during the initial attacks in 4 patients (6.5%) but never recurred. Pleural effusions were present during the first attack in 22 patients (36.0%) and liver involvement in 5 (8%). No patients developed constrictive pericarditis. Echocardiographic examination produced no evidence of chronic myocardial disease. Response to therapy was good. Thirty-one patients (50.8%) are in sustained remission, without any therapy; their total observation period has averaged 10.3 years. In idiopathic patients, antinuclear antibodies were present in 56.2% and anti-Ro/SSA in 8.3%. Mutations linked to familial Mediterranean fever were absent. In conclusion, in this large series of difficult patients with recurrent acute pericarditis and a very long follow-up, the long-term prognosis is good

13. BRUNNER H, I, SHERRARD TMet KLEIN-GITELMAN MS: Cost of treatment of childhood-onset systemic lupus erythematosus, Arthritis &amp; Rheumatism, Vol. 55, 184-188., 2006
    Organism:Univ Cincinnati, Cincinnati Childrens Hosp, Med Ctr, William Rowe Div Rheumatol, E 4010,3333 Burnet Ave, Cincinnati, OH 45229 USA USA
    Abstract:Objective. To determine the direct cost of care of children with childhood-onset systemic lupus erythematosus (cSLE), and to determine the direct cost per quality-adjusted life year (QALY) with cSLE.Methods. Administrative databases from 2 large tertiary pediatric rheumatology centers in the United States were reviewed for all patients with cSLE (n = 119) diagnosed and regularly treated in these centers between January 2001 and April 2004. Health-related quality of life estimates for patients with cSLE (n = 297) reported in the literature were used to calculate QALYs based on global health ratings of the Child Health Questionnaire (range 0-100).Results. Information on 3,184 patient-months of followup was included in the analysis. During a mean SD followup of 27 +/- 11.8 months, the direct cost of care for the cohort amounted to $3,965,048, excluding outpatient medications. Irrespective of patient sex, the mean SD cost of cSLE per month was $1,245 +/- $2,352, or similar to$14,944 per year. Inpatient and day hospital care accounted for 28% of the cost, laboratory testing accounted for 21%, inpatient/day-patient medication costs accounted for 13%, and dialysis accounted for 11%. Visits to the rheumatology clinic only contributed 9% to the direct cost of care. When including an estimated outpatient medication cost of $1,190, the direct cost of cSLE per QALY was $30,908.Conclusion. Children diagnosed with cSLE were found to have a considerable direct cost of care. The treatment of cSLE appears to be far more costly than that of adult SLE and juvenile idiopathic arthritis reported in the literature

14. BURNHAM JM, SHULTS J, WEINSTEIN R, LEWIS JDet LEONARD MB: Childhood onset arthritis is associated with an increased risk of fracture: a population based study using the General Practice Research Database, Ann.Rheum.Dis., Vol. 65(8), 1074-1079., 2006
    Organism:Children's Hospital of Philadelphia, Room 1579 CHOP North, 3535 Market Street, Philadelphia, PA 19104, USA burnhams@emailchopeduFAU - Burnham, J M
    Abstract:BACKGROUND: Childhood onset arthritis is associated with low bone mass and strength. OBJECTIVE: To determine whether childhood onset arthritis is associated with greater fracture risk. METHODS: In a retrospective cohort study all subjects with onset of arthritis between 1 and 19 years of age in the United Kingdom General Practice Research Database were identified. As controls, all sex and age matched subjects from a practice that included a subject with arthritis were included. Incidence rate ratios (IRRs) for first fracture were generated using Mantel-Haenszel methods and Poisson regression. RESULTS: 1939 subjects with arthritis (51% female) and 207 072 controls (53% female) were identified. The median age at arthritis diagnosis was 10.9 years. A total of 129 (6.7%) first fractures were noted in subjects with arthritis compared with 6910 (3.3%) in controls over a median follow up of 3.90 and 3.95 years in the subjects with arthritis and controls, respectively. The IRR (95% confidence interval) for first fracture among subjects with arthritis, compared with controls, according to the age at the start of follow up were 1.49 (0.91 to 2.31) for age <10 years, 3.13 (2.21 to 4.33) at 10-15 years, 1.75 (1.18 to 2.51) at 15-20 years, 1.40 (0.91 to 2.08) at 20-45 years, and 3.97 (2.23 to 6.59) at >45 years. CONCLUSIONS: Childhood onset arthritis is associated with a clinically significant increased risk of fracture in children, adolescents and, possibly, adults. Studies are urgently needed to characterise the determinants of structural bone abnormalities in childhood arthritis and devise prevention and treatment strategies

15. CARLI C, EHLIN AG, KLARESKOG L, LINDBLAD Set MONTGOMERY SM: Trends in disease modifying antirheumatic drug prescription in early rheumatoid arthritis are influenced more by hospital setting than patient or disease characteristics, Ann.Rheum.Dis., Vol. 65(8), 1102-1105., 2006
    Organism:Medical Management Centre, Department of Learning, Informatics, Management & Ethics, Karolinska Institutet, Karolinska University Hospital D2:01, 171 76 Stockholm, Sweden CherylCullinane-Carli@karolinskaseFAU - Carli, C
    Abstract:OBJECTIVE: To characterise temporal trends and factors associated with the prescription of disease modifying antirheumatic drugs (DMARDs) at the initial consultation in early rheumatoid arthritis (RA). METHODS: Data from 2584 patients with early RA at 19 hospitals were extracted from the Swedish Rheumatoid Arthritis Register for the period 1997-2001. Disease characteristics and DMARD prescription at first consultation with the rheumatologist were investigated using cross tabulation and logistic regression. RESULTS: DMARD prescriptions, particularly for methotrexate, increased from 1997 to 2001 independently of patient characteristics. Stratification by hospital type showed that patients in district hospitals were less likely to be prescribed DMARDs than those in university hospitals (adjusted odds ratio (OR) = 0.53 (95% confidence interval (CI) 0.40 to 0.69), p<0.001), independently of confounding factors. Association of the DAS28 with the likelihood of DMARD prescription was greater among patients attending district hospitals (OR = 1.65 (1.34 to 2.02), p<0.001) than those at university hospitals (OR = 1.23 (1.07 to 1.41), p = 0.003) and county hospitals (OR = 1.34 (1.01 to 1.63), p = 0.003). Interaction testing indicated that the difference was significant (p = 0.007). CONCLUSIONS: Temporal trends in DMARD prescription indicate an increasingly aggressive approach to disease management among Swedish rheumatologists. However, the association of hospital type with DMARD prescription suggests that the adoption of research findings in clinical care varies considerably

16. CASSIDY J, KIVLIN J, LINDSLEY C, NOCTON J, SECTION Ret SECTION O: Ophthalmologic examinations in children with juvenile rheumatoid arthritis, Pediatrics, Vol. 117, 1843-1845., 2006
    Organism:Unlike the joints, ocular involvement with juvenile rheumatoid arthritis is most often asymptomatic; yet, the inflammation can cause serious morbidity with loss of vision. Scheduled slit-lamp examinations by an ophthalmologist at specific intervals can detect ocular disease early, and prompt treatment can prevent vision loss

17. CHEUNG Cet WIRRELL E: Adolescents' perception of epilepsy compared with other chronic diseases: "through a teenager's eyes", J.Child Neurol., Vol. 21(3), 214-222., 2006
    Organism:Faculty of Medicine, Queen's University, Kingston, ON, CanadaFAU - Cheung, Christina
    Abstract:Adolescent perception of physical and social impact of chronic illness was assessed to determine (1) if there is greater prejudice toward epilepsy than other chronic disease and (2) if adolescents with chronic disease have less prejudice toward similarly affected peers with all types of chronic disease or just their specific chronic disease. Cognitively normal teens aged 13 to 18 years without chronic disease (n = 41) and with epilepsy (n = 32), asthma (n = 38), diabetes (n = 21), and migraine (n = 17) were interviewed in the outpatient clinics of a tertiary care pediatric center regarding their perceptions of the physical and social impact of eight chronic diseases (epilepsy, asthma, diabetes, Down syndrome, arthritis, migraine, leukemia, human immunodeficiency virus [HIV] infection). Epilepsy was perceived to have a more adverse physical impact than all chronic illnesses except Down syndrome. The perception was that it more frequently caused mental handicap, injured the afflicted individual and bystanders, and led to death. Epilepsy was also perceived to have a more negative social impact, particularly on behavior, honesty, popularity, adeptness at sports, and fun. Significantly more adolescents expressed reluctance to befriend peers with epilepsy, both from their own and their perceived parental perspectives. Having a chronic disease did not generally alter the adolescents' perceptions of peers with chronic disease. However, cases with epilepsy ranked this disease to have less social impact than teens with other chronic diseases. In conclusion, adolescents consider epilepsy to have a greater physical and social impact than most chronic diseases. Educational efforts should focus on the "normality" of most persons with epilepsy and emphasize the low risk of injury when proper first aid is followed

18. CHOU CT: The clinical application of etanercept in Chinese patients with rheumatic diseases, Mod.Rheumatol., Vol. 16(4), 206-213., 2006
    Organism:Division of Allergy-Immunology-Rheumatology, Veterans General Hospital Taipei, No 201, Sec 2, Shipai Road, Beitou Chiu, Taipei, 112, Taiwan, ctchou@vghtpegovtwFAU - Chou, Chung-Tei
    Abstract:Over a 2-year period, to evaluate the efficacy and safety of biologic agents, etanercept (25 mg twice per week, s.c.) was used to treat 57 rheumatoid arthritis (RA) patients, 9 ankylosing spondylitis (AS) patients, 6 psoriatic arthritis (PSA) patients, and 4 juvenile rheumatoid arthritis (JRA) patients. In addition to inflammatory arthritis, I have used this tumor necrosis factor (TNF) blocker in other rheumatic diseases including idiopathic thrombocytopenic purpura (ITP), Behcet's disease with intractable oral ulcer, fibromyalgia syndrome, and systemic lupus erythematosis with intractable pleural effusion and acute lumbar disc herniation. For RA, after 6 months of etanercept treatment, all the parameters including number of swollen joints, number of tender joints, disease activity score, erythrocyte sedimentation rate, C-reactive protein, and global health status were rapidly improved (P < 0.001 or P < 0.0001). The anticyclic citrullinated peptide (anti-CCP) antibody and rheumatoid factor also significantly declined. For spondyloarthropathy, it also gave a similar effect as to RA. Both Bath Ankylosing Spondylitis Disease Activity Index and Bath Ankylosing Spondylitis Functional Index also improved. One of the two cases with Behcet's disease with intractable oral ulcer had a long-term remission after etanercept. The other Behcet's disease patient with oral ulcer and another with ITP obtained a good response temporarily. The short-term use of etanercept (<3 months) did not bring a significant effect for cases of fibromyalgia syndrome, pleural effusion, and lumbar disc herniation. In conclusion, a dramatic and rapid clinical response in different kinds of arthritis patients can be achieved by etanercept. Moreover, the TNF-alpha inhibitor also can treat other severe rheumatic-related symptoms. In general, except for a few cases with infection and two cases with malignancy, etanercept was safe in our arthritis patients. We need to study a larger number of patients in order to better understand the efficacy and safety of etanercept

19. CHOUDHARY A, HARDING SP, BUCKNALL RCet PEARCE IA: Mycophenolate mofetil as an immunosuppressive agent in refractory inflammatory eye disease, Journal of Ocular Pharmacology and Therapeutics, Vol. 22, 168-175., 2006
    Organism:Univ Liverpool, UCD, Dept Med, Unit Ophthalmol, Duncan Bldg,Daulby St, Liverpool L69 3GA, Merseyside, UK UK
    Abstract:Purpose: The aim of this study was to assess the role of mycophenolate mofetil (MMF) in refractory inflammatory eye disease.Methods: Retrospective, noncomparative, interventional case series of all patients commenced on MMF between 1999 and 2005 for refractory inflammatory eye disease at St Paul's Eye Unit (Liverpool, UK). Main outcome measures noted were control of inflammation, steroid-sparing effect, and adverse effects of MMF therapy.Results: Ten (10) patients (2 with sarcoid, 2 with intermediate uveitis, 1 with Vogt-Koyanagi Harada (VKH) syndrome, 1 with ankylosing spondylitis, 1 with juvenile chronic arthritis (JCA), and 3 with scleritis) who were unresponsive or intolerant to previous therapy and/or as a steroid-sparing agent, received 2-3 g of MMF per day for a mean period of 40.5 months (range, 3-67). Nine (9) patients had a favorable response, with diarrhea and insomnia being the main side-effects. MMF had to be withdrawn in 1 patient because of side-effects and in another because of active arthropathy (with stable uveitis). Average number of relapses was reduced from 3.1 per patient per year to 0.8 per patient per year (P &lt; 0.005). A steroid-sparing effect was achieved in all patients. Visual acuity improved in 8 patients.Conclusions: MMF appears to be a safe and effective second- or third-line adjunct/alternative immunosuppressant in these difficult cases and works well in combination with cyclosporin A, tacrolimus, and antitumor necrosis factor (TNF) agents. It has potential as a first- or second-line agent and can be considered at a dose of 3 g/day in refractory cases

20. DAVIES R: Haemopoietic stem cell transplantation for idiopathic juvenile arthritis (Still's disease), S263, 19-03-2001

21. DE BENEDETTI F: Inflammatory diseases in childhood and their impact on bone, S18, 10-05-2001

22. DE VIVO M, DE CHIARA C, SABBATINO MSet DE SETA L: A persistent fever with transient rash and an increase in antistreptolysine antibodies|, 2006
    Organism:A child of 11 years and 10 months of age comes to observation for persistent fever and arthralgias mainly in his hands. A few hours after hospital admission a transient rash appears. The laboratory tests show an increase in erythrocyte sedimentation rate, C-reactive protein, leukocyte count, antistreptolysine antibodies (from 1630 UI/ml to 2090 UI/ml after 7 days), and a negative throat swab for culture. After 10 days of hospitalization, for persistent arthralgias and fever, a therapy with non steroid anti-inflammatory agents is prescribed, with a light response. A negative bone marrow aspiration permits to start a therapy with prednisone with a drastic reduction of symptoms after 48 hours. A differential diagnosis with acute rheumatic fever and chronic juvenile arthritis gives us a high index of suspicion for a poststreptococcal reactive arthritis; a therapy with amoxicilline and afterwards a prophylaxis with benzathine penicilline for 12 months confirms after a 2 year follow-up, our diagnostic suspect

23. DOSTAL C, PAVELKA K, ZVAROVA J, HANZLICEK Pet OLEJAROVA M: Some principles of the development of a clinical database/national register of selected inflammatory rheumatic diseases in the Czech Republic, Int.J.Med.Inform., Vol. 75(3-4), 216-223., 2006
    Organism:Institute of Rheumatology, Na Slupi 2, 128 50 Prague 2, Czech Republic dost@revmaczFAU - Dostal, Ctibor
    Abstract:According to the World Health Organisation, rheumatic diseases are likely to go on occupying a prominent place worldwide. As to US statistics, rheumatic diseases are currently the most frequent chronic disorders and leading cause of disability. The development of functional clinical database or rheumatic diseases represents an essential condition how to acquire necessary epidemiological and other information on disorders under study. In 1999-2003, Institute of Rheumatology in cooperation with EuroMISE have developed clinical database/national register of selected systemic inflammatory rheumatic diseases inclusive of bank of sera and DNA. Aims of this phase of the pilot research have been formulated into following relevant and time borders: to gather clinical, laboratory, genetic but also pharmaco- and socio-economic data in a representative sample of patients with systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, mixed connective tissue disease; rheumatoid arthritis, juvenile chronic arthritis, ankylosing spondylitis, psoriatic arthritis and reactive arthritis. The data about patients entering the register are differentiated according to the disease of the patient. However, many diseases have several data in common. Therefore, a simple common data structure for examination of all monitored diseases was chosen. In 2002, the preset number of over 2000 registered patients had been achieved with collaboration of 34 territorial and 20 institutional rheumatologists in the whole covering the majority of the Czech Republic. Some first acquired information inclusive comparison with German database is demonstrated

24. DOSTAL C, PAVELKA K, ZVAROVA J, HANZLICEK Pet OLEJAROVA M: Some principles of the development of a clinical database/national register of selected inflammatory rheumatic diseases in the Czech Republic, International Journal of Medical Informatics, Vol. 75, 216-223., 2006
    Organism:Inst Rheumatol, Na Slupi 2, Prague 12850 2, Czech Republic Czech Republic
    Abstract:According to the World Health Organisation, rheumatic diseases are likely to go on occupying a prominent place worldwide. As to US statistics, rheumatic diseases are currently the most frequent chronic disorders and leading cause of disability. The development of functional clinical database or rheumatic diseases represents an essential condition how to acquire necessary epidemiological and other information on disorders under study. In 1999-2003, Institute of Rheumatology in cooperation with EuroMISE have developed clinical database/national register of selected systemic inflammatory rheumatic diseases inclusive of bank of sera and DNA. Aims of this phase of the pilot research have been formulated into following relevant and time borders: to gather clinical, laboratory, genetic but also pharmaco-and socio-economic data in a representative sample of patients with systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, mixed connective tissue disease; rheumatoid arthritis, juvenile chronic arthritis, ankylosing spondylitis, psoriatic arthritis and reactive arthritis. The data about patients entering the register are differentiated according to the disease of the patient. However, many diseases have several data in common. Therefore, a simple common data structure for examination of all monitored diseases was chosen. In 2002, the preset number of over 2000 registered patients had been achieved with collaboration of 34 territorial and 20 institutional rheumatotogists in the whole covering the majority of the Czech Republic. Some first acquired information inclusive comparison with German database is demonstrated. (c) 2005 Elsevier Ireland Ltd. All rights reserved

25. DUARTE C, GOMES C, CORREIA AJet SALGADO M: Renal amyloidosis: An uncommon complication of juvenile idiopathic arthritis|, 2006
    Organism:A 9-year-old girl presented with systemic-onset juvenile idiopathic arthritis, diagnosed at 3.5 of age and which was difficult to control despite several therapeutic trials. Five years after diagnosis of juvenile idiopathic arthritis, nephrotic proteinuria was noticed. Renal biopsy confirmed the diagnosis of amyloidosis, and chlorambucil was initiated, with general improvement of the disease and reduction of proteinuria. (c) Clinical Rheumatology 2005

26. FARROW SJ: Sir George Frederick Still (1868-1941), Rheumatology (Oxford)., Vol. 45(6), 777-778., 2006
    Organism:

27. FEIZY Vet GHOBADI A: Atopic dermatitis and systemic autoimmune diseases: a descriptive cross sectional study, Dermatol.Online.J., Vol. 12(3), 3, 2006
    Organism:Department of Dermatology, Tehran University of Medical Sciences vfeizy@doctorcomFAU - Feizy, Vida
    Abstract:Atopic dermatitis (AD) has a Th2 (T-helper 2) immune-reactivity pattern. However, the majority of systemic autoimmune diseases, such as rheumatoid arthritis, multiple sclerosis, and insulin dependent diabetes mellitus show a Th1 (T-helper 1) reactivity pattern. From this, one may hypothesize that AD and the Th1 autoimmune diseases could be inversely associated and AD may be more common in the minority of autoimmune diseases with a Th2 overactivity pattern such as systemic lupus erythematosus. A cross sectional study was designed. Our patients were enrolled from a general university hospital (all systemic autoimmune patients in every medical ward based on definite diagnoses in their medical records). Information on atopic dermatitis was obtained by questionnaires and physical examination by a dermatologist. A total of 63 patients were studied; 17.5 percent of cases had atopic dermatitis in the past or present. There were 31 patients 49.2 %) who carried a diagnosis known to be associated with Th1 reaction, and 21 patients (33.3 %) who had a disease associated with Th2-type reactivity. In 11 patients (17.5 %) the T-cell reaction type was not definitively classified. The relative frequency of AD was 9.7 percent (3 of 31 cases) in Th1-related autoimmune diseases, 28.6 percent (6 of 21 cases) in Th2-related autoimmune diseases and 18.2 percent (2 of 11 cases) in the unclassified category, a difference not statistically significant. Although the power of this study is not high enough to show a statistical significance, AD seems to be uncommon in patients with autoimmune diseases associated with Th1 overactivity

28. FERNANDEZ A, QUINTANA G, RONDON F, RESTREPO JF, SANCHEZ A, MATTESON ELet IGLESIAS A: Lupus arthropathy: a case series of patients with rhupus, Clin.Rheumatol., Vol. 25(2), 164-167., 2006
    Organism:Department of Internal Medicine, Rheumatology Unit, Universidad Nacional de Colombia, Bogota, ColombiaFAU - Fernandez, Andres
    Abstract:Among the clinical manifestations of systemic lupus erythematosus (SLE) is an arthropathy, which is usually nonerosive. In many cases the joint involvement is mild. A subset of patients have deforming, nonerosive Jaccoud's arthropathy, and a minority have an arthropathy with clinical findings similar to rheumatoid arthritis (RA) that has been called "rhupus." We report our series of eight patients (seven female, one male) with rhupus arthropathy. Patients were between the ages of 17 and 38 years (average: 30.3 years) at disease onset. All had deforming or Jaccoud's arthropathy, and three had erosive disease. The arthritis was typically the first disease manifestation. Other symptoms of lupus including vasculitis and glomerulonephritis appeared after an average of 2.8 years. All had positive antinuclear antibody and rheumatoid factor. Rhupus arthritis is not a combination of RA and SLE, but should be regarded as a variant of the arthropathy of lupus

29. FOSTER HEet CABRAL DA: Is musculoskeletal history and examination so different in paediatrics?, Best.Pract.Res.Clin.Rheumatol., Vol. 20(2), 241-262., 2006
    Organism:Musculoskeletal Research Group, Medical School, University of Newcastle, Framlington Place, Catherine Cookson Building, NE2 4HH Newcastle, UK hefoster@nclacukFAU - Foster, Helen E
    Abstract:Musculoskeletal (MSK) complaints in children and adolescents are common. The differential diagnosis is broad and based predominantly on clinical assessment. The skills both for eliciting history and for examination require understanding of the child/young person's specific emotional and cognitive developmental stage; interpretation of the findings requires knowledge of normal (and abnormal) motor and musculoskeletal growth and development. We specifically describe the different approach, unique skills and knowledge required by all clinicians who assess children and adolescents with MSK complaints; children and adolescents are not 'just little adults'. We emphasize the importance of clinical competence in ensuring that patients with juvenile idiopathic arthritis are diagnosed early and referral to specialist centres is not delayed with consequential suboptimal management and outcome. There is evidence that physician clinical skills in MSK assessment are inadequate, probably as a result of systemic deficiencies in the education process. Current and proposed solutions are discussed

30. FUERST M, FINK Bet RUTHER W: Survival analysis and longterm results of elbow synovectomy in rheumatoid arthritis, J.Rheumatol., Vol. 33(5), 892-896., 2006
    Organism:Department of Orthopaedics, Clinic of Joint replacement, Markgroningen, GermanyFAU - Fuerst, Martin
    Abstract:OBJECTIVE: To evaluate longterm results and survival rate of open synovectomy of the elbow joint in patients with rheumatoid arthritis (RA). METHODS: Between 1986 and 2000, synovectomy of the elbow was performed on 103 joints in 92 patients with RA. Eighty-five joints were included in this study. Mean age at time of surgery was 52 years (range 13 to 62 yrs). On 13 elbows with Larsen stage I and II disease, early synovectomy preserving the radial head was performed; in 72 cases with Larsen stage III and IV, late synovectomy with radial head resection was necessary. RESULTS: In early synovectomy, one joint received prosthetic joint replacement and 2 joints underwent resynovectomy a mean of 9 years after primary surgery. The survival rate (no further operations) was 91% after 5 years and 78% after 10 years. In late synovectomy, 16 elbow joints were operated again a mean of 4.6 years after primary surgery (10 prosthetic joint replacements, 2 resection interposition arthroplasties, 4 resynovectomies). Survival rate was 82% after 5 years and 66% after 10 years. Sixty-one elbows were examined clinically at a mean followup period of 8.7 years (range 2.8-17.3 yrs). There was a significant improvement of the Morrey score at followup, especially due to effective relief of pain. Improvement of joint motion was seen in late synovectomy for pronation and supination. The mean preoperative Larsen stage was 3.11, which decreased significantly to 3.66 at followup. CONCLUSION: Our findings suggest that synovectomy is a safe and effective procedure in differential treatment of RA of the elbow

31. GARCIA-MUNITIS P, BANDEIRA M, PISTORIO A, MAGNI-MANZONI S, RUPERTO N, SCHIVO A, MARTINI Aet RAVELLI A: Level of agreement between children, parents, and physicians in rating pain intensity in juvenile idiopathic arthritis, Arthritis &amp; Rheumatism, Vol. 55, 177-183., 2006
    Organism:Ist Giannina Gaslini, Largo G Gaslini 5, I-16147 Genoa, Italy Italy
    Abstract:

32. GHOSH JB, GUPTA Det CHATTOPADHYAY N: Juvenile idiopathic arthritis with peripheral gangrene, Indian J.Pediatr., Vol. 73(8), 739-741., 2006
    Organism:Department of Pediatric Medicine, IPGMER & SSKM Hospital, Kolkata, IndiaFAU - Ghosh, J B
    Abstract:Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disorder of the childhood and is manifested by synovitis with or without systemic features. Secondary vasculitis occurring in response to JIA is reflected clinically on different structures or systems of the body. Here is reported a rare case of systemic onset JIA (SOJIA) with vasculitis leading to peripheral gangrene

33. GIBSON DS, BLELOCK S, BROCKBANK S, CURRY J, HEALY A, MCALLISTER Cet ROONEY ME: Proteomic analysis of recurrent joint inflammation in juvenile idiopathic arthritis, J.Proteome.Res., Vol. 5(8), 1988-1995., 2006
    Organism:Queen's University Belfast, Arthritis Research Group, Musculoskeletal Education and Research Unit, Musgrave Park Hospital, Belfast BT9 7JB, Northern Ireland dgibson@qubacukFAU - Gibson, David S
    Abstract:The synovial fluid proteome in juvenile idiopathic arthritis was investigated to isolate joint-specific biomarkers that are expressed in patients displaying recurrent joint inflammation. To identify the synovial specific proteome, matched synovial fluid and plasma samples were subjected to protein separation by 2-dimension electrophoresis (2DE). Forty-three protein spots, overexpressed in the joint, were identified. Synovial fluids from children with single-event knee joint inflammation were then compared with a group with recurrent knee disease. Nine synovial specific proteins were significantly differentially expressed in the recurrent group. Proteolytic fragments of collagen X, fibrin beta-chain, and T-cell receptor alpha-region have been identified among this protein cluster. Putative biomarkers, overexpressed in the joint and differentially expressed in children with recurrent joint inflammation, have been identified. These proteins may play a significant role determining the pathological state within the chronically inflamed joint and influence disease progression in JIA. This is the first study of the synovial proteome in children

34. GLUSZEK Jet KOSICKA T: [Cardiovascular events in patients with systemic lupus erythematosus and rheumatoid arthrosis. New view on the old problem], Pol.Arch.Med.Wewn., Vol. 114(2), 785-791., 2005
    Organism:Katedra i Klinika Nadcisnienia Tetniczego, Chorob Naczyn i Chorob Wewnetrznych Akademii Medycznej w PoznaniuFAU - Gluszek, Jerzy
    Abstract:

35. GURAIEB-IBARROLA Ret GURAIEB-CHAHIN P: [Presentation of a clinical case of systemic juvenile rheumatoid arthritis], Rev.Med.Inst.Mex.Seguro.Soc., Vol. 44(4), 355-364., 2006
    Organism:Equipo de Pediatria, Hospital ABC, Mexico rguraiba@hotmailcomFAU - Guraieb-Ibarrola, Ricardo
    Abstract:A case of a 6-year-old male child was studied. Initially, he presented large-joint arthritis of the right knee and heel, with clinical data of systemic inflammation, such as fever, increase in sedimentation rate and leucocytosis, during more than six weeks of evolution. A wrong initial diagnosis of septic arthritis was made, but the lack of response to antibiotics and surgical drainage of the knee led to a trial with prednisone, which induced clinical remission. The patient remained hospitalized for 24 days and underwent surgical drainage, knee arthroscopy, two bone gammagrams, nuclear magnetic resonance, and antibiotics, as well as considerable stress and uncertainty for him and his family. Thus, it is very important to make an early diagnosis of juvenile rheumatoid arthritis and avoid complications. Recent literature was examined with special emphasis on opportune clinical diagnosis, as well as on therapeutic innovations, which have been able to modify the course of the disease and improve the quality of life of these patients. Pediatricians and general practitioners should take into account this diagnosis when seeing a patient with arthritis, especially when the signs point towards a systemic disease affecting two or more joints

36. HAMALAINEN H, ARKELA-KAUTAINEN M, HAAPASAARI J, KAUTIAINEN H, KOTANIEMI A et LEIRISALO-REPO M: Bone mineral density and healt related quality of life in young adults with juvenile idiopathic arthritis, S140, 10-05-2001

37. HARVEY H, ROWE M, WYLIE R et DAVIES B: Autologous stem cell transplantation for children with idiopathic juvenile arthritis - the challenge for nurse specialists, S272, 19-03-2001

38. HEAD AJ, MYERS LK, WATSKY MA, GREENWELL MW, BARROW KD, MICHELSON JAet CARBONE LD: Bone mineral density and turnover in non-corticosteroid treated African American children with juvenile rheumatoid arthritis, J.Rheumatol., Vol. 33(5), 1001-1003., 2006
    Organism:Department of Medicine, Division of Rheumatology; Children's Foundation Research Center at LeBonheur Children's Medical Center; University of Tennessee Health Sciences Center, Memphis, Tennessee 38163, USAFAU - Head, Andrew J
    Abstract:OBJECTIVE: To determine bone mineral content (BMC), bone mineral density (BMD), Z scores, and markers of bone turnover in African American children with juvenile rheumatoid arthritis (JRA). METHODS: Eight children with JRA with no prior exposure to corticosteroids were evaluated. Lumbar spine (L1-L4) and total body and total hip BMC and BMD were determined using dual x-ray absorptiometry (DXA), and Z scores (BMD) were calculated. Serum samples of markers of bone turnover including pyridinoline (PYR), N-terminal propeptide of type I procollagen (P1NP), osteocalcin (OC), and bone-specific alkaline phosphatase (BSAP) were measured. RESULTS: The mean Z score (BMD) at the lumbar spine (L1-L4) in patients with JRA was -1.2+/-0.8. Z scores for total body and total hip were within 1 standard deviation of normal compared with healthy historical controls matched for age, sex, and race. CONCLUSION: BMD was normal for chronological age (defined as Z score >or= 2.0) in African American children with JRA who had not previously been treated with corticosteroids. Further studies are needed on the effects of JRA on skeletal health in African American children

39. HOFER M: Spondylarthropathies in children--are they different from those in adults?, Best.Pract.Res.Clin.Rheumatol., Vol. 20(2), 315-328., 2006
    Organism:Centre Multisite Romand de Rhumatologie Pediatrique, Department of Paediatrics, University of Lausanne, Lausanne and University of Geneva, Geneva, Switzerland michaelhofer@chuvchFAU - Hofer, Michael
    Abstract:Juvenile spondylarthropathies (JSpAs) comprise a group of rheumatic diseases distinct from other categories of juvenile arthritis. Several classification systems have been applied, and some are specific for children, such as the seronegative enthesopathy and arthropathy (SEA) syndrome and the enthesitis-related arthritis, diagnostic forms in the International League of Associations for Rheumatism (ILAR) classification. JSpA seems more frequent than was previously believed, but actual epidemiological data show important variations between studies. Compared to adult patients, children with JSpA present with peripheral arthritis and enthesitis early in disease but sacroiliac and spine joints involvement many years later. A multidisciplinary team in a paediatric environment should be responsible for the management of children with spondylarthropathies to ensure the best care for these children with their chronic disease and risk of long-term disability. Recent advances in the treatment of rheumatic diseases with biological agents show promising results in children with JSpA. Further research needs to be conducted to increase our knowledge of the long-term outcome of these patients, to improve management, and to prevent long-term consequences of the disease

40. HOPPE B, HAUPL T, GRUBER R, KIESEWETTER H, BURMESTER GR, SALAMA Aet DORNER T: Detailed analysis of the variability of peptidylarginine deiminase type 4 in German patients with rheumatoid arthritis: a case-control study, Arthritis Res.Ther., Vol. 8(2), R34, 2006
    Organism:Institute of Transfusion Medicine, Campus Virchow-Klinikum, Charite-Universitatsmedizin Berlin, Germany bertholdhoppe@charitedeFAU - Hoppe, Berthold
    Abstract:Peptidylarginine deiminase type 4 (PADI4) genotypes were shown to influence susceptibility to rheumatoid arthritis (RA) in the Japanese population. Such an association could not previously be confirmed in different European populations. In the present study, we analysed exons 2-4 of PADI4 in 102 German RA patients and 102 healthy individuals to study the influence of PADI4 variability on RA susceptibility by means of haplotype-specific DNA sequencing. Analyses of the influence of PADI4 and HLA-DRB1 genotypes on disease activity and on levels of anti-cyclic citrullinated peptide antibodies were performed.Comparing the frequencies of PADI4 haplotype 4 (padi4_89*G, padi4_90*T, padi4_92*G, padi4_94*T, padi4_104*C, padi4_95*G, padi4_96*T) (patients, 14.7%; controls, 7.8%; odds ratio = 2.0, 95% confidence interval = 1.1-3.8) and carriers of this haplotype (patients, 27.5%; controls, 13.7%; odds ratio = 2.4, 95% confidence interval = 1.2-4.8), a significant positive association of PADI4 haplotype 4 with RA could be demonstrated. Other PADI4 haplotypes did not differ significantly between patients and controls. Regarding the individual PADI4 variants, padi4_89 (A-->G), padi4_90 (C-->T), and padi4_94 (C-->T) were significantly associated with RA (patients, 49.5%; controls, 38.7%; odds ratio = 1.6, 95% confidence interval = 1.1-2.3). Considering novel PADI4 variants located in or near to exons 2, 3, and 4, no quantitative or qualitative differences between RA patients (8.8%) and healthy controls (10.8%) could be demonstrated. While the PADI4 genotype did not influence disease activity and the anti-cyclic citrullinated peptide antibody level, the presence of the HLA-DRB1 shared epitope was significantly associated with higher anti-cyclic citrullinated peptide antibody levels (P = 0.033).The results of this small case-control study support the hypothesis that variability of the PADI4 gene may influence susceptibility to RA in the German population. Quantitative or qualitative differences in previously undefined PADI4 variants between patients and controls could not be demonstrated

41. HORNEFF G: Biologics for treatment of juvenile idiopathic arthritis. Consensus statement of the 7th Worlitzer Expertengesprache 2004 for the German Arbeitsgemeinschaft Kinder- und Jugendrheumatologie, Zeitschrift fuer Rheumatologie, Vol. 65, 152-156., 2006
    Organism:Univ Halle Wittenberg, Univ Klin and Poliklin Kinder and Jugendmed, Ernst Grube Str 40, D-06120 Halle, Germany Germany
    Abstract: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 Arbe itsgemeinschaft Kinder- und Jugendrheumatologie is updating the current recommendation for the treatment of juvenile idiopathic arthritis using biologics [6]

42. JIANG H, SUN Get LUO G: Retrospective analysis of 46 cases of juvenile idiopathic arthritis, Journal of China Medical University, Vol. 35, 322-323., 2006
    Organism:

43. JONES OY, SPENCER CH, BOWYER SL, DENT PB, GOTTLIEB BSet RABINOVICH CE: A multicenter case-control study on predictive factors distinguishing childhood leukemia from juvenile rheumatoid arthritis, Pediatrics, Vol. 117, E840-E844, 2006
    Organism:Childrens Natl Med Ctr, Dept Pediat Rheumatol, 111 Michigan Ave NW,WW 3-5,Suite 300, Washington, DC 20010 USA USA
    Abstract:OBJECTIVE. Acute lymphocytic leukemia ( ALL) often presents with musculoskeletal concerns such as pain or swelling, even before appearance of blasts in the peripheral blood. Such presentation may lead to misdiagnosis of a child with juvenile rheumatoid arthritis ( JRA). This study was designed to identify the predictive factors for leukemia using basic clinical and laboratory information.METHODS. A retrospective chart review was performed using a simple questionnaire to compare the clinical and laboratory findings present during the initial visit to a pediatric rheumatology clinic for 277 children who were ultimately diagnosed with either JRA ( n = 206) or ALL ( n = 71). Sensitivity and specificity analysis of a variety of parameters, both singly and in combination, was performed to identify predictive value for ALL.RESULTS. The majority ( 75%) of children with ALL did not have blasts in the peripheral blood at the time of evaluation by pediatric rheumatologists. In children presenting with unexplained musculoskeletal complaints, the 3 most important factors that predicted a diagnosis of ALL were low white blood cell count ( &lt; 4 x 10(9)/L), low-normal platelet count ( 150-250 x 10(9)/L), and history of nighttime pain. In the presence of all 3, the sensitivity and specificity for a diagnosis of ALL were 100% and 85%, respectiv ely. Other findings, including antinuclear antibody, rash, and objective signs of arthritis, were not helpful in differentiating between these diagnoses because they occurred at similar rates in both groups.CONCLUSIONS. When a child develops new-onset bone-joint complaints, the presence of subtle complete blood count changes combined with nighttime pain should lead to consideration of leukemia as the underlying cause

44. KAHN P, WEISS M, IMUNDO LFet LEVY DM: Favorable response to high-dose infliximab for refractory childhood uveitis, Ophthalmology, Vol. 113, 860-864., 2006
    Organism:Columbia Univ, Div Pediat Rheumatol, Morgan Stanley Childrens Hosp New York Presbyteri, Med Ctr, 3959 Broadway,CHN-106, New York, NY 10032 USA USA
    Abstract:Objective: Uveitis in children most commonly is associated with juvenile idiopathic arthritis. In addition to topical glucocorticoids, treatment may include systemic immunosuppressive agents. Tumor necrosis factor a (TNF-alpha) has been implicated in the pathogenesis of uveitis; therefore, TNF-alpha blockade seems to be a reasonable therapeutic option to investigate. We report successful treatment of children with uveitis using infliximab.Study Design: A retrospective study of our complete experience using infliximab for the treatment of childhood uveitis was conducted.Participants: Seventeen children (14 females, 3 males) with chronic uveitis were administered high-dose infliximab (10-20 mg/kg/dose).Main Outcome Measures: Our main outcome measure was the ability to eliminate all signs of intraocular inflammation.Results: All 17 patients demonstrated a dramatic, rapid response, with no observed inflammation in 13 patients after the second infusion, and 4 patients requiring 3 to 7 infusions to achieve disease quiescence. Additional immunosuppressives and topical glucocorticoids were tapered when patients achieved no intraocular inflammation.Conclusions: In this series, high-dose infliximab was a rapidly effective, well-tolerated therapeutic agent for the treatment of chronic, medically refractory, noninfectious uveitis

45. KASAPCOPUR O, YOLOGLU N, OZYAZGAN Y, ERCAN G, CALISKAN S, SEVER L, OZDOGAN Het ARISOY N: Uveitis and anti nuclear antibody positivity in children with juvenile idiopathic arthritis, Indian Pediatr., Vol. 41(10), 1035-1039., 2004
    Organism:Department of Pediatrics, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey ozgurcopur@e-kolaynetFAU - Kasapcopur, Ozgur
    Abstract:This study was conducted to determine the frequency of antinuclear antibodies (ANA) positivity and uveitis in our newly diagnosed juvenile idiopathic arthritis (JIA) patients classified according to International League Against Rheumatology (ILAR) classification criteria. Ninety-two girls and 106 boys, totally 198 children were enrolled in the study. of them 36 (18.2 percent) were found to be ANA positive. Chronic anterior uveitis was detected in 20 (10.1 percent) patients. ANA positivity was determined in 4 of the systemic JIA patients, in whom no uveitis had been detected. Twenty-five of 37 patients with oligoarticular JIA were ANA positive, in 10 of them uveitis was also diagnosed. ANA were positive in 3 of 34 patients with RF positive polyarticulat JIA, only one patient had positive ANA, and another one had uveitis. Nine patients were extended JIA and in none of them, ANA positivity or uveitis were present. Of 43 patients classified as enthesitis related arthritis (ERA), uveitis was diagnosed in 6 and there was no evidence of ANA positivity, but one had uveitis. We conclude that the incidence of ANA positivity and uveitis is low in Turkish children with JIA

46. KHAIRALLAH M, ATTIA S, ZAOUALI S, YAHIA SB, KAHLOUN R, MESSAOUD R, ZOUID Set JENZERI S: Pattern of childhood-onset uveitis in a referral center in Tunisia, north Africa, Ocul.Immunol.Inflamm., Vol. 14(4), 225-231., 2006
    Organism:Department of Ophthalmology, Fattouma Bourguiba University Hospital, Monastir, TunisiaFAU - Khairallah, Moncef
    Abstract:Purpose: To analyze the pattern of childhood uveitis in a referral center in Tunisia, North Africa. Methods:The study included 64 patients with uveitis examined at the Department of Ophthalmology of Monastir (Tunisia) from January 1994 to July 2005. All patients had a comprehensive ocular and systemic history, including an extensive review of medical systems. Complete ophthalmic examination was performed in all cases, including best-corrected Snellen visual acuity, slit-lamp examination, applanation tonometry, and dilated fundus examination with 3-mirror lens. Standard diagnostic criteria were employed for all syndromes or entities of uveitis. Results: The mean age at onset of uveitis was 12.4 years. The male-to-female ratio was 0.68. The process was unilateral in 51.6% of patients. Mean follow-up was 43.2 months. Anterior and intermediate uveitis each represented 31.25% of cases, posterior uveitis 20.3%, and panuveitis 17.2%. Noninfectious uveitis (75%) was the most frequent type of inflammation. Idiopathic uveitis was found in 50% of patients. Infectious uveitis was responsible for 25% of the cases, with toxoplasmosis (14.1%) being the most frequent cause. Twenty percent of the patients had systemic associations; juvenile idiopathic arthritis was found in 6.25%. Ocular complications occurred in 74.7% of affected eyes, of which the most common were posterior synechiae (28.4%), cataract (17.9%), cystoid macular edema (19%), and optic disc edema (32.6%). Fifty-seven affected eyes (60%) had a final visual acuity more than 20/40 and nine (9.5%) had a final visual acuity less than 20/200. Conclusions: In a hospital population in Tunisia, a specific cause of uveitis in children was found in half the patients. Idiopathic intermediate uveitis was the leading cause of uveitis in our study, followed by idiopathic anterior uveitis and toxoplasmosis. Uveitis associated with juvenile idiopathic arthritis was rare. Visual prognosis appeared to be good

47. KIMURA Y, WALCO GA, SUGARMAN E, CONTE PMet SCHANBERG LE: Treatment of pain in juvenile idiopathic arthritis: A survey of pediatric rheumatologists, Arthritis &amp; Rheumatism, Vol. 55, 81-85., 2006
    Organism:Hackensack Univ, Med Ctr, Joseph M Sanzari Childrens Hosp, 30 Prospect Ave, Hackensack, NJ 07601 USA USA
    Abstract:Objective. To assess the opinions and current practice of pediatric rheumatologists regarding treatment of chronic pain in children with juvenile idiopathic arthritis (JIA). Methods. Standardized questionnaires were distributed to pediatric rheumatologists who are members of the Children's Arthritis and Rheumatology Research Alliance. Demographic data, opinions, and attitudes were solicited about pain assessment, current treatment. of JIA with residual pain, and actual use of opioids to treat pain in children with JIA. Results. Of 99 rheumatologists who were contacted, 53. responses were received (53.5%). No significant demographic differences were found in attitudes about pain management and use of opioids. A total of 77.3% of respondents agreed that there are patients who continue to have significant pain despite adequate treatment. However, 59.6% disagreed with the use of opioid analgesics for treatment of those patients. Cross tabulations showed significant relationships between attitudes about opioid use and concerns for side effects, including drowsiness, fatigue, and constipation (chi(2) = 1.16, P &gt; 0.05), as well as addiction (chi(2) = 5.51, P = 0.019). Thirty percent of those who strongly disagreed with opioid use and 52.4% of those who disagreed had in fact prescribed opioids in the past year. The most commonly prescribed opioids were codeine and oxycodone. Practitioners' perceived knowledge of the drugs significantly affected their likelihood to prescribe them. Conclusion. Pediatric rheumatologists are divided in their attitudes regarding treatment of residual pain in children with JIA. Concern for side effects appears to be a major factor in the decision to prescribe these analgesics. More data are needed to facilitate clearer cost-benefit analyses in the decision to prescribe opioids to this clinical population

48. KORIAKINA EV, BELOVA SVet BLINNIKOVA VV: [Optimization of laboratory assessment of the activity of an inflammatory process in patients with rheumatoid arthritis], Klin.Lab Diagn., Vol. (6), 43-46., 2006
    Organism:The paper proposes to measure the serum content of medium-weight molecules as a simple and available means of rapidly and adequately assessing the total activity of an inflammatory process (including the minimal one) in patients with rheumatoid arthritis. The method may be performed at any powered clinical diagnostic laboratories; it does not require expensive reagents and equipment, and special staff training

49. KUEK A, HAZLEMAN BL, GASTON JHet OSTOR AJ: Successful treatment of refractory polyarticular juvenile idiopathic arthritis with rituximab, Rheumatology (Oxford)., Vol. ., 2006
    Organism:Rheumatology Research Unit, Addenbrooke's Hospital, Cambridge University Teaching Hospitals NHS, Foundation Trust, Cambridge, UK
    Abstract:

50. KUMP LI, CASTANEDA RA, ANDROUDI SN, REED GFet FOSTER CS: Visual Outcomes in Children with Juvenile Idiopathic Arthritis-Associated Uveitis, Ophthalmology., Vol. ., 2006
    Organism:Massachusetts Eye Research and Surgery Institute, Cambridge, Massachusetts; National Eye Institute, National Institutes of Health, Bethesda, Maryland
    Abstract:PURPOSE: To analyze visual outcomes in children affected by juvenile idiopathic arthritis (JIA)-associated uveitis. DESIGN: Retrospective interventional case series. PARTICIPANTS: Eighty-nine children with JIA-associated uveitis. METHODS: Charts of children with JIA-associated uveitis were reviewed. MAIN OUTCOME MEASURE: Change in patients' visual acuities (VAs). RESULTS: Of 269 children with uveitic syndromes referred, 89 (33%) had JIA-associated uveitis. The process was bilateral in 76 children. Seventy-three patients were female, and 84% of patients were Caucasian. Mean age of onset of uveitis was 5.7 years. Mean follow-up was 2.96 years. Antinuclear antibody positivity was detected in 56 patients, 44 of them female. Patients with JIA-associated uveitis developed numerous complications in the course of their disease: of 165 affected eyes, 105 (64%) developed cataracts, 33 (20%) developed increased intraocular pressure, and 76 (46%) developed band keratopathy; posterior synechiae were present in 96 (58%). Of 89 children, 73% were treated with immunomodulators, 40% were treated with nonsteroidal antiinflammatory agents alone or in combination with immunomodulators, and 21% were treated with topical and/or systemic steroids. Of 65 children who required immunomodulation, only one chemotherapeutic agent was used in 30, two agents in 21, and >/=3 in 14. Visual acuities of 65 children (122 eyes) were documented and compared at standard intervals. By mixed-models linear regression, improvement in VA of 0.03 logarithm of the minimum angle of resolution units per year was not found to be statistically significant (standard error, 0.02, P = 0.089). CONCLUSIONS: Juvenile idiopathic arthritis-associated uveitis is a sight-threatening disease. However, much of the children's vision can be preserved if patients are treated appropriately

51. LEE EHet HUI JH: The potential of stem cells in orthopaedic surgery, J.Bone Joint Surg.Br., Vol. 88(7), 841-851., 2006
    Organism:Department of Orthopaedic Surgery, Yong Loo Lin School of Medicine, National University of Singapore, Level 3, Main Building 1, National University Hospital, 5 Lower Kent Ridge Road, Singapore 119074 dosleeeh@nusedusgFAU - Lee, E H
    Abstract:

52. LEE EY, YIM JJ, LEE HS, LEE YJ, LEE EBet SONG YW: Dinucleotide repeat polymorphism in intron II of human Toll-like receptor 2 gene and susceptibility to rheumatoid arthritis, Int.J.Immunogenet., Vol. 33(3), 211-215., 2006
    Organism:Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, KoreaFAU - Lee, E Y
    Abstract:Human Toll-like receptors (TLRs) participate in innate immune response and signal the activation of adaptive immunity. The presence of a functional intronic polymorphism consisting of guanine-thymine repeats in TLR2 gene was recently reported. Here, we investigated a dinucleotide repeat polymorphism in intron II of TLR2 in Korean patients with rheumatoid arthritis (RA). The numbers of guanine-thymine [(GT)(n)] repeats in intron II of the TLR 2 gene were counted in 183 patients with RA and in 148 healthy controls, using the gene scanning technique. We classified alleles into two subclasses for further analysis, 12-16 GT repeats (S allele) and 17-28 repeats (L allele). By subgroup analysis, we also examined whether the S allele is associated with the presence of shared epitope (SE), rheumatoid factor (RF), joint erosion and extra-articular complications. S-allele frequency was significantly increased in patients with RA than in healthy controls [30.3% vs. 23.0%, P = 0.03, or 1.46, 95% confidence interval (CI) 1.03-2.07], and genotypes containing S alleles were more frequent in patients with RA than in healthy controls (54.4% vs. 46.5%. P = 0.04, or 1.57, 95% CI 1.01-2.42). A skewed S-allele distribution was not found to be related to the presence of SE. Subgroup analysis showed no genotypic or allele frequency differences between patients with/without RF, joint erosion, or extra-articular complications. Genotype containing shorter GT repeats in intron II of the TLR2 gene may confer susceptibility to RA in Koreans

53. LEE PPW, LEE TL, WONG WHSet LAU YL: The use of methotrexate in juvenile idiopathic arthritis: A single center experience|, 2006
    Organism:In the recent decade, an increasing number of disease-modifying anti-rheumatic drugs (DMARDs) have been developed for treatment in juvenile idiopathic arthritis (JIA). Currently, methotrexate (MTX) is the DMARD of first choice particularly in oligoarticular and polyarticular JIA, but its efficacy in systemic-onset JIA and enthesitis-related JIA was less satisfactory. A retrospective study on 40 patients followed up at Queen Mary Hospital for JIA was performed to review the treatment outcome and adverse effects associated with use of MTX. We concluded that MTX was safe and well tolerated in maj ority of patients, but treatment response varied with different JIA subtypes. Combination of MTX with other antiinflammatory agents was often required to achieve disease remission in patients with more severe disease. Large, randomised controlled trials are needed to determine the efficacy of individual drug and their combination in each JIA subtype

54. LI J, HEINZ C, ZUREK-IMHOFF Bet HEILIGENHAUS A: Intraoperative intraocular triamcinolone injection prophylaxis for post-cataract surgery fibrin formation in uveitis associated with juvenile idiopathic arthritis, J.Cataract Refract.Surg., Vol. 32(9), 1535-1539., 2006
    Organism:From the Department of Ophthalmology, St Franziskus Hospital, Muenster, GermanyFAU - Li, Jin
    Abstract:PURPOSE: To assess the efficacy of a single intraoperative intraocular injection of triamcinolone acetonide during cataract surgery to prevent postoperative fibrin formation in patients with iridocyclitis associated with juvenile idiopathic arthritis. SETTING: Department of Ophthalmology, St. Franziskus Hospital, Muenster, Germany. METHODS: The charts of 22 patients (16 girls and 6 boys) with juvenile idiopathic arthritis and chronic iridocyclitis having lensectomy and anterior vitrectomy were retrospectively reviewed. In 12 patients (14 eyes), triamcinolone acetonide 4 mg was injected into the anterior chamber at the end of the surgery (triamcinolone group). Another 10 patients (13 eyes) received an intraoperative intravenous injection of methylprednisolone and postoperative oral prednisolone (systemic treatment group). No intraocular lenses were implanted. Postoperatively, prednisolone acetate 1% eyedrops were given. The main problems included intraocular inflammation, the need for additional systemic corticosteroids, and intraocular pressure (IOP) elevation. RESULTS: The mean patient age was 10.6 years +/- 3.1 (SD) in the triamcinolone group and 7.4 +/- 2.7 years in the systemic treatment group. The mean follow-up was 9.9 +/- 3.6 months and 10.9 +/- 1.2 months, respectively. All patients were taking systemic immunosuppression before surgery, and the medication was continued postoperatively. Fibrin formation was not seen after surgery in the triamcinolone group but occurred in 5 patients in the systemic treatment group (P = .02). Additional systemic corticosteroids were not required in the triamcinolone group. All patients had visual acuity improvement. No increase in IOP was noted after the triamcinolone acetate injections. CONCLUSIONS: Intraoperative intraocular injection of 4 mg of triamcinolone acetonide may be more effective than intraoperative intravenous methylprednisolone and additional postoperative short-term oral prednisolone in preventing postoperative fibrin formation after cataract surgery in patients with juvenile idiopathic arthritis and iridocyclitis

55. LISTING J, STRANGFELD A, RAU R, KEKOW J, GROMNICA-IHLE E, KLOPSCH T, DEMARY W, BURMESTER GRet ZINK A: Clinical and functional remission: even though biologics are superior to conventional DMARDs overall success rates remain low--results from RABBIT, the German biologics register, Arthritis Res.Ther., Vol. 8(3), R66, 2006
    Organism:German Rheumatism Research Centre, Berlin, Germany Listing@drfzdeFAU - Listing, Joachim
    Abstract:We investigated the frequency of remission according to the disease activity score (DAS28) definition, modified American Rheumatology Association (ARA) criteria, and the frequency of an achievement of a functional status above defined thresholds ('functional remission', 'physical independence') in rheumatoid arthritis (RA) patients treated with either biologics or conventional DMARDs. We used the data of a prospective cohort study, the German biologics register RABBIT (German acronym for Rheumatoid Arthritis--Observation of Biologic Therapy) to investigate the outcomes in RA patients with two or more DMARD failures who received new treatment with biologics (BIOL; n = 818) or a conventional DMARD (n = 265). Logistic regression analysis was applied to adjust for differences in baseline risks. Taking risk indicators such as previous DMARD failures or baseline clinical status into account, we found that biologics doubled the chance of remission compared to conventional DMARD therapies (DAS28 remission, adjusted odds ratio (OR) 1.95 (95% confidenece interval (CI) 1.2-3.2)); ARA remission, OR 2.05 (95% CI 1.2-3.5)). High remission rates (DAS28 remission, 30.6%; ARA remission, 16.9%) were observed in BIOL patients with a moderate disease activity (DAS28, 3.2 to 5.1) at the start of treatment. These rates decreased to 8.5% in patients with DAS28 > 6. Sustained remission at 6 and 12 months was achieved in <10% of the patients. Severely disabled patients (< or = 50% of full function) receiving biologic therapies were significantly more likely to achieve a status indicating physical independence (> or = 67% of full function) than controls (OR 3.88 (95% CI 1.7-8.8)). 'Functional remission' (> or = 83% of full function) was more often achieved in BIOL than in controls (OR 2.18 (95% CI 1.04-4.6)). In conclusion, our study shows that biologics increase the chance to achieve clinical remission and a status of functional remission or at least physical independence. However, temporary or even sustained remission remain ambitious aims, which are achieved in a minority of patients only

56. LOPEZ-LONGO FJ, RODRIGUEZ-MAHOU M, SANCHEZ-RAMON S, ESTECHA A, BALSERA M, PLAZA R, FERNANDEZ-CRUZ Eet PEREZ LC: Anti-cyclic citrullinated peptide versus anti-Sa antibodies in diagnosis of rheumatoid arthritis in an outpatient clinic for connective tissue disease and spondyloarthritis, J.Rheumatol., Vol. 33(8), 1476-1481., 2006
    Organism:Department of Rheumatology, Hospital General Universitario Gregorio Maranon, Madrid, SpainFAU - Lopez-Longo, Francisco-Javier
    Abstract:OBJECTIVE: . To compare the diagnostic value of anti-cyclic citrullinated peptide (anti-CCP) and anti-Sa antibodies in serum for prediction of rheumatoid arthritis (RA) in an outpatient clinic for connective tissue diseases and spondyloarthritides. METHODS: A cross-sectional study was carried out to analyze the presence or absence of anti-CCP and anti-Sa antibodies in the sera of 250 randomly selected patients. The disease distribution in the study was as follows: 87 patients had RA (34.8%); 90 (36%) had other connective tissue diseases (CTD); 50 (20%) spondyloarthritis; 19 (7.6%) polymyalgia rheumatica; and 4 (1.6%) juvenile idiopathic arthritis. RESULTS: Anti-CCP antibodies were detected in 63 patients with RA and in 9 patients with other illnesses [sensitivity 72.4%, specificity 94.4%, positive predictive value (PPV) 87.5%]. Anti-Sa antibodies were detected in 38 patients with RA and in 6 patients with other illnesses (sensitivity 43.6%, specificity 96.3%, PPV 86.3%). Anti-CCP and anti-Sa results were discordant in up to 47 of 87 RA patients. No relation between the presence of anti-Sa and higher or lower titers of anti-CCP antibodies was observed. CONCLUSION: The diagnostic value in RA is similar for both antibodies. However, the sensitivity of anti-CCP detection is higher than that of anti-Sa. Our results suggest that presence of anti-Sa antibodies in serum may be useful as a complementary assay when anti-CCP antibodies are negative and RA is suspected

57. LORRAINE PJ, HAMMOCK RLet BLANTON JM: Predictors of self-rated health status among Texas residents, Prev.Chronic.Dis., Vol. 2(4), A12, 2005
    Organism:The University of Texas at Austin School of Nursing, 1700 Red River, Austin, TX 78701-1499, USA lorrainephillips@yahoocomFAU - Lorraine, Phillips J
    Abstract:INTRODUCTION: The purpose of this study was to investigate the predictors of self-rated health status for Texas adults using the current 2003 Behavioral Risk Factor Surveillance System data. Self-rated health is generally accepted as a valid measure of health status in population studies, and understanding its correlates may help public health professionals prioritize health-promotion and disease-prevention interventions. METHODS: The two research questions addressed by this study involved the predictors of self-rated health: 1) "Do demographic characteristics, health care coverage, leisure-time physical activity, and body mass index predict self-rated health status for Texas residents aged 18 to 64 years?" and 2) "Does choice of interview language (English vs Spanish) predict self-rated health status for Texas residents of Hispanic ethnicity aged 18 to 64 years?" Key analysis variables were identified, and descriptive statistics were used to describe the major variables and determine whether the number of respondents for each variable was sufficient for analysis. Multivariate regression analysis was used to assess the variables. RESULTS: Multiple logistic regression analysis (controlling for diabetes and arthritis) of the self-rated health predictors indicated that older age, lack of health care coverage, lack of a college education, being Hispanic, having a lower income, obesity, and not exercising explained 19.4% of the variance of fair and poor self-rated health. The interview language (English or Spanish), age, sex, education, income, obesity, health insurance coverage, and physical activity (controlling for chronic illness) explained 22.8% of the variance in fair and poor self-rated health for Hispanic respondents. CONCLUSION: The results of this study suggest that a college education, a lower body mass index, non-Hispanic ethnicity, and participation in physical activity are associated with good, very good, or excellent self-rated health status. The finding that the interview language significantly predicted fair and poor self-rated health substantiates previous research and emphasizes the importance of culturally sensitive approaches to health care services

58. MAKAY B, YILMAZ S, TURKYILMAZ Z, UNAL N, OREN Het UNSAL E: Etanercept for therapy-resistant macrophage activation syndrome, Pediatr.Blood Cancer., Vol. ., 2006
    Organism:Department of Pediatric Rheumatology, Dokuz Eylul University Faculty of Medicine, Balcova, Izmir, Turkey
    Abstract:Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of childhood rheumatic diseases, especially systemic onset juvenile idiopathic arthritis (SoJIA). We report a 4-year-old girl with probable SoJIA who presented with MAS. She did not respond to pulse methyl prednisolone and Cyclosporine A (CsA). She also failed to respond to intravenous immunoglobulin (IVIG) therapy. Etanercept was started, based on the observation of increased serum levels of tumor necrosis factor-alpha (TNF-alpha) in patients with MAS. Her condition improved following etanercept, suggesting that etanercept might have a therapeutic role in resistant MAS. Pediatr Blood Cancer (c) 2006 Wiley-Liss, Inc

59. MALEMUD CJ: Growth hormone, VEGF and FGF: Involvement in rheumatoid arthritis, Clin.Chim.Acta., Vol. ., 2006
    Organism:Department of Medicine/Division of Rheumatic Diseases, Case Western Reserve University School of Medicine and University Hospitals of Cleveland, 2061 Cornell Road, Room 207 Cleveland, OH 44106-5076, USA
    Abstract:Adult rheumatoid arthritis (RA), a systemic autoimmune disorder of unknown etiology, is characterized by dysfunctional cellular and humoral immunity, enhanced migration and attachment of peripheral macrophages and pro-inflammatory leukocytes to the synovium and articular cartilage of diarthrodial joints. The progressive destruction of cartilage and bone in RA is a result of elevated pro-inflammatory cytokine gene expression, synovial neovascularization, proteinase-mediated dissolution of articular cartilage matrix and osteoclast-mediated subchondral bone resorption. Juvenile chronic arthritis (JCA) is disease with manifestations similar to adult RA that occurs in childhood. JCA usually causes precocious joint destruction and often also presents with evidence of growth plate anomalies and reduced stature. Three proteins play an integral role in both adult RA and JCA. These are somatotropin (also called pituitary growth hormone (GH)), vascular endothelial growth factor (VEGF) and fibroblast growth factor (FGF). GH is responsible for regulating long bone growth and skeletal maturation through its capacity to stimulate insulin-like growth factor-I (IGF-1) synthesis by hepatocytes. Mechanisms responsible for growth plate disturbances and short stature in children with JCA include deficient GH production, GH-insensitivity resulting from defects in the GH receptor, suppressed IGF-1 synthesis or neutralization of IGF-1 action by IGF-1 binding proteins (IGFBPs). In addition, GH has also been implicated in perpetuating inflammation and pain in adult RA. VEGF has been shown to be the critical angiogenesis factor responsible for vascular proliferation and blood vessel invasion of the synovial lining membrane in RA. Acidic FGF (FGF-1) and basic FGF (FGF-2) have also been implicated in aberrant synoviocyte proliferation (i.e. synovial hyperplasia) and apoptosis resistance in adult RA

60. MARCIANO R, GIACOPELLI F, DIVIZIA MT, GATTORNO M, FELICI E, PISTORIO A, MARTINI A, RAVAZZOLO Ret PICCO P: A polymorphic variant inside the osteopontin gene shows association with disease course in oligoarticular juvenile idiopathic arthritis, Annals of the Rheumatic Diseases, Vol. 65, 662-665., 2006
    Organism:G Gaslini Inst Children, Mol Genet Lab, Largo G Gaslini 5, I-16147 Genoa, Italy Italy
    Abstract:Background: Oligoarticular onset juvenile idiopathic arthritis (JIA) has a variable disease course. In some patients the disease remains confined to a few joints ( persistent oligoarticular) while in others it extends to affect more joints ( oligoarticular extended). Osteopontin is thought to play a role in the pathogenesis.Objective: To investigate whether a polymorphic variant in the human osteopontin gene, which is in linkage disequilibrium with recently characterised promoter variants, is associated with the disease course in oligoarticular JIA.Methods: Genotyping of the two base pair inse rtion/deletion variant at + 245 in the first intron was undertaken by polymerase chain reaction (PCR) amplification of DNA fragments, using a fluorescently labelled primer, followed by allele detection after rapid separation of PCR products on an automated DNA sequencer.Results: Allele 2 of the polymorphic variant in the osteopontin first intron was significantly associated with the persistent oligoarticular form rather than the extended form of JIA. This was verified at the level of genotype and allele frequencies.Conc lusions: The results suggest that osteopontin gene polymorphism is associated with the disease course in oligoarticular JIA and might therefore represent a useful genetic marker to characterise patients with oligoarticular JIA who are at risk of a worse outcome

61. MARTINEZ TOLEDO MM, MARTINI G, GIGANTE C, DA DALT L, TREGNAGHI Aet ZULIAN F: Is There a Role for Arthroscopic Synovectomy in Oligoarticular Juvenile Idiopathic Arthritis?, J.Rheumatol., Vol. ., 2006
    Organism:OBJECTIVE: To evaluate the longterm efficacy and safety of arthroscopic synovectomy (AS) in children with oligoarticular juvenile idiopathic arthritis (JIA). METHODS: Patients with oligoarticular JIA and persistent monoarticular involvement, refractory to nonsteroidal antiinflammatory drugs (NSAID) and/or intraarticular corticosteroid (IAC) treatment underwent AS followed, one month later, by IAC. The efficacy of AS was prospectively evaluated, and a good response was defined as absence of synovitis or > 60% decrease in articular score from baseline. Clinical, laboratory, and radiological variables (radiographs, ultrasound, magnetic resonance imaging) were noted to examine possible factors predictive of the result. RESULTS: Twenty-two patients with JIA (15 female, 7 male) entered the study. Age at disease onset was 77 months (range 13-168). Mean disease duration at the time of AS was 50 months (3-324). Nineteen knees, 2 temporomandibular joints, and one shoulder were treated; the mean followup was 57 months (12-168). Thirty-six percent of patients relapsed within 12 months of the procedure, 14% within 24 months, and 14% thereafter. Eight patients (36%) remain in remission after a mean 65 months' followup. Variables found to be predictive of good response were persistent monoarticular course (p = 0.004), short disease duration at the time of AS (p = 0.03), and normal erythrocyte sedimentation rate and C-reactive protein at baseline (p = 0.008 and 0.01, respectively). CONCLUSION: AS is a safe but only partially effective procedure in patients with oligoarticular JIA. Best results are achieved early in the disease course in children with persistent monoarticular involvement and no evidence of systemic inflammation

62. MATES M, ZEVIN S, BREUER GS, NAVON Pet NESHER G: Desensitization to hydroxychloroquine - Experience of 4 patients, Rinsho Ganka, Vol. 33, 814-816., 2006
    Organism:Shaare Zedek Med Ctr, Dept Internal Med, POB 3235, IL-91031 Jerusalem, Israel Israel
    Abstract:Hydroxychloroquine (HCQ) is an antimalarial agent with immunomodulatory effects. It is widely used in rheumatologic diseases, and has a very high efficacy/toxicity ratio. It is particularly important in the treatment of systemic lupus erythematosus (SLE) since it reduces new organ involvement and disease flares, and relieves skin and joint symptoms. Some patients develop hypersensitivity rash in response to HCQ. In such patients the drug is withdrawn and replaced by another medication. All the alternative medications for rheumatological patients are significantly more toxic than HCQ. We describe our initial experience of HCQ slow oral desensitization. All 4 patients who were recruited completed the procedure successfully without significant difficulty. Our results suggest that HCQ slow oral desensitization is safe, effective, and easy to perform

63. MATTYASOVSZKY S, SKAPENKO A, KALDEN JR, LIPSKY PEet SCHULZE-KOOPS H: IFNGR1 single nucleotide polymorphisms in rheumatoid arthritis, Arthritis Res.Ther., Vol. 8(3), R63, 2006
    Organism:Nikolaus Fiebiger Center for Molecular Medicine, Clinical Research Group III, University of Erlangen, GermanyFAU - Mattyasovszky, Stefan
    Abstract:On the basis of their biological function, potential genetic candidates for susceptibility to rheumatoid arthritis can be postulated. IFNGR1, encoding the ligand-binding chain of the receptor for interferon gamma, IFNgammaR1, is one such gene because interferon gamma is involved in the pathogenesis of the disease. In the coding sequence of IFNGR1, two nucleotide positions have been described to be polymorphic in the Japanese population. We therefore investigated the association of those two IFNGR1 single nucleotide polymorphisms with rheumatoid arthritis in a case-control study in a central European population. Surprisingly, however, neither position was polymorphic in the 364 individuals examined, indicating that IFNGR1 does not contribute to susceptibility to rheumatoid arthritis, at least in Caucasians

64. MCDONAGH Jet MURRAY K: Preface: Paediatric and adolescent rheumatology, Best.Pract.Res.Clin.Rheumatol., Vol. 20(2), 177-178., 2006
    Organism:Institute of Child Health, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK jemcdonagh@bhamacukFAU - McDonagh, Janet
    Abstract:

65. MELKOWSKI T, EMERICH Ket ADAMOWICZ-KLEPALSKA B: The evaluation of the muscle and TMJ status among children and adolescents suffering from juvenile chronic arthritis|, 2005
    Organism:The clinical dental examination was carried out in 1998 and 2000 among the Polish population in a group of 100 people aged 4-18, who were found clinically and diagnostic suffering from juvenile chronic arthritis (JCA). The whole examined population suffering from juvenile chronic arthritis was divided into three age groups: 4-11, 12 and 13-18 years of age. The healthy control group of 100 people matched the amount, age and sex to those suffering from JCA. In the study among young patients suffering from JCA, besides complex evaluation of the dental health the muscle and TMJ status were also examined. The escalation of pathological changes and TMJ dysfunctions in JCA increases with ageing. People with JCA syndroe represent a high-risk group of special health-care that need to be provided with early health education, prevention, professional multispecialistic dental treatment and rehabilitation

66. METIVIER Het HASSON SM: Use of the faces pain scale to evaluate pain of a pediatric patient with pauciarticular juvenile rheumatoid arthritis, Physiotherapy Theory and Practice, Vol. 22, 91-96., 2006
    Organism:Univ Connecticut, 358 Mansfield Rd,Unit 2101, Storrs, CT 06269 USA USA
    Abstract:The Faces Pain Scale developed by Wong and Baker is a common assessment tool that uses cartoon-like faces to assess self-reported pain in children. The purpose of this case report is to explore the appropriateness of the Faces Pain Scale as an outcome measure for a young child with pauciarticular juvenile rheuniatoid arthritis. The patient was a four-year-old boy who had undergone a synovectomy on his, right knee secondary to pauciarticular JRA. Each session the patient was asked to rate his pain using the Faces Pain Scale. The patient gained full knee range of motion and his functional moblitity improved compared to initial visit. His subjective pain rating remained constant at "no hurt" throughout three weeks of visits. His functional mobility did not match his subjective rating of pain via the Faces Pain Scale. Further research is needed to determine the relationship of pain, stiffness, and junction in children with rheumatic diseases

67. MINDEN K: What are the costs of childhood-onset rheumatic disease?, Best.Pract.Res.Clin.Rheumatol., Vol. 20(2), 223-240., 2006
    Organism:German Rheumatism Research Centre Berlin, Rheumatology Unit, Berlin, Germany minden@drfzdeFAU - Minden, Kirsten
    Abstract:Juvenile rheumatic diseases have important impacts on health, i.e. on patients' body functions and structures, activities, and social participation. The identification and treatment of these disorders is costly. Treatment involves periods of hospitalization, the use of physicians and other professional services, drugs and other treatments. Frequent outpatient visits are needed, as may be surgery. This all imposes a large financial burden on health-care systems and on families of children who suffer from rheumatic illnesses. Costs borne by families are both out-of-pocket as well as related to time spent in providing care, which may involve loss of income. Of course, the burdens are not only monetary, and they sometimes continue for life. The measurement of the different types of cost is essential to get a full picture of the burden of childhood-onset rheumatic illnesses. This chapter presents data on the costs of these illnesses, and introduces methodologies and their limitations for cost evaluation within paediatric rheumatology

68. MISHLANOV VI, TUEV AV, SHUTOV AA, BAIDINA TV, SIUTKINA OVet OBUKHOVA OV: [Method for determining leukocytic lipid-releasing capacity in the diagnosis of mechanisms responsible for atherogenesis in patients with coronary heart disease and ischemic stroke], Klin.Lab Diagn., Vol. (5), 9-13., 2006
    Organism:The authors have studied the mechanism responsible for neutrophilic accumulation and release of protein-lipid complexes in patients with atherosclerosis and the clinical value of this reaction. They present the results of experimental studies and clinical observations in the groups of patients with ischemic stroke, coronary heart disease, bronchial asthma, chronic obstructive pulmonary disease, and rheumatoid arthritis. Whether the process of neutrophilic and release of protein-lipid can be in vitro modulated in a 3-day culture has been demonstrated. The high diagnostic value of the method for determining the lipid-releasing capacity of leukocytes is shown. Some proteins involving in the formation of pathogenic protein-lipid complexes are identified. There is evidence for the use of new differential criteria for visceral diseases originating from atherosclerosis

69. MORELAND LW, WEINBLATT ME, KEYSTONE EC, KREMER JM, MARTIN RW, SCHIFF MH, WHITMORE JBet WHITE BW: Etanercept treatment in adults with established rheumatoid arthritis: 7 years of clinical experience, J.Rheumatol., Vol. 33(5), 854-861., 2006
    Organism:Clinical Immunology and Rheumatology,University of Alabama at Birmingham, Birmingham, AL 35294-7201, USA larrymoreland@cccuabeduFAU - Moreland, Larry W
    Abstract:OBJECTIVE: To evaluate safety and efficacy of longterm etanercept treatment in patients with disease modifying antirheumatic drug (DMARD) refractory rheumatoid arthritis (RA). METHODS: Safety results are reported for 714 patients who received etanercept in one of 7 initial trials or a longterm extension. Efficacy results are reported for 581 patients who enrolled in the extension. RESULTS: Of the 714 patients enrolled in the initial trials, 581 (81%) enrolled in the extension, and 388 (54%) patients are continuing to receive etanercept therapy. The longest individual treatment was 8.2 years, with 3139 total patient-years of etanercept exposure. Rates of serious adverse events (overall rate=14.8 events/100 patient-yrs), serious infections (overall rate=4.2 events/100 patient-yrs), cancer (overall rate=1.0 events/100 patient-yrs), and deaths (overall rate=0.7 events/100 patient-yrs) were stable each year, through 8 years of etanercept exposure. For 356 patients who completed 6 years of etanercept treatment, response rates were ACR20=73%, ACR50=52%, ACR70=27%, DAS28 CRP good response=52%, and DAS28 CRP remission=37% of patients. Similar responses occurred in 167 patients who completed Year 7. Doses of concomitant methotrexate or corticosteroids were reduced in many patients who maintained clinical responses. CONCLUSION: The safety profile of etanercept was consistent over time, with rates of adverse events similar to those reported for patients with RA in general. Durable clinical responses were observed in some patients for 7 years or more. The benefit-to-risk ratio for longterm etanercept treatment remains highly favorable

70. MRSIC M, STAVLJENIC-RUKAVINA A, FUMIC K, LABAR B, BOGDANIC V, POTOCKI K et KARDUM-SKELIN I: Croatian model of financing the treatment of diseases requiring expensive drugs, 144, 14-04-2001

71. MUNOZ R, ESPINOZA M, ESPINOZA O, ANDRADE A, BRAVO Eet GONZALEZ F: Cyclosporine-associated leukoencephalopathy in organ transplant recipients: experience of three clinical cases, Transplant.Proc., Vol. 38(3), 921-923., 2006
    Organism:Department of Nephrology, Hospital del Salvador, Santiago, Chile romunoz@msncomFAU - Munoz, R
    Abstract:Leukoencephalopathy is a structural alteration of cerebral white matter mainly involving damage to myelin. Several reports have linked cyclosporine (CsA) with this alteration. The clinical features vary from qualitative alterations of consciousness to neurological deficits. Magnetic resonance imaging (MRI) of the brain demonstrates the damage to the white matter, which is essential for the differential diagnosis. We describe three clinical cases of leukoencephalopathy. The first case is a 43-year-old man received a cadaveric kidney transplant using immunosuppression with of mycophenolate mofetil, prednisone, and CsA. Four months later he developed meningism and bilateral sixth nerve palsy. The second case is a 50-year-old man with a cadaveric kidney transplant received immunosuppressive treatment with azathioprine and prednisone. As a result of gouty arthritis of the ankle, azathioprine was replaced with CsA to allow addition of allopurinol. Two weeks later he developed confusion and personality changes. The third case is a 16-year-old man received a orthotopic liver transplant. Postoperatively he suffered generalized tonic-clonic seizures. In all patients the CsA levels were toxic and signs of neurological alterations were present on MRI. All patients recovered rapidly after CsA withdrawal

72. NGUYEN THI TH, ZLACKA D, VAVRINCOVA P, SEDLACEK Pet HROMADNIKOVA I: Detection of antibodies against 60-, 65- and 70-kDa heat shock proteins in paediatric patients with various disorders using Western blotting and ELISA, Clinical Chemistry and Laboratory Medicine, Vol. 44, 442-449., 2006
    Organism:Charles Univ, Fac Med 2, Dept Paediat, Cell Biol Lab, V Uvalu 84, Prague 15006 5, Czech Republic Czech Republic
    Abstract:Background: We examined antibodies against 60-, 65- and 70-kDa heat shock proteins ( HSPs) in paediatric healthy individuals, patients with juvenile idiopathic arthritis ( JIA) and those undergoing allogeneic stem-cell transplantation for various malignant and nonmalignant diseases.Methods: Western blotting and ELISA were used to examine HSP-directed humoral immune responses.Results: Using ELISA we detected anti-Hsp60, -Hsp65 and -Hsp70 IgG antibodies in patient sera before, during and after conditioning and at all post-transplant times, as well as in JIA patients and controls. Western blotting showed positivity for anti-Hsp60 and anti-Hsp65 antibodies in all samples with a HSP concentration of 0.5 mu g/lane. However, anti-Hsp70 antibodies were not detected at all when both sodium dodecyl sulphate polyacrylamide gel electrophoresis ( SDS-PAGE) and native PAGE were used, except for one JIA patient, for whom a positive signal was only achieved in native PAGE when Hsp70 was increased to 2 mu g/lane and serum dilution decreased to 1:10.Conclusion: Western blotting is convenient for the detection of anti-Hsp60 and anti-Hsp65 antibodies, but it is not sensitive enough for the detection of anti-Hsp70 antibodies. ELISA, which is more sensitive, might be preferentially used to screen anti-Hsp60, -Hsp65 and -Hsp70 antibodies in sera of ch ildren with various disorders

73. NIGROVIC PAet WHITE PH: Care of the adult with juvenile rheumatoid arthritis, Arthritis &amp; Rheumatism, Vol. 55, 208-216., 2006
    Organism:Brigham and Womens Hosp, CAPRI, Div Rheumatol Allergy and Immunol, 1 Jimmy Fund Way,Smith 536, Boston, MA 02115 USA USA
    Abstract:

74. NUNEZ C, ALECSANDRU DM, MENDOZA JL, URCELAY E, DIAZ-RUBIO M, DE LA CONCHA EGet MARTINEZ A: Genetic markers linked to rheumatoid arthritis are also strongly associated with articular manifestations in ulcerative colitis patients, Hum.Immunol., Vol. 67(4-5), 324-330., 2006
    Organism:Department of Clinical Immunology, Hospital Clinico San Carlos, Madrid, Spain connunez@uscesFAU - Nunez, Concepcion
    Abstract:Ulcerative colitis is often accompanied by the development of extraintestinal, mainly articular, manifestations. Genetic differences could be underlying that clinical heterogeneity. We performed a case-control study to determine whether TNFab microsatellites or HLA-DR alleles were associated with the development of articular manifestations in patients with ulcerative colitis. With that aim, a total of 84 ulcerative colitis patients with articular manifestations and 172 without them were genotyped for TNFab microsatellites and HLA-DR. A healthy control sample (n = 595) was also included for comparative purposes. Haplotypes were inferred with the Arlequin software. The influence of HLA-DRB1*0103 and HLA-B27, factors previously known to be associated with extraintestinal manifestations, was specifically addressed. We observed that TNFa6b5 minihaplotype increases the susceptibility to developing articular manifestations in ulcerative colitis patients (p = 0.003, OR = 2.39). The locus HLA-DR does not appear to be involved in these extraintestinal manifestations by itself; however, the frequency of subjects carrying TNFa6b5 in combination with DR1, DR7, or DR11 is very significantly increased in patients with articular manifestations (p = 3.9 x 10(-8)). The associations found were independent of DRB1*0103 and HLA-B27. Thus, it seems that the development of articular manifestations in ulcerative colitis patients appears to be influenced by some genetic factor(s) present in some major histocompatibility complex haplotypes

75. PASSARELLI CM, ROIZENBLATT S, LEN CA, MOREIRA GA, LOPES MC, GUILLEMINAULT C, TUFIK Set HILARIO MARIA OE: A case-control sleep study in children with polyarticular juvenile rheumatoid arthritis, Rinsho Ganka, Vol. 33, 796-802., 2006
    Organism:Univ Fed Sao Paulo, Dept Psicobiol, Av Angelica 1045 Andar 5, BR-01227100 Sao Paulo, Brazil Brazil
    Abstract:Objective. To investigate the relationship between clinical manifestations and sleep abnormalities in patients with juvenile rheumatoid arthritis (JRA).Methods. Twenty-one patients with active polyarticular JRA and 20 healthy controls were enrolled consecutively. Pain and functional impairment were assessed with standardized, validated Brazilian questionnaires. Sleep evaluation was based on parent reporting of their child's sleep habits and polysomnography subjects underwent an adaptation night in the sleep laboratory. Sleep architecture was analyzed and spectral analysis of non-rapid eye move ment (REM) sleep was carried out by electroencephalography.Results. Patients with JRA exhibited higher indexes of periodic leg movements (PLM, p = 0.02). isolated leg movements (LM), and arousals, as well as increases in alpha activity in non-REM sleep (all p &lt; 0.01), in spite of similar frequency of sleep complaints in comparison to controls. Among JRA patients, greater alpha activity in non-REM sleep was observed in the participants with greater joint involvement assessed by the Escola Paulista de Medicina-Pediatric Range of Motion Scale (p = 0.03) or joint count (p = 0.02). Correlation was observed between morning stiffness and PLM and/or LM (r(S) = 0.75, Sr = 0.74, p &lt; 0.001 for both), and between self-rating scores of pain and al pha activity in non-REM sleep (r(S) = 0.74, p &lt; 0.001).Conclusion. Pain symptoms and disability are related to sleep fragmentation in patients with active polyarticular JRA

76. PAY S, TURKC c, KALYONCU M, IMS c, BEYAN E, ERTENLI I, OZTURK MA, DUZGUN N, ERDEM H, OZBALKAN Z, KIRAZ S, KINIKLI G, BESBAS N, DINC c, ATES c, OLMEZ U, ALGUNERI M, TIRYAKI AYDINTUG c, BAKKALOG c, TURAN M, TURGAY M, KARAASLAN Y, TOPALOG c, DUMAN Met OZEN S: A multicenter study of patients with adult-onset Still's disease compared with systemic juvenile idiopathic arthritis|, 2006
    Organism:Adult-onset Still's disease (AOSD) has often been regarded as the adult spectrum of systemic juvenile idiopathic arthritis (sJIA). The present study aims to compare the clinical and laboratory features, the disease course and the response to treatment in patients having AOSD with those having sJIA. Retrospective review of all available data that were filled out by adult and paediatric rheumatologists from six centers using a standard data extraction form was performed. A total of 95 patients with AOSD and 25 patients with sJIA were recruited for the study. The frequency of fever, rash, myalgia, weight loss and sore throat was higher in patients with AOSD. The pattern of joint involvement differed slightly. Laboratory findings were similar in both groups, except that liver dysfunction and neutrophilia were more common among adults. A multiphasic pattern dominated the childhood cases, whereas the most frequent course was a chronic one in adults. Corticosteroids and methotrexate were the most commonly employed therapy; however, chloroquine was another popular therapy in the adult group. We showed a difference in the rate of clinical and laboratory features between patients with AOSD and those with sJIA. AOSD and sJIA may still be the same disease, and children may simply be reacting differently as the result of the first encounter of the putative antigens with the immune system. (c) Clinical Rheumatology 2006

77. PEAKE NJ, FOSTER HE, KHAWAJA K, CAWSTON TEet ROWAN AD: Assessment of the clinical significance of gelatinase activity in patients with juvenile idiopathic arthritis using quantitative protein substrate zymography, Annals of the Rheumatic Diseases, Vol. 65, 501-507., 2006
    Organism:Univ Newcastle Upon Tyne, Sch Clin Med Sci, Musculoskeletal Res Grp, Med Sch Cookson Bldg, Newcastle Upon Tyne NE2 4HH, Tyne and Wear, UK UK
    Abstract:Objective: To measure gelatinase activities in paired synovial fluid (SF) and serum of patients with juvenile idiopathic arthritis (JIA), and to assess how these activities relate to clinical and laboratory measures of disease activity.Methods: A quantitative protein substrate zymography method was adapted and validated for use with serum and SF. Bands of activity were measured by densitometry and correlated with standard laboratory indicators of inflammation: erythrocyte sedimentation rate and platelet count.Results: Gelatinase activity was found consistently in patients with JIA, with reproducible, quantified bands of activity corresponding to pro-matrix metallopro teinase-9 (pro-MMP-9), including the neutrophil associated lipocalin complex, and pro- and active forms of MMP-2. Both active MMP-2 and pro-MMP-9 were higher in JIA serum than in controls, though no differences were seen between patients grouped according to age, disease duration, or JIA subtype. However, SF MMP-9 correlated significantly with the laboratory indicators of inflammation, as did the relative level of active MMP-2.Conclusions: Both MMP-2 and MMP-9 gelatinolytic activities are raised during active JIA and associated with inflammatory activity regardless of age and disease duration, supporting a role for MMPs in the breakdown of joint components from early in disease. These MMPs may be specific markers of active joint destruction linked to inflammatory JIA, MMP-9 as a product of infiltrating cells, and the activation of MMP-2 produced within the joint

78. PEEK R, SCOTT-JUPP R, STRIKE H, CLINCH Jet RAMANAN AV: Psoriasis after treatment of juvenile idiopathic arthritis with etanercept, Ann.Rheum.Dis., Vol. 65(9), 1259, 2006
    Organism:Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, UK avramanan@hotmailcomFAU - Peek, R
    Abstract:

79. PHAROAH DS, VARSANI H, TATHAM RW, NEWTON KR, DE JAGER W, PRAKKEN BJ, KLEIN Net WEDDERBURN LR: Expression of the inflammatory chemokines CCL5, CCL3 and CXCL10 in juvenile idiopathic arthritis, and demonstration of CCL5 production by an atypical subset of CD8+ T cells, Arthritis Res.Ther., Vol. 8(2), R50, 2006
    Organism:Rheumatology Unit, Institute of Child Health, UCL, London, UKFAU - Pharoah, Daniel S
    Abstract:This study focuses upon three chemokines, namely CCL5, CXCL10 and CCL3, which are potential novel therapeutic targets in arthritis. The aim of the study was to analyse the expression and production of these three chemokines within the joints of children with juvenile idiopathic arthritis (JIA) of the oligoarticular and polyarticular subtypes. All three of these chemokines are highly expressed at the level of mRNA, with the most significant increase in mRNA levels being demonstrated for CCL5 when compared with matched peripheral blood samples and controls. We show that high levels of all three chemokines are present in synovial fluid of children with JIA. We investigate the major source of CCL5 from inflammatory synovial cells, which we show to be CD8+ T cells. This CD8+ synovial T cell population has an unexpected phenotype that has not been described previously, being CCR7- yet predominantly CD28+ and CD45RA-. These cells contain high levels of stored intracellular CCL5, and rapid release of CCL5 takes place on T cell stimulation, without requiring new protein synthesis. In addition, we demonstrate that CCL5 is present in synovial biopsies from these patients, in particular on the endothelium of small and medium sized vessels. We believe this to be the first in depth analysis of these mediators of inflammation in JIA

80. PHELAN JDet THOMPSON SD: Genomic progress in pediatric arthritis: recent work and future goals, Curr.Opin.Rheumatol., Vol. 18(5), 482-489., 2006
    Organism:William S Rowe Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
    Abstract:PURPOSE OF REVIEW: Pediatric arthritis is a heterogeneous group of chronic arthropathies that are influenced by complex genetic and perhaps environmental factors. Interacting genetic traits may one day be identified that provide the basis for predicting disease risk and other characteristics such as course, age of onset, and disease severity. The purpose of this review is to describe the recent progress towards identifying the multiple genes related to pediatric arthritis and understand how they relate to each other and to disease pathology. RECENT FINDINGS: Candidate gene studies are by far the most widely reported type of genetic studies to date for juvenile arthritis with only one genome-wide screen for juvenile rheumatoid/idiopathic arthritis published. Particular attention is paid to studies of candidate genes with potential immunological roles and those associated with other forms of autoimmunity. SUMMARY: Genomic studies may perhaps one day provide information to allow future classification systems of childhood arthritis to include molecular biomarkers as a complement to clinical observations, as well as understand how these genes or proteins relate to each other and to disease pathogenesis

81. POUYE A, FALL S, DIALLO S, NDONGO S, LEYE Y, SECK SM, LEYE A, KA MM, NIANG Aet MOREIRA DT: [Polyarticular gout in young adults: a curable rheumatic disease], Med.Trop.(Mars.)., Vol. 66(3), 273-276., 2006
    Organism:Clinique Medicale I, CHU A le Dantec docpouye@yahoofrFAU - Pouye, A
    Abstract:Juvenile chronic gout in its polyarticular deformative form has rarely been described in medical literature. We report a rare case of destructive polyarticular tophaceous gout in a 31-year-old Senegalese man. He consulted for bilateral asymmetric polyarthritis with deformities of the hands and feet that had been ongoing in recurrent episodes since the age of 18 years in association with tophus. He had received no previous medication. All laboratory investigations were normal except hyperuricemia 104 mg/l. Radiographs of affected joints demonstrated evidence of destructive polyarthritis, i.e., articular narrowing and osteo-condensation of the left great toe. The patient responded favourably to colchicine, allopurinol and diet. Gouty arthropathy must be differentiated from rheumatoid arthritis, psoriasic arthritis and distal chronic osteoarthrosis. In our case, definitive diagnosis of gouty arthropathy was based on chronic polyarthritis associated with tophus, hyperuricemia and therapeutic response to colchicine. Polyarticular gout can be suspected in case of chronic seronegative polyarthritis and diagnosis can be confirmed on the basis of plain radiographs and laboratory investigations showing uricemia. Treatment is effective, well tolerated and inexpensive

82. RAYMAEKERS A, FOETS B, WOUTERS Cet CASTEELS I: Visual outcome in children with juvenile idiopathic arthritis related uveitis, Bull.Soc.Belge Ophtalmol., Vol. (300), 67-72., 2006
    Organism:Department of Ophthalmology, University Hospitals, Leuven, BelgiumFAU - Raymaekers, A
    Abstract:PURPOSE: To determine the incidence and characteristics of uveitis in a cohort of patients with juvenile idiopathic arthritis (JIA) as well as the nature of treatment and the risk factors for visual loss. PATIENTS AND METHODS: Retrospective review of 52 patients with JIA, screened for uveitis between 1995 and 2005. The first group, presenting with symptoms of arthritis and uveitis, was diagnosed at screening. The second group presented with symptoms of uveitis, without any rheumatological complaints at the time of diagnosis. During follow-up, reactivation of uveitis and complications were registered and treated when indicated. RESULTS: Seventeen patients had symptoms of uveitis at time of presentation or developed uveitis at follow-up. Ten of this patient group had oligo-arthritis, 16 were antinuclear antibody (ANA) positive, 16 were girls. Three patients presented with ophthalmological symptoms without rheumatological complaints. In this group, complications were more pronounced. Treatment in all patients consisted of topical corticosteroids and dilating drops at different intervals. Visual acuity was good in most patients. CONCLUSION: In this retrospective study, the risk of uveitis was higher in ANA- positive girls with oligoarthritis. To reduce severe disease at presentation, earlier diagnosis of JIA, earlier referral for slit lamp examination and universal screening of vision in childhood are necessary

83. REIFF A, LOVELL DJ, ADELSBERG JV, KISS MH, GOODMAN S, ZAVALER MF, CHEN PY, BOLOGNESE JA, JR PC, REICIN ASet GIANNINI EH: Evaluation of the comparative efficacy and tolerability of rofecoxib and naproxen in children and adolescents with juvenile rheumatoid arthritis: a 12-week randomized controlled clinical trial with a 52-week open-label extension, J.Rheumatol., Vol. 33(5), 985-995., 2006
    Organism:Children's Hospital Los Angeles, Los Angeles, CA, USAFAU - Reiff, Andreas
    Abstract:OBJECTIVE: To compare the safety and efficacy of rofecoxib* to naproxen for the treatment of juvenile rheumatoid arthritis (JRA). METHODS: This was a 12-week, multicenter, randomized, double-blind, double-dummy, active comparator-controlled, non-inferiority study with a prespecified 52-week open-label active comparator-controlled extension. Children (ages 2-11 yrs) and adolescents (ages 12-17 yrs) received lower-dose (LD)-rofecoxib [0.3 mg/kg/day up to 12.5 mg/day (base study only)]; or higher-dose (HD)-rofecoxib (0.6 mg/kg/day up to 25 mg/day) or naproxen 15 mg/kg/day as oral suspensions. Adolescents received daily rofecoxib (LD) 12.5 (base study only) or (HD) 25 mg, or naproxen 15 mg/kg/day (maximum 1,000 mg/day) as tablets. The primary endpoint was the time-weighted average proportion of patients meeting the American College of Rheumatology Pediatric-30 (ACR Pedi 30) response criteria. A prespecified bound for the 95% confidence interval for the ratio of the percentage of ACR Pedi 30 responders was used to assess non-inferiority of treatment response between groups. Safety was assessed throughout the study. RESULTS: A total of 310 patients ages 2-17 years (181 (3/4) age 11) were randomized to receive LD-rofecoxib (N=109), HD-rofecoxib (N=100), or naproxen (N=101). The ACR Pedi 30 response rates following 12 weeks of treatment were 46.2%, 54.5%, and 55.1%, respectively. The relative rates of response compared to naproxen were 0.81 (95% CI 0.61, 1.07) and 0.98 (95% CI 0.76, 1.26) for LD- and HD-rofecoxib, respectively. Both rofecoxib doses were not inferior to naproxen. Patients (N=227) entering the extension received HD-rofecoxib or naproxen with efficacy maintained during the extension. All treatments were generally well tolerated throughout the study. CONCLUSION: Daily treatment of JRA patients with rofecoxib up to 12.5 or 25 mg was well tolerated, providing sustained clinical effectiveness comparable to naproxen 15 mg/kg. *On September 30, 2004, Merck & Co., Inc. announced the voluntary worldwide withdrawal of rofecoxib from the market

84. SAKURAI Y, FUKUDA K, NAKAJIMA M, YOSHIOKA A, MORIMOTO K, MIYAGAWA S, TAKETANI Fet HARA Y: Long-term follow-up of a patient with sporadic Blau syndrome|, 2006
    Organism:We report a female patient with sporadic Blau syndrome who was followed up for more than 10 years before being diagnosed at the age of 19. Recurrent skin, eye, and bone involvement started in infancy. Her initial clinical diagnosis was SLE and juvenile idiopathic arthritis (JIA) later. At 9 years old, the diagnosis of early-onset sarcoidosis (EOS) was confirmed with histopathologic diagnosis from skin biopsy. Since clinical manifestations of EOS are very similar to those of JIA, skin biopsy is essential for differential diagnosis. Underlying etiology of EOS had been unraveled to that date. DNA analysis revealed that the patient had a heterozygous missense mutation for CARD15/NOD2 (caspase recruitment domain-containing protein 15), the gene for an intracellular receptor for bacterial products in monocytes that transduces signals leading to NF-kappaB activation. This case showed an arg334trp mutation that had been reported in Blau syndrome that shows phenotypic overlap with EOS. As her parents showed no mutation in CARD15, she was diagnosed as having a sporadic Blau syndrome. She had been treated with oral and/or intravenous steroids for a long time. Since she had intraocular lens implantation due to steroid cataract at the age of 12, intravenous liposteroid, a lipid emulsion containing a water-soluble dexamethasone in lipid vesicles, was started with oral prednisolone. This treatment lessened the side effects of the steroid and allowed us to control the manifestation of EOS/Blau syndrome

85. SANTIAGO JL, MARTINEZ A, DE LA CH, FERNANDEZ-ARQUERO M, FIGUEREDO MA, DE LA CONCHA EGet URCELAY E: Evidence for the association of the SLC22A4 and SLC22A5 genes with type 1 diabetes: a case control study, BMC.Med.Genet., Vol. 7, 54, 2006
    Organism:Immunology Department, Hospital Universitario San Carlos, Madrid, Spain jlsanti@hotmailcomFAU - Santiago, Jose Luis
    Abstract:BACKGROUND: Type 1 diabetes (T1D) is a chronic, autoimmune and multifactorial disease characterized by abnormal metabolism of carbohydrate and fat. Diminished carnitine plasma levels have been previously reported in T1D patients and carnitine increases the sensitivity of the cells to insulin. Polymorphisms in the carnitine transporters, encoded by the SLC22A4 and SLC22A5 genes, have been involved in susceptibility to two other autoimmune diseases, rheumatoid arthritis and Crohn's disease. For these reasons, we investigated for the first time the association with T1D of six single nucleotide polymorphisms (SNPs) mapping to these candidate genes: slc2F2, slc2F11, T306I, L503F, OCTN2-promoter and OCTN2-intron. METHODS: A case-control study was performed in the Spanish population with 295 T1D patients and 508 healthy control subjects. Maximum-likelihood haplotype frequencies were estimated by applying the Expectation-Maximization (EM) algorithm implemented by the Arlequin software. RESULTS: When independently analyzed, one of the tested polymorphisms in the SLC22A4 gene at 1672 showed significant association with T1D in our Spanish cohort. The overall comparison of the inferred haplotypes was significantly different between patients and controls (chi2 = 10.43; p = 0.034) with one of the haplotypes showing a protective effect for T1D (rs3792876/rs1050152/rs2631367/rs274559, CCGA: OR = 0.62 (0.41-0.93); p = 0.02). CONCLUSION: The haplotype distribution in the carnitine transporter locus seems to be significantly different between T1D patients and controls; however, additional studies in independent populations would allow to confirm the role of these genes in T1D risk

86. SARAUX A, FAUTREL B, MAILLEFERT JF, FLIPO RM, KAYE O, LAFFORGUE P, GOURVES Ket GUILLEMIN F: Laboratory and imaging studies used by French rheumatologists to evaluate patients with early arthritis, J.Rheumatol., Vol. 33(5), 897-902., 2006
    Organism:Rheumatology Units of the Brest, Dijon, Paris La Pitie, Lille, and Marseille La Timone Teaching Hospitals, France AlainSaraux@univ-brestfrFAU - Saraux, Alain
    Abstract:OBJECTIVE: To conduct a practice survey of laboratory and imaging studies used by French rheumatologists to identify the cause of recent-onset arthritis. METHODS: We selected a random sample of 210 rheumatologists, who were asked to recruit all patients with recent-onset arthritis (at least one joint involved, for less than one year) during a 2 week period, and to record laboratory and imaging studies performed. Results were analyzed in the overall group, in diagnostic subgroups, and in clinical presentation subgroups. RESULTS: The 119 rheumatologists who participated recruited 104 patients. Investigations done in 50% to 75% of patients were blood cell counts; erythrocyte sedimentation rate; serum assays of C-reactive protein, rheumatoid factors, antinuclear antibodies; and hand radiographs. Investigations in 50% to 74% of patients were serum ASAT/ALAT, creatinine, and uric acid; and foot radiographs. Finally, 25% to 49% of patients were tested for proteinuria; antikeratin antibodies; hepatitis B, hepatitis C, and Lyme serologies; creatine phosphokinase; blood iron; HLA-B27; and radiographs of chest and pelvis. No differences were found between investigations in patients with suspected rheumatoid arthritis and/or undifferentiated arthritis and those in other patients. In contrast, suspected diagnoses and presence of extraarticular manifestations classically associated with specific diseases modified the selection of investigations. CONCLUSION: Although considerable variability occurred, our study suggests that a limited panel of laboratory and imaging studies is performed in at least 25% of patients with recent-onset arthritis, regardless of clues suggesting a specific diagnosis

87. SAWHNEY Set MAGALHAES CS: Paediatric rheumatology--a global perspective, Best.Pract.Res.Clin.Rheumatol., Vol. 20(2), 201-221., 2006
    Organism:Department of Paediatric Rheumatology, Centre for Child Health, Sir Ganga Ram Hospital, New Delhi 110060, India sujatasawney@vsnlnetFAU - Sawhney, Sujata
    Abstract:This chapter aims to give a global perspective to paediatric rheumatology. The main points covered are the incidence, recognition of paediatric autoimmune diseases, and ethnic/geographic distribution. The most prevalent disease is juvenile idiopathic arthritis; robust data are still required for childhood-onset systemic lupus erythematosus, dermatomyositis, and scleroderma. Mimicking or overlapping infections are a major challenge in developing countries, and immunization policies in our patients in these areas need specific attention. The delivery of paediatric rheumatology care is also overviewed. Discrepancies in health-care resources and priorities are found in developing countries. Although most anti-rheumatic treatments are available worldwide, they are prohibitively expensive in many countries. For more traditional anti-rheumatic drugs there is still an ongoing need for good core outcome data across the world to ensure valid comparisons. Parent/patient education has been implemented worldwide in paediatric rheumatology through the power of the Internet. Physician and undergraduate training goals must be met to facilitate competent musculoskeletal assessment, a proper understanding of age-dependent variations, diagnosis, referral to specialists, and improved standards of care

88. SHENG PY, KONTTINEN L, LEHTO M, OGINO D, JAMSEN E, NEVALAINEN J, PAJAMAKI J, HALONEN Pet KONTTINEN YT: Revision total knee arthroplasty: 1990 through 2002. A review of the Finnish arthroplasty registry, J.Bone Joint Surg.Am., Vol. 88(7), 1425-1430., 2006
    Organism:COXA Hospital for Joint Replacement, Tampere, FinlandFAU - Sheng, Pu-Yi
    Abstract:BACKGROUND: National and regional arthroplasty registries have been used to study the results of primary total knee arthroplasties. The purpose of this paper was to present the results of revision total knee replacements and describe predictors of survival of those replacements, with repeat revision as the end point. METHODS: The nationwide Finnish Arthroplasty Registry include