Bibliography April 2008

1. Anonymous, [Role of magnetic resonance imaging in the detection of chondral and osteochondral lesions in chronic juvenile arthritis], Vestn.Rentgenol.Radiol., Vol. (2), 16-22., 2007
   Organism:Chronic juvenile arthritis (CJA) is the most common inflammatory disease of joints in children. There are numerous studies showing the limited informative value of X-ray in the evaluation of CJA progression. Contrast-enhanced magnetic resonance imaging (MRI) using intravenous gadolinium is currently in the foreground in diagnosing arthritis in children, in infants in particular. Knee joints are most frequently afflicted in CJA, showing significant manifestations of the disease. The purpose of the study was to describe the patterns of changes in the nonossified epiphyseal and articular cartilages in the distal epiphyses of femurs in the knee joints of patients with manifestations of chronic juvenile arthritis and to define the role of contrast-enhanced MRI in evaluating the epiphyseal changes in this disease. Sixty-nine patients aged 1.5-14 years who have clinical laboratory and ultrasound signs of CJA lasting 6 months to 5 years underwent contrast-enhanced MRI for the evaluation of changes in the articular and nonossified epiphyseal cartilages. Intravenous contrast enhancement identified several specific features and patterns of epiphyseal changes: subchondral hyperemia of epiphyses and recorded thickened epiphyseal chondral vascular channels, chondral and osteochondral erosions as manifestations of changes in the growing epiphyseal cartilage and articular one in children with chronic arthritis. Thus, contrast-enhanced MRI allows differentiation of different patterns of epiphyseal changes in CJA

2. Anonymous, Early sulfasalazine in juvenile idiopathic arthritis, Arch.Dis.Child., Vol. 93(5), 413, 2008
   Organism:

3. ACEVEDO S, QUINONES K, RAO V, CERVANTES-CASTANEDA RAet FOSTER CS: Cataract surgery in children with juvenile idiopathic arthritis associated uveitis, Int.Ophthalmol.Clin., Vol. 48(2), 1-7., 2008
   Organism:

4. ALPIGIANI MG, HAUPT R, PARODI S, CALCAGNO A, POGGI Eet LORINI R: Coeliac disease in 108 patients with juvenile idiopathic arthritis: a 13-year follow-up study, Clin.Exp.Rheumatol., Vol. 26(1), 162, 2008
   Organism:

5. BALDASSARI CM, MITCHELL RB, SCHUBERT Cet RUDNICK EF: Pediatric obstructive sleep apnea and quality of life: a meta-analysis, Otolaryngol.Head Neck Surg., Vol. 138(3), 265-273., 2008
   Organism:Department of Otolaryngology-Head and Neck Surgery, Virginia Commonwealth University Medical Center, Richmond, VA 23298, USA cbaldassari@mcvh-vcueduFAU - Baldassari, Cristina M
   Abstract:OBJECTIVE: 1) To assess the quality of life (QOL) in children with obstructive sleep apnea (OSA) compared with QOL of children with chronic medical conditions, and 2) To determine QOL in children with OSA after adenotonsillectomy in short- and long-term follow-up. DATA SOURCES/REVIEW METHODS: A literature review on QOL in pediatric OSA using the PubMed database. RESULTS: The literature search yielded 10 articles that satisfied inclusion and exclusion criteria. In three studies, the Child Health Questionnaire (CHQ) survey was used to compare 193 patients who had OSA with 93 children who had juvenile rheumatoid arthritis (JRA) and with 815 healthy children. Of 12 CHQ subscale scores for children with OSA, 8 scores were significantly lower (indicating a poorer QOL) than controls. Children with OSA scored 19.23 points lower than healthy children in the subscale of parental impact-emotional. Children with OSA had QOL scores that were similar to those of children with JRA. In seven publications, 369 children with OSA undergoing adenotonsillectomy were studied by using the OSA-18 QOL instrument. The total OSA-18 score and each of the domain scores showed significant improvement (P < 0.0001) after adenotonsillectomy. At long-term follow-up, QOL scores remained significantly improved. CONCLUSIONS: Pediatric OSA has a significant impact on QOL. QOL in pediatric OSA is similar to that of children with JRA. Large improvements in QOL occur after adenotonsillectomy, and these findings are maintained in the long-term. The literature lacks control studies on QOL in pediatric OSA

6. BICA BE, GOMES NM, FERNANDES PD, LUIZ RRet KOATZ VL: Nitric oxide levels and the severity of juvenile idiopathic arthritis, Rheumatol.Int., Vol. 27(9), 819-825., 2007
   Organism:Clinica de Reumatologia, Hospital Universitario Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, BrazilFAU - Bica, Blanca Elena R G
   Abstract:In this study, we evaluate the distribution of nitric oxide (NO) in the serum of juvenile idiopathic arthritis (JIA) patients, correlating it with parameters of the severity of the disease. Ninety-seven patients with mean age 11.7 years and disease duration 4.8 years, showing active disease or not, grouped as oligoarticular (n = 34), polyarticular (n = 29) and systemic (n = 34) group, presenting uveitis and positive RF with erosive arthritis or active disease and erosions had significantly high levels of NO than the inactive ones. NO correlated with TNF-alpha in the oligoarticular subtype (P < 0.03), with pain in the polyarticular subtype with active disease (P < 0.04) and with ESR in the systemic subtype with active disease (P < 0.03). TNF-alpha concentration was high in all patients with active disease, accompanying NO production. The data confirm the production of NO in JIA patients, indicating a possible positive correlation between the production of NO and severity of the disease

7. BIRLEA SA, FAIN PRet SPRITZ RA: A Romanian population isolate with high frequency of vitiligo and associated autoimmune diseases, Arch.Dermatol., Vol. 144(3), 310-316., 2008
   Organism:FACMG, Human Medical Genetics Program, University of Colorado at Denver and Health Sciences Center, PO Box 6511, Mail Stop 8300, Aurora, CO 80045, USAFAU - Birlea, Stanca A
   Abstract:OBJECTIVE: To characterize the epidemiology and genetics of vitiligo and associated autoimmune diseases in a population isolate in Romania in which there is a high frequency of these diseases. DESIGN: Prospective and retrospective ascertainment of all patients and extended families with these disorders in the study community. SETTING: A geographically isolated community in the mountains of northern Romania. Patients Fifty-one affected individuals and their close relatives from 35 nuclear families in an extended kindred that effectively constitutes the entire community population. MAIN OUTCOME MEASURES: Demographic, phenotypic, and genetic aspects of vitiligo and other autoimmune diseases in the extended kindred. RESULTS: The frequencies of vitiligo and several other autoimmune diseases, including autoimmune thyroid disease, adult-onset autoimmune diabetes mellitus, and rheumatoid arthritis, are greatly elevated. The age of vitiligo onset in this village is relatively delayed, suggesting that the causes of vitiligo in this community may be somewhat atypical. Genetic segregation analysis is most consistent with a single major locus recessive model, although incomplete penetrance and heritability suggest that other genes and nongenetic factors likely influence occurrence of disease in homozygotes. CONCLUSIONS: The high frequency of vitiligo and other autoimmune diseases in this isolated inbred community and an unusual aspect of the vitiligo phenotype suggest that susceptibility to these disorders in this "special population" may be unusual, likely involving a major recessive gene. Whereas disease susceptibility seems to involve a major genetic component, actual onset of vitiligo in genetically susceptible individuals seems to require exposure to environmental triggers

8. BONGARTZ T: Tocilizumab for rheumatoid and juvenile idiopathic arthritis, Lancet., Vol. 371(9617), 961-963., 2008
   Organism:Department of Internal Medicine and Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA bongartztim@mayoeduFAU - Bongartz, Tim
   Abstract:

9. BONNER MJ, HARDY KK, WILLARD VW, ANTHONY KK, HOOD Met GURURANGAN S: Social Functioning and Facial Expression Recognition in Survivors of Pediatric Brain Tumors, J.Pediatr.Psychol., Vol. ., 2008
   Organism:Department of Psychiatry, Department of Surgery, Duke University Medical Center, Department of Psychology & Neuroscience, Duke University and Department of Pediatrics, Duke University Medical Center
   Abstract:OBJECTIVE: To assess social functioning and facial expression recognition skill in survivors of pediatric brain tumors (BT) as compared to children with juvenile rheumatoid arthritis (JRA). METHODS: The social functioning of 51 survivors of BT and 31 children with JRA was assessed using a facial expression recognition task, questionnaire ratings of social functioning, and an IQ screener. RESULTS: After controlling for estimated IQ, survivors of BT made significantly more errors interpreting adult facial expressions as compared to children with JRA. Additionally, history of therapy and diagnosis age predicted performance on the child portion of the facial recognition task. Finally, survivors of BT demonstrated significantly impaired social functioning across multiple measures when compared to children with JRA. CONCLUSIONS: Survivors of pediatric BT showed significant deficits in social functioning as compared to an illness comparison group. Errors in facial expression recognition represent another method for evaluating deficits that contribute to social outcomes

10. BROWN ES, WOOLSTON DJet FROL AB: Amygdala volume in patients receiving chronic corticosteroid therapy, Biol.Psychiatry., Vol. 63(7), 705-709., 2008
    Organism:Psychoneuroendocrine Research Program, Department of Psychiatry, University of Texas Southwestern Medical Center, Dallas, Texas 75390-8849, USA SherwoodBrown@UTSouthwesterneduFAU - Brown, E Sherwood
    Abstract:BACKGROUND: Hippocampal volume reduction and declarative memory deficits are reported in humans and animals exposed to exogenous corticosteroids. The amygdala is another brain structure involved in the stress response that has important interactions with the hypothalamic-pituitary-adrenal axis. To our knowledge, no studies in animals or humans have examined the impact of exogenous corticosteroid administration on the amygdala. We assessed amygdala volume in patients receiving chronic prescription corticosteroid therapy and control subjects with similar medical histories not receiving corticosteroids. METHODS: Fifteen patients on long-term prednisone therapy and 13 control subjects of similar age, gender, ethnicity, education, height, and medical history were assessed with magnetic resonance imaging. Amygdala volume was manually traced and compared between groups using a two-way analysis of variance (ANOVA). Correlations between amygdala volume, age, and corticosteroid dose/duration were assessed using Pearson's correlation coefficient. RESULTS: Compared with control subjects, corticosteroid-treated patients had significantly smaller amygdala volumes. Right amygdala volume correlated significantly with age in control subjects and with duration of corticosteroid therapy in patients. CONCLUSIONS: Patients receiving chronic corticosteroid therapy had smaller amygdala volumes than control subjects that correlated with duration of corticosteroid therapy. These findings suggest that corticosteroid exposure may be associated with changes in the amygdala as well as hippocampus

11. COHRAN VC, GRIFFITHS Met HEUBI JE: Bone Mineral Density in Children Exposed to Chronic Glucocorticoid Therapy, Clin.Pediatr.(Phila)., Vol. ., 2008
    Organism:The objective of this study was to determine the impact of glucocorticoid exposure on lumbar spine bone mineral density (BMD) in children while concurrently measuring their calcium intake, serum 25-OH vitamin D levels, and physical activity. Forty-three patients (4-18 years) with renal glomerular diseases, dermatomyositis, inflammatory bowel disease, juvenile rheumatoid arthritis, post-solid organ transplant, and Duchenne muscular dystrophy were studied. All received at least 5 mg per day of prednisone for more than 6 months. The mean BMD z score was 0 +/- 0.2 (range, -3.8 to +3.3) with 2 patients (5%) having z scores less than -2. The mean daily calcium intake was 1147 +/- 145 g, with 1 patient having hypovitaminosis D (<15 ng/mL). The mean physical activity level was 7.8 +/- 0.8 h/wk. The small reductions in BMD observed in our population suggest that screening is likely not warranted in all children with chronic glucocorticoid exposure

12. DELL'ERA L, FACCHINI Ret CORONA F: Knee synovectomy in children with juvenile idiopathic arthritis, J.Pediatr.Orthop.B., Vol. 17(3), 128-130., 2008
    Organism:aClinica Pediatrica De Marchi bOrthopedic Clinic, University of Milan, Milan, Italy
    Abstract:The purpose of the study was to assess the outcomes of knee synovectomies in children with juvenile idiopathic arthritis. Thirty-one arthroscopic synovectomies were performed in 19 children (six oligoarthritis, 20 polyarthritis, five psoriatic arthritis). The percentage of recurrence in the group with oligoarthritis was 67%, in the group with polyarthritis was 95%, whereas all psoriatic arthritis recurred. The overall mean survival (i.e. free from recurrence) was 1.05 years (95% confidence interval, 0.74-1.35). Mean survival time was 1.69, 0.80 and 1.30 years, respectively, for oligoarthritis, polyarthritis and psoriatic arthritis. After synovectomy we observed two complications: thrombophlebitis of the omolateral superior femoral vein and septic arthritis. In conclusion, the mainstay of therapy for juvenile idiopathic arthritis remains medical treatment and intensive physiotherapy. The aim of arthroscopic synovectomy is to allow to make the most of nonsurgical therapy. It revealed more accurate and less invasive results than open synovectomy, maintained the range of motion of the joint, allowed early mobilization and required shorter hospitalization. Best results were observed in the group of oligoarthritis

13. DONN R, ELLISON S, LAMB R, DAY T, BAILDAM Eet RAMANAN AV: Genetic loci contributing to hemophagocytic lymphohistiocytosis do not confer susceptibility to systemic-onset juvenile idiopathic arthritis, Arthritis Rheum., Vol. 58(3), 869-874., 2008
    Organism:Arthritis Research Campaign Epidemiology Unit, University of Manchester, Manchester, UK Rachelledonn@manchesteracukFAU - Donn, Rachelle
    Abstract:OBJECTIVE: To investigate whether single-nucleotide polymorphisms (SNPs) within the genes PRF1, GZMB, UNC13D, and Rab27a, which are involved in natural killer cell dysfunction and known to contribute to the risk of hemophagocytic lymphohistiocytosis (HLH), confer an increased risk of susceptibility to systemic-onset juvenile idiopathic arthritis (JIA). METHODS: Four SNPs across the PRF1 gene locus, 5 for GZMB, 7 for UNC13D, and 11 for Rab27a were investigated using MassArray genotyping in 133 UK Caucasian patients with systemic-onset JIA and 384 ethnically matched unrelated control subjects. Additional control genotypes were accessed from the data generated by the Wellcome Trust Case Control Consortium. RESULTS: No significant association was found between any SNP within the 4 selected loci and systemic-onset JIA, by either single-point or haplotype analysis. CONCLUSION: The results of this study demonstrate that genes involved in HLH do not confer a significant risk of association with systemic-onset JIA

14. FALCINI F, CIMAZ R, RICCI L, FANNER S, DE MARTINO Met CERUSO M: A boy with bizarre hands mimicking an inflammatory chronic disease, Clin.Exp.Rheumatol., Vol. 25(5), 790-791., 2007
    Organism:

15. FINCKH Aet GABAY C: At the horizon of innovative therapy in rheumatology: new biologic agents, Curr.Opin.Rheumatol., Vol. 20(3), 269-275., 2008
    Organism:Division of Rheumatology, University Hospitals of Geneva and University of Geneva, SwitzerlandFAU - Finckh, Axel
    Abstract:PURPOSE OF REVIEW: To review the rational and the results regarding the use of novel biologic agents in inflammatory rheumatic diseases. RECENT FINDINGS: Recent findings show that excessive IL-1 processing and release contribute to different rheumatic conditions, including periodic fever syndromes, systemic-onset juvenile idiopathic arthritis, adult Still's disease, and crystal-induced arthritis. Preliminary results indicate that administration of IL-1 receptor antagonist and other IL-1 inhibitors improves these conditions. IL-6 also plays a major role in the control of inflammatory responses. Several clinical trials have shown that inhibition of IL-6 by a monoclonal antibody against its receptor is efficacious in rheumatoid arthritis and systemic-onset juvenile idiopathic arthritis patients. Accumulating evidence indicates that other cytokines, including IL-15, IL-18, and IL-21 may also play an important role in rheumatoid arthritis. Several signaling pathways involved in the immune and inflammatory responses may also constitute novel targets. Preliminary data on an agent targeting the Janus kinase/Signal transducer and activators of transcription pathway are encouraging. SUMMARY: Beyond tumor necrosis factor alpha targeting, the use of inhibitors against other cytokines and cytokine-induced intracellular responses is leading to a promising therapy in the future

16. FUCHS CE, SINNEMA G, VAN GEELEN SM, HERMANS HJet KUIS W: Self-investigation to explore the impact of juvenile arthritis on adolescent life: A case-study, Patient.Educ.Couns., Vol. ., 2008
    Organism:Department of Pediatric Psychology, Wilhelmina Children's Hospital, University Medical Center Utrecht, The Netherlands
    Abstract:OBJECTIVE: To gain insight into the personal experience and feelings of an adolescent with a chronic disease. METHODS: We report on the application of the self-confrontation method (SCM), illustrated by a case-example of an adolescent with juvenile idiopathic arthritis. RESULTS: Although taken at face value she was not impeded by the arthritis, through self-assessment with the SCM this adolescent acknowledged and addressed the emotional struggle to keep the arthritis secret and to constantly test the physical limits of her body. After the process of self-reflection, the adolescent showed a better integration of her arthritis experiences into her life story. CONCLUSION: With the SCM the adolescent could explore her own functioning and well-being on a manifest, as well as on an emotional and motivational level. PRACTICE IMPLICATIONS: In future research, by studying the self-investigations of a group of adolescents with chronic diseases, common risk factors for the development of a stable identity during adolescence might be identified. In clinical care, the SCM promotes self-knowledge, allowing for an intrinsic motivation to deal with the emotional impact of the disease

17. GATTORNO M, PICCINI A, LASIGLIE D, TASSI S, BRISCA G, CARTA S, DELFINO L, FERLITO F, PELAGATTI MA, CAROLI F, BUONCOMPAGNI A, VIOLA S, LOY A, SIRONI M, VECCHI A, RAVELLI A, MARTINI Aet RUBARTELLI A: The pattern of response to anti-interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis, Arthritis Rheum., Vol. 58(5), 1505-1515., 2008
    Organism:G Gaslini Institute, Genoa, Italy
    Abstract:OBJECTIVE: To assess the clinical response to interleukin-1 (IL-1) blockade and in vitro IL-1beta and IL-18 secretion in patients with systemic-onset juvenile idiopathic arthritis (JIA). METHODS: Twenty-two patients with systemic-onset JIA were treated with the IL-1 receptor antagonist (IL-1Ra) anakinra. Monocytes from 18 patients and 20 healthy donors were activated by different Toll-like receptor ligands. Intracellular and secreted IL-1beta and IL-18 were analyzed by Western blotting and enzyme-linked immunosorbent assay. RESULTS: Ten patients with systemic-onset JIA exhibited a dramatic response to anakinra and were classified as complete responders. Eleven patients had an incomplete response or no response, and 1 patient could not be classified in terms of response. Compared with patients who had an incomplete response or no response, complete responders had a lower number of active joints (P = 0.02) and an increased absolute neutrophil count (P = 0.02). In vitro IL-1beta and IL-18 secretion in response to various stimuli was not increased and was independent of treatment efficacy. Likewise, secretion of IL-1Ra by monocytes from patients with systemic-onset JIA was not impaired. An overall low level of IL-1beta secretion upon exposure to exogenous ATP was observed, unrelated to treatment responsiveness or disease activity. CONCLUSION: Two subsets of systemic-onset JIA can be identified according to patient response to IL-1 blockade. The 2 subsets appear to be characterized by some distinct clinical features. In vitro secretion of IL-1beta and IL-18 by monocytes from patients with systemic-onset JIA is not increased and is independent of both treatment outcome and disease activity

18. GORSKA A, URBAN M, BARTNICKA M, ZELAZOWSKA-RUTKOWSKA Bet WYSOCKA J: Bone mineral metabolism in children with juvenile idiopathic arthritis - preliminary report, Ortop.Traumatol.Rehabil., Vol. 10(1), 54-62., 2008
    Organism:Division of Family Medicine and Environmental Nursing, Medical University of BialystokFAU - Gorska, Anna
    Abstract:Introduction: There are very few reports assessing bone mineral mass and its metabolism in the course of juvenile idiopathic arthritis (JIA). Study objective: To assess the levels of selected serum markers of bone formation (OCN) and resorption (CTx) in JIA children.Material and methods: The study involved 52 children with JIA diagnosed according to the EULAR criteria of 1997, aged 6-18 years. All patients underwent densitometric measurements using dual-energy X-ray absorptiometry (DXA) to assess TBBMD (g/cm(2)), Spine BMD (g/cm(2)), Z-score for SBMD, TBBMC (g), and LBM (g). The following parameters were determined in blood serum: the level of osteocalcin (OCN) and C-terminal type I alpha-collagen chain telopeptide (CTx) using the Elecsys (R)2010 system (N-MID Osteocalcin(R), Beta-CrossLaps(R)). A gender- and age-matched control group consisted of 16 healthy children.Results: The mean concentrations of both osteocalcin (p<0.001) and CTx (p<0.005) were significantly higher in JIA patients as compared to the healthy controls (OCN 113.2+/-54.9 ng/ml vs. 70.2+/-48.3 ng/ml; CTx 1.4+/-0.5 mug/l vs. 1.2 +/-0.45 mug/l). The concentrations of the bone turnover markers were significantly reduced in children with higher degrees of joint destruction compared to those with anatomically normal joints (p<0.05). The mean concentration of CTx showed a significant negative correlation with the TB BMC/LBM Z-score (p<0.05). Reduced bone mass (Z-score for SBMD< -2.0) was found in 23.6% of the affected children.<br /> Conclusions: The JIA patients had elevated levels of OCN and CTx compared to the healthy controls. Reduced bone turnover was observed in children with higher degrees of joint destruction

19. GRIPENTROG JM, MILLS JS, SAARI GJet MIETTINEN HM: Variable responses of formyl peptide receptor haplotypes toward bacterial peptides, Immunogenetics., Vol. 60(2), 83-93., 2008
    Organism:Department of Microbiology, Montana State University, MT 59717, USAFAU - Gripentrog, Jeannie M
    Abstract:The chemoattractant neutrophil formyl peptide receptor (FPR) binds bacterial and mitochondrial N-formylated peptides, which allows the neutrophils to find the bacterial source and/or site of tissue damage. Certain inflammatory disorders may be due in part to an impaired innate immune system that does not respond to acute bacterial damage in a timely fashion. Because the human FPR is encoded by a large number of different haplotypes arising from ten single-nucleotide polymorphisms, we examined the possibility that some of these haplotypes are functionally distinct. We analyzed the response of three common FPR haplotypes to peptides from Escherichia coli, Mycobacterium avium ssp. paratuberculosis, and human mitochondria. All three haplotypes responded similarly to the E. coli and mitochondrial peptides, whereas one required a higher concentration of the M. avium peptide fMFEDAVAWF for receptor downregulation, receptor signaling, and chemotaxis. This raises the possibility of additional bacterial species differences in functional responses among FPR variants and establishes a precedent with potentially important implications for our innate immune response against bacterial infections. We also investigated whether certain FPR haplotypes are associated with rheumatoid arthritis (RA) by sequencing FPR1 from 148 Caucasian individuals. The results suggested that FPR haplotypes do not significantly contribute toward RA

20. HAN C, SMOLEN JS, KAVANAUGH A, VAN DER HD, BRAUN J, WESTHOVENS R, ZHAO N, RAHMAN MU, BAKER Det BALA M: The impact of infliximab treatment on quality of life in patients with inflammatory rheumatic diseases, Arthritis Res.Ther., Vol. 9(5), R103, 2007
    Organism:Centocor Research and Development, Inc, 200 Great Valley Parkway, Malvern, Pennsylvania 19355, USA chan3@cntusjnjcomFAU - Han, Chenglong
    Abstract:In this study, we compare the health-related quality of life (HRQoL) of patients with moderate-to-severe rheumatoid arthritis (RA), psoriatic arthritis (PsA), and ankylosing spondylitis (AS), and study the effect of treatment with infliximab on the HRQoL of patients with these diseases. Short Form Health Survey-36 (SF-36) data from the placebo-controlled phases of 4 studies of infliximab in patients with inflammatory rheumatic diseases (n = 1990) were evaluated. Data came from the Anti-TNF Trial in Rheumatoid Arthritis with Concomitant Therapy (ATTRACT) (n = 428), the Safety Trial for Rheumatoid Arthritis with REMICADE Therapy (START) (n = 1083), the Ankylosing Spondylitis Study for the Evaluation of Recombinant Infliximab Therapy (ASSERT) (n = 279), and the Infliximab Multinational Psoriatic Arthritis Clinical Trial II (IMPACT II) (n = 200). SF-36 assessments were made at weeks 0, 10, 30, and 54 in ATTRACT, weeks 0, 6, and 22 in START, weeks 0, 12, and 24 in ASSERT, and weeks 0 and 14 in IMPACT II. All patient populations had significantly impaired physical aspects of HRQoL at baseline relative to the general population of the United States, and the magnitude of impairment was similar across the diseases. Mean baseline physical component summary scores were 29 in the RA cohort, 32 in the PsA cohort, and 29 in the AS cohort. In all 3 diseases, patients who received infliximab showed significant improvement in physical component summary scores compared with those who received placebo. The magnitude of the difference of improvement (effect size, 95%CI) between infliximab and placebo groups was similar in the AS (10.1, 9.2-11.0), PsA (8.6, 7.8-9.4), and RA (10.1, 9.2-11.0) cohorts. Patients with RA and those with PsA treated with infliximab also showed greater improvement in the mental component summary score than those in the placebo group with an effect size of 4.6 (4.2-5.1) in RA and 2.7 (2.4-3.1) in PsA. Patients in large randomized controlled studies of infliximab in RA, PsA, and AS had similar impairment in physical aspects of HRQoL at baseline and showed significantly greater improvement in HRQoL after treatment with infliximab

21. HARJACEK M: [Pediatric rheumatic diseases--per aspera ad astra], Reumatizam., Vol. 54(2), 11-23., 2007
    Organism:Klinika za djecje bolesti Zagreb, Klaiceva 16, 10000 ZagrebFAU - Harjacek, Miroslav
    Abstract:Progress in the field of pediatric rheumatolgy has been extraordinary; subspecialty is accepted as essential, vital, indispensible and distinct from adult rheumatology. It is clear that arthritis in children is more heterogeneous than RA. Although there are similarities between the inflammatory arthritides occuring in adults and children, RA and JIA appear to be distinct phenotypically with exception for the older child with RF-positive polyarticular arthritis. Progress in molecular biology has enabled us to diagnose those children earlier, and treat them more efficaciously with variety of drugs and biologic agents. In recent years a new group of hereditary autoinflammatory disorders has emerged. These rare syndromes are charaterized by recurrent episodes of seemingly unprovoked, intermittent inflammation. In the near future we will be able to distinguish various subtypes of autoimune/autoinflammatory diseases earlier in the course, have a better understanding of the biomarkers and other prognostic factors, and most importantly treat them earlier with extended set of various new exciting drugs and biological therapy

22. HAZEN MM, WOODWARD AL, HOFMANN I, DEGAR BA, GROM A, FILIPOVICH AHet BINSTADT BA: Mutations of the hemophagocytic lymphohistiocytosis-associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis, Arthritis Rheum., Vol. 58(2), 567-570., 2008
    Organism:Children's Hospital Boston, Boston, Massachusetts, USAFAU - Hazen, Melissa M
    Abstract:The clinical syndromes of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are both characterized by dysregulated inflammation with prolonged fever, hepatosplenomegaly, coagulopathy, hematologic cytopenias, and evidence of hemophagocytosis in the bone marrow or liver. While HLH is either inherited or acquired, children with severe rheumatic diseases, most notably systemic juvenile idiopathic arthritis, are at risk for MAS. The phenotypic similarity between HLH and MAS raises the possibility that they share common pathogenetic mechanisms. Familial forms of HLH have been attributed to mutations in the genes encoding perforin (PRF1) and Munc13-4 (UNC13D), among others, and are characterized by defective cytotoxic lymphocyte function. While some patients with systemic JIA have decreased levels of perforin protein expression and natural killer (NK) cell function, mutations of HLH-associated genes in patients with systemic JIA have not been reported. We report the case of an 8-year-old girl with systemic JIA without MAS who was found to have compound heterozygous mutations of UNC13D and reduced NK cell cytotoxic function. This case broadens the range of clinical phenotypes attributable to UNC13D mutations and offers new insights into the etiology and pathogenesis of systemic JIA

23. HERLIN Tet THASTUM M: [Pain and coping strategies in juvenile idiopathic arthritis], Ugeskr.Laeger., Vol. 170(8), 636-638., 2008
    Organism:Arhus Universitetshospital, Skejby, Borneafdelingen, Arhus N therlin@dadlnetdkFAU - Herlin, Troels
    Abstract:Pain is one of the primary symptoms of juvenile idiopathic arthritis (JIA). JIA patients have reduced pain tolerance and pain threshold compared to healthy controls. In children with JIA the greater use of coping strategies such as problem-solving, positive self-statements and distraction consistently have predicted less arthritis-related pain, even after controlling for relevant medical and demographic variables. Interventions specifically designed to modify maladaptive pain coping strategies and pain-related health beliefs may be effective in reducing pain in children with JIA

24. HERRINTON LJ, LIU L, SHOOR Set MINES D: Risk of lymphoproliferative cancer among patients with severe rheumatoid arthritis, 1996-2002, Ann.Rheum.Dis., Vol. 67(4), 574-575., 2008
    Organism:

25. HORNEFF G, DE BOCK F, FOELDVARI I, GIRSCHICK HJ, MICHELS H, MOEBIUS Det SCHMELING H: Safety and efficacy of combination of Etanercept and Methotrexate compared to treatment with Etanercept only in patients with juvenile idiopathic arthritis (JIA). Preliminary data from the German JIA Registry, Ann.Rheum.Dis., Vol. ., 2008
    Organism:Asklepios Clinic Sankt Augustin, Germany
    Abstract:OBJECTIVE: Etanercept monotherapy has been studied and approved for treatment of polyarticular JIA. The following study evaluates the safety and efficacy of combination therapy of Etanercept&Methotrexate compared to Etanercept monotherapy in JIA. PATIENTS AND METHODS: This is an open, non-randomized study on patients who failed to respond to at least 1 DMARD. 722 JIA patients in whom at least 1 item of follow-up data was recorded were identified. 118 patients treated with further slow acting drugs were excluded. 504 patients were treated with a combination of Etanercept&Methotrexate. 100 patients treated with Etanercept only were in the control group. Efficacy was calculated using the PedACR30/50/70-scores. Adverse events and serious adverse events were reported. RESULTS: After 12 months 55 patients in the monotherapy group and 376 patients in the Etanercept&Methotrexate group were available for comparison. For the intention-to-treat analysis 65 patients discontinuing treatment prematurely were included. All activity parameters decreased significantly in both treatment groups. After 12 months 81%/74%/62% of patients of the Etanercept&Methotrexate group and 70%/63%/45% of patients of the Etanercept monotherapy group achieved PedACR30/50/70 scores (p<0.05 for PedACR30, p<0.01 for PedACR70). The likelihood of achieving a PedACR70 increased with combination therapy with an odds ratio of 2.1 (CI 1.2-3.5). 25 infectious and 23 non-infectious SAE including three malignancies occurred in the Etanercept&Methotrexate group, 1 infectious and 3 non-infectious SAE in the single Etanercept group. CONCLUSIONS: The patients'disease activity improved during both Etanercept monotherapy and Etanercept&Methotrexate combination therapy. Tolerability in both treatment groups was comparable

26. IMMONEN K, FINNE P, HAKALA M, KAUTIAINEN H, PETTERSSON Tet GRONHAGEN-RISKA C: No Improvement in Survival of Patients with Amyloidosis Associated with Inflammatory Rheumatic Diseases -- Data from the Finnish National Registry for Kidney Diseases, J.Rheumatol., Vol. ., 2008
    Organism:From the Departments of Medicine, North-Karelia Central Hospital, Joensuu and Helsinki University Central Hospital, Helsinki; Rheumatism Foundation Hospital, Heinola; and the Department of Musculoskeletal Medicine and Rehabilitation, Medical School, University of Tampere, Tampere, Finland
    Abstract:OBJECTIVE: To assess the incidence and outcome of renal replacement therapy (RRT) among patients with amyloidosis associated with inflammatory rheumatic diseases. METHODS: Patients with amyloidosis entering RRT from 1987 to 2002 were identified from the Finnish Registry for Kidney Diseases. Five hundred two patients were identified, 80% of whom had amyloidosis associated with an underlying rheumatic disease. They were followed from the time of entering RRT until death or until the end of 2003 using the Finnish national mortality files. RESULTS: During the study period, there was no decline in the number of patients with amyloidosis entering RRT. Mean age of patients with rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) increased significantly from 1987 to 2002 (p < 0.001). Male sex and a diagnosis of JIA indicated an increased risk of mortality. The median survival time after entering RRT was 2.11 years for RA (95% CI 1.93 to 2.69), 2.37 years for ankylosing spondylitis (95% CI 1.11 to 4.31), and 3.05 years for JIA (95% CI 2.19 to 4.23). The 5-year survival rates among patients with the corresponding diagnoses were 18% (95% CI 14% to 23%), 30% (95% CI 14% to 48%), and 27% (95% CI 14% to 41%), respectively. CONCLUSION: No decline was seen in the number of patients with amyloidosis associated with inflammatory rheumatic diseases accepted for RRT, but over the years, the age of patients with RA or JIA entering RRT was seen to increase. The outcome of patients with amyloidosis and endstage renal disease associated with rheumatic diseases remains poor

27. IMMONEN K, SAVOLAINEN A, KAUTIAINEN Het HAKALA M: Longterm Outcome of Amyloidosis Associated with Juvenile Idiopathic Arthritis, J.Rheumatol., Vol. ., 2008
    Organism:From the Department of Medicine, North Karelia Central Hospital, Joensuu; Rheumatism Foundation Hospital, Heinola; and Department of Musculoskeletal Medicine and Rehabilitation, Medical School, University of Tampere, Tampere, Finland
    Abstract:OBJECTIVE: To determine the outcome of amyloidosis associated with juvenile idiopathic arthritis (JIA) in a hospital-based series. METHODS: Patient registers and amyloidosis biopsy files of the Department of Pediatrics of Rheumatism Foundation Hospital, the main tertiary center for inflammatory joint disorders in children in Finland, were scrutinized from 1976 to the end of 2003 to look for amyloidosis in patients under age 19 years. Medical records were reviewed and patients were interviewed by telephone. The causes of any deaths were obtained from death certificates. RESULTS: Twenty-four patients under age 19 years with biopsy-proven amyloidosis were found. As a sign of renal disease at the time of diagnosis of amyloidosis, 16 patients (67%) had proteinuria, but none had renal insufficiency. The 5-year survival rate of the series was 87.5% (95% CI 75% to 100%), and 10-year survival was 75% (54% to 92%). Ten patients (42%) out of the 24 died during a mean followup of 15.4 (range 1.5-27.6) years. The main cause of death was related to JIA in all patients but one. Patients treated with prednisolone alone from the diagnosis of amyloidosis onward had a mortality rate significantly higher than those taking disease modifying antirheumatic drugs and/or cytostatics (p = 0.001). At the end of followup, 14 patients (58%) were alive, 12 with normal renal function (3 of them had undergone renal transplantation), one had renal insufficiency, and one proteinuria. Proteinuria disappeared in 4 patients who were proteinuric (2 with nephrotic syndrome) at baseline, and their renal function remained normal. All the live patients had completed at least the 9 years of compulsory education, and 4 had academic degrees. Two female patients had delivered healthy children. CONCLUSION:The outcome of JIA-associated amyloidosis is poor. However, renal disease regressed in some patients under vigorous treatment. Successful treatment makes an active life possible for these patients

28. ISHIKAWA S, MIMA T, AOKI C, YOSHIO-HOSHINO N, ADACHI Y, IMAGAWA T, MORI M, TOMIITA M, IWATA N, MURATA T, MIYOSHI M, TAKEI S, AIHARA Y, YOKOTA S, MATSUBARA Ket NISHIMOTO N: Abnormal expression of the genes involved in cytokine networks and mitochondrial function in systemic juvenile idiopathic arthritis identified by DNA maicroarray analysis, Ann.Rheum.Dis., Vol. ., 2008
    Organism:Laboratory of Immune Regulation, Graduate School of Frontier Bioscience, Osaka University, Japan
    Abstract:OBJECTIVES: Systemic juvenile idiopathic arthritis (sJIA) is a rheumatic disease in childhood characterized by systemic symptoms and a relatively poor prognosis. Peripheral leukocytes are thought to play pathological roles in sJIA although the exact cause is still obscure. In this study, we aimed to clarify the cellular functional abnormality in sJIA. METHODS: We analyzed the gene expression profile in peripheral leukocytes from 51 patients with sJIA, 6 patients with poly-articular type JIA (polyJIA) and 8 healthy children utilizing DNA microarrays. Gene ontology analysis and network analysis were performed on the genes differentially expressed in sJIA to clarify the cellular functional abnormalities. Result: A total of 3,491 genes were differentially expressed in patients with sJIA compared to healthy individuals. They were functionally categorized mainly into a defense-response group and a metabolism group according to gene ontology, suggesting the possible abnormalities in these functions. In the defense-response group, molecules predominantly constituting IFN-gamma and TNF network cascades were up-regulated. In the metabolism group, oxidative phosphorylation-related genes were down-regulated, suggesting a mitochondrial disorder. Expression of mitochondrial DNA-encoded genes including cytochrome c oxidase (MT-CO) 1 and MT-CO2 were suppressed in patients with sJIA but not in patients with polyJIA or healthy children. However, nuclear DNA-encoded cytochrome c oxidases were intact. CONCLUSION: Our findings suggest that sJIA is not only an immunological disease but also a metabolic disease involving mitochondria disorder

29. KAMENOV B, VOJINOVIC J, NAJMAN S, DIMITRIJEVIC H, MITROVIC V, NIKOLIC Set NIKOLIC O: [Importance of immunoregulation and erythropoiesis estimation in disorders with activated immune system], Srp.Arh.Celok.Lek., Vol. 122 Suppl 1, 32-35., 1994
    Organism:Complicated immunoregulatory events in different disorders depend on the pathologic process itself and the phase of disease. Immune parameters aberrations in advanced phase of disease, compared to initial ones, may be significant, making hard the understanding of basic immunoregulatory process of the disease. Intending to explain immune mechanisms in different disorders with activation of the immune system as basic pathogenic event (infections, autoimmune diseases), particularly in advanced phase of the disease, on a parallel with standard laboratory investigations of erythropoiesis (hemoglobin, red blood cell count, hematocrit) the following examinations of the immune system were done: white blood cell count, immunophenotyping of lymphocytes, ability of the phagocytes to reduce NBT, chemiluminescent response after stimulation with opsonized and nonopsonized particles. Analysis of erythropoietic parameters in order to see the consequences of the regulatory mechanisms of the immune system (cytokines, interleukins) on hemopoiesis may enable us to understand the mechanisms of disease with the initial activation of the immune system. Finding anemia might be useful in defining the immunoregulatory mechanisms involved in particular pathologic process important for therapeutic approach

30. KANAKOUDI-TSAKALIDOU F, TZIMOULI V, PRATSIDOU-GERTSI P, CHRONOPOULOU Eet TRACHANA M: The significance of persistent newly developed autoantibodies in JIA patients under long-term anti-TNF treatment, Cytokine., Vol. ., 2008
    Organism:First Department of Pediatrics, Aristotle University, Ippokration General Hospital, 49 Konstantinoupoleos str, 54642 Thessaloniki, Greece
    Abstract:Objective: To study the significance of persistent (12 months) new autoantibodies, in Juvenile Idiopathic Arthritis (JIA) patients treated with either Infliximab (INFL) or Etanercept (ET) for 2 years. Patients-methods: 26 children under INFL (n=12) or ET (n=14) were prospectively studied. A large panel of autoantibodies was tested using indirect immunofluorescence (ANA, anti-dsDNA, anti-ENA, SMA, LKM, AMA, PCA, anti-R1, ATA), ELISA (ANA, anti-ENA, anti-cardiolipin, ANCA), immunoblotting assay (anti-ENA: anti-Ro, anti-La, anti-Sm, anti-URNP, anti-Jo, anti-Scl70, anti-centromere, anti-ribosomal and anti-histone) and rate nephelometry (RF). Results: Apart from the positive patients for ANA (13/26) and RF (2/26) prior to anti-TNF treatment, 6/26 patients (23%) developed new autoantibodies (SMA, anti-R1, ATA) which persisted for 12-50 months. None developed antibodies to nuclear antigens. In only one case, ATA was associated with the development of Hashimoto's thyroiditis. Conclusions: These findings indicate that in JIA patients in contrast to adult RA patients, development of new autoantibodies to various nuclear antigens is rare. Other non relevant to rheumatic diseases autoantibodies, may appear and persist for >12 months, but very rarely they may be related to clinical entities, especially in the presence of a positive family history of autoimmunity

31. KANG ESet LEE J: Genotypic analysis of Asp299Gly and Thr399Ile polymorphism of Toll-like receptor 4 in systemic autoimmune diseases of Korean population, Rheumatol.Int., Vol. 27(9), 887-889., 2007
    Organism:

32. LAUFER I, GREENFIELD JP, ANAND VK, HARTL Ret SCHWARTZ TH: Endonasal endoscopic resection of the odontoid process in a nonachondroplastic dwarf with juvenile rheumatoid arthritis: feasibility of the approach and utility of the intraoperative Iso-C three-dimensional navigation. Case report, J.Neurosurg.Spine., Vol. 8(4), 376-380., 2008
    Organism:Department of Neurological Surgery and, 2 Otolaryngology, Weill Cornell Medical College, New York Presbyterian Hospital, New York, New York 10021, USAFAU - Laufer, Ilya
    Abstract:The authors report a case of a nonachondroplastic dwarf with severe basilar invagination and compression of the cervicomedullary junction (CMJ) due to juvenile rheumatoid arthritis. Initially excellent reduction of the invagination and decompression of the CMJ was achieved using posterior fixation. However, 1 month postoperatively symptoms recurred and the authors found imaging evidence of recurrence as well. The patient subsequently underwent an endoscopic transnasal resection of the dens with assistance of Iso-C navigation. He recovered well and tolerated regular diet on postoperative Day 2

33. LEHMANN HW, PLENTZ A, VON LANDENBERG P, KUSTER RMet MODROW S: Different patterns of disease manifestations of parvovirus B19-associated reactive juvenile arthritis and the induction of antiphospholipid-antibodies, Clin.Rheumatol., Vol. 27(3), 333-338., 2008
    Organism:Department of Pediatrics, University of Giessen, Giessen, Germany hartwiglehmann@paediatmeduni-giessendeFAU - Lehmann, Hartwig W
    Abstract:Children with rheumatic oligo- and polyarthritis frequently establish persistent parvovirus B19 infections, which may be associated with the production of antiphospholipid antibodies. Reported in this paper are the data of five girls with polyarticular rheumatic diseases of different types and persistent parvovirus B19 infection associated in four cases with the presence of antibodies against phospholipids. Clinical parameters, virus load, and antiphospholipid-IgG levels were determined during an observation period up to 92 months. In two patients, erythema infectiosum preceded the development of arthritis and B19 viremia persisted. Two other girls showed antibodies against parvoviral structural proteins at time of the manifestation of the rheumatic disease. Subsequent samples also revealed persistent B19 infection. In the fifth patient, parvovirus B19-specific IgG antibodies were detected for the first time after 120 months of progressing disease at an age of 11 1/2 years. Five years later, quantitative polymerase chain reaction (PCR) revealed viral DNA. In a synovial tissue specimen subsequently obtained, parvovirus B19 structural proteins could be detected by immunohistochemistry. Three of five patients recovered completely without severe sequels. One patient is in remission under immunosuppressive therapy. The fifth patient suffers from progressive erosions despite intensive therapeutical efforts. In consequence, parvovirus B 19 should generally be taken into consideration as a trigger of various forms of juvenile arthritis and persistence of infection should be evaluated

34. LIN P, BHULLAR SS, TESSLER HHet GOLDSTEIN DA: Immunologic markers as potential predictors of systemic autoimmune disease in patients with idiopathic scleritis, Am.J.Ophthalmol., Vol. 145(3), 463-471., 2008
    Organism:Department of Ophthalmology, University of Illinois at Chicago, Chicago, Illinois 60612, USAFAU - Lin, Phoebe
    Abstract:PURPOSE: To determine the clinical value of serological testing in patients with idiopathic scleritis. DESIGN: Retrospective case series. METHODS: Medical records of patients with scleritis seen at an institutional referral center over an 11-year period were reviewed. RESULTS: Of 119 patients with scleritis seen at the University of Illinois Uveitis Clinic, 91 (76.5%) patients had no known etiology at initial presentation. Seventy of the 91 patients were tested for rheumatoid factor (RF), 19 (27.1%) of whom had a positive result. Ten (52.6%) of these RF positive patients were subsequently diagnosed with rheumatoid arthritis (RA) during a mean follow-up of 10.6 months (range, zero to 72 months), whereas only one of 51 (2.0%) RF negative patients developed RA, producing an odds ratio for developing RA in RF positive patients of 55.6 (95% confidence interval (CI) 7.8 to 369.8, P=.00001). Of the 70 patients who were tested for anti-neutrophil cytoplasmic antibody (ANCA), seven (10.0%) tested positive. Three (42.9%) of the ANCA positive patients subsequently developed Wegener granulomatosis (WG), whereas only two of 63 ANCA negative patients (3.2%) developed WG during a mean follow-up of 8.4 months (range, zero to 72 months). The odds ratio for developing WG in patients with idiopathic scleritis and a positive ANCA screen compared with a negative ANCA was 22.9 (95% CI 3.4 to 154.2, P=.006). CONCLUSIONS: The likelihood of patients with idiopathic scleritis developing RA and WG was increased if they had a positive RF or ANCA, supporting the role of immunologic marker testing in patients who present without systemic disease

35. LOVELL DJ, REIFF A, ILOWITE NT, WALLACE CA, CHON Y, LIN SL, BAUMGARTNER SWet GIANNINI EH: Safety and efficacy of up to eight years of continuous etanercept therapy in patients with juvenile rheumatoid arthritis, Arthritis Rheum., Vol. 58(5), 1496-1504., 2008
    Organism:Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
    Abstract:OBJECTIVE: To evaluate the safety and efficacy of up to 8 years of etanercept treatment in patients with polyarticular-course juvenile rheumatoid arthritis (JRA). METHODS: Patients with JRA who previously participated in a randomized controlled trial (RCT) of etanercept were eligible to receive etanercept in a long-term open-label extension (OLE) trial. Safety end points included the incidences of serious adverse events (SAEs), medically important infections (MIIs), and death. Efficacy end points included the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30), Pedi 50, Pedi 70, Pedi 90, and Pedi 100 criteria for improvement. RESULTS: Of the 69 patients originally enrolled in the RCT, 58 (84%) participated in the OLE, for a total of 318 patient-years of etanercept exposure. A total of 42 of the 58 patients (72%) entered the fourth year of continuous etanercept treatment, and 26 patients (45%) entered the eighth year. Sixteen patients (23% of those entering the RCT) reported 39 SAEs. The overall rate of SAEs (0.12 per patient-year) did not increase with long-term exposure to etanercept. The rate of MIIs (0.03 per patient-year) remained low; 1 new MII was reported in patients with >/=5 years of etanercept exposure. No cases of tuberculosis, opportunistic infections, malignancies, lymphomas, lupus, demyelinating disorders, or deaths were reported. An ACR Pedi 70 response or higher was achieved by 100% of patients with 8 years of data (11 of 11) and by 61% of patients according to the last observation carried forward data (28 of 46). CONCLUSION: These data suggest that the acceptable safety profile of etanercept therapy is maintained for up to 8 years in this population of JRA patients. Improvements in the signs and symptoms of JRA were also maintained for up to 8 years

36. MAHEVAS M, VAIDA I, LE PAGE L, SID-IDRIS S, ROYER B, GAREDI R, DAMAJ G, DUHAUT P, CLAISSE JF, DUCROIX JPet MAROLLEAU JP: [Haematopoietic stem cell transplantation in the treatment of autoimmune diseases], Rev.Med.Interne., Vol. 29(2), 115-121., 2008
    Organism:Service de medecine interne, hopital Nord, CHU d'Amiens, place Victor-Pauchet, 80054 Amiens cedex 1, France mahevasm@yahoofr <mahevasm@yahoofr>FAU - Mahevas, M
    Abstract:PURPOSE: During the past ten years, more than 1000 patients suffering from severe autoimmune disease have received an autologous haematopoietic stem cell transplant. These new therapeutic have been used in systemic sclerosis (scleroderma), multiple sclerosis, rheumatoid arthritis, juvenile idiopathic arthritis and systemic lupus erythematosus. CURRENT KNOWLEDGE AND KEY POINTS: Autologous haematopoietic stem cell transplantation has become a curative option for condition with very poor prognosis as severe systemic sclerosis, lupus erythematosus or other systemic diseases. This review summarizes the current experience in the phase I and II clinical trials in Europe and North America. We describe the main results and the limits of stem cell transplantation in systemic diseases. FUTURE PROSPECTS AND PROJECTS: Autologous haematopoietic stem cell transplant in the treatment of autoimmune disease has evolved from a experimental concept to a clinically feasible and powerful therapy for selected patients with severe disease

37. MCMILLAN R, BUSSEL JB, GEORGE JN, LALLA Det NICHOL JL: Self-reported health-related quality of life in adults with chronic immune thrombocytopenic purpura, Am.J.Hematol., Vol. 83(2), 150-154., 2008
    Organism:Scripps Research Institute, La Jolla, California, USA robtmcmillan@sbcglobalnetFAU - McMillan, Robert
    Abstract:Adult chronic immune thrombocytopenic purpura (ITP) is a disorder manifested by varying degrees of purpura and mucosal bleeding, rarely including intracranial hemorrhage. Therapy is aimed at increasing the patient's platelet count to safe levels and includes a wide variety of treatments. While the diagnosis, treatment, and prognosis of chronic ITP have been extensively discussed, the effect of ITP and its treatment on patient quality of life has not been evaluated in adults. In this study, the Short-Form 36 questionnaire was used to evaluate the health-related quality of life (HRQOL) of 73 adult ITP patients compared with that of the general U.S. population and of patients with six other relatively common chronic disorders. This study shows that the HRQOL of adult patients with ITP is significantly worse than that of the general U.S. population. It is also worse than that of patients with hypertension, arthritis, or cancer; similar to that of patients with diabetes; but better than that of patients with congestive heart failure or a missing or paralyzed limb. Future studies need to address the effects of treatment not only on the platelet count and bleeding but also on HRQOL

38. MIYAMAE T, NEMOTO A, IMAGAWA T, OHSHIGE K, MORI M, NISHIMAKI Set YOKOTA S: Cross-cultural adaptation and validation of the Japanese version of the Childhood Health Assessment Questionnaire (CHAQ), Mod.Rheumatol., Vol. ., 2008
    Organism:Department of Pediatrics, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawaku, Yokohama, 236-0004, Japan, tmiyamae@medyokohama-cuacjp
    Abstract:To assess cross-cultural adaptation, and to validate the parent's version of a health-related quality-of-life instrument, the Childhood Health Assessment Questionnaire (CHAQ) was investigated after its translation into Japanese. A total of 132 subjects were enrolled: 63 patients with juvenile idiopathic arthritis (JIA) (34 systemic and 29 polyarticular) and 69 healthy children. The CHAQ distinguished clinically between healthy subjects and the two JIA subtypes of patients. The average disability index (DI) scores for systemic JIA (sJIA) and polyarticular JIA (pJIA) patients and healthy subjects were 1.5, 1.2, and 0.0, respectively. All variables in the questionnaire were shown to be significant (P < 0.001). Patients with pJIA showed better correlation than those with sJIA. Significant correlation was seen in the polyarticular group with CRP, ESR, parents' VAS, the number of joints with pain, and the number of active joints. However, there was even a negative correlation between DI and parent's assessment of overall well-being for the sJIA group. The Japanese version of the CHAQ was a reliable and valid tool for the functional assessment of children with pJIA. Functional ability, as assessed by the CHAQ, may not be the main consideration of sJIA patients' parents when assessing their child's status

39. MURATSUGU M, YAZAWA A, FUJIWARA S, NISHIDA Set FUKUI T: Quantitation of biotin-binding immunoglobulins G, A, and M in Human Sera Using F(ab')2anti-human immunoglobulin-coated microplates, Biol.Pharm.Bull., Vol. 31(3), 507-510., 2008
    Organism:Bioanalytical Science Laboratory, Department of Clinical Nutrition, Osaka Prefecture University, Habikino, Osaka 583-8555, Japan mmakoto@rehabosakafu-uacjpFAU - Muratsugu, Makoto
    Abstract:Biotin-binding IgG (B-IgG) in human sera was quantified using previously developed F(ab')(2)anti-human IgG-coated multiwell microplates (Muratsugu M. et al., 2003, Biol. Pharm. Bull., 26, 1605--1608). The levels of B-IgG in sera, however, were higher than those we predicted. In this study, we modified the assay using F(ab')2anti-human IgG-coated multiwell microplates and successfully quantified the levels of B-IgG in sera. The cause of the unpredicted results was discussed in the text. In addition, the levels of biotin-binding IgA (B-IgA) and IgM (B-IgM) in sera could be measured using F(ab')2anti-human IgA- or IgM-coated multiwell microplates. We quantified B-IgG, B-IgA, and B-IgM in sera from healthy specimens and patients with bronchial asthma, atopic dermatitis, epilepsy, and juvenile rheumatoid arthritis

40. NISTALA K, MONCRIEFFE H, NEWTON KR, VARSANI H, HUNTER Pet WEDDERBURN LR: Interleukin-17-producing T cells are enriched in the joints of children with arthritis, but have a reciprocal relationship to regulatory T cell numbers, Arthritis Rheum., Vol. 58(3), 875-887., 2008
    Organism:University College London, London, UKFAU - Nistala, Kiran
    Abstract:OBJECTIVE: To identify interleukin-17 (IL-17)-producing T cells from patients with juvenile idiopathic arthritis (JIA), and investigate their cytokine production, migratory capacity, and relationship to Treg cells at sites of inflammation, as well as to test the hypothesis that IL-17+ T cell numbers correlate with clinical phenotype in childhood arthritis. METHODS: Flow cytometry was used to analyze the phenotype, cytokine production, and chemokine receptor expression of IL-17-producing T cells in peripheral blood and synovial fluid mononuclear cells from 36 children with JIA, in parallel with analysis of forkhead box P3 (FoxP3)-positive Treg cells. Migration of IL-17+ T cells toward CCL20 was assessed by a Transwell assay. Synovial tissue was analyzed by immunohistochemistry for IL-17 and IL-22. RESULTS: IL-17+ T cells were enriched in the joints of children with JIA as compared with the blood of JIA patients (P = 0.0001) and controls (P = 0.018) and were demonstrated in synovial tissue. IL-17+ T cell numbers were higher in patients with extended oligoarthritis, the more severe subtype of JIA, as compared with patients with persistent oligoarthritis, the milder subtype (P = 0.046). Within the joint, there was an inverse relationship between IL-17+ T cells and FoxP3+ Treg cells (r = 0.61, P = 0.016). IL-17+,CD4+ T cells were uniformly CCR6+ and migrated toward CCL20, but synovial IL-17+ T cells had variable CCR4 expression. A proportion of IL-17+ synovial T cells produced IL-22 and interferon-gamma. CONCLUSION: This study is the first to define the frequency and characteristics of "Th17" cells in JIA. We suggest that these highly proinflammatory cells contribute to joint pathology, as indicated by relationships with clinical phenotypes, and that the balance between IL-17+ T cells and Treg cells may be critical to outcome

41. OGUNTONA ASet ADELOWO O: Systemic onset juvenile chronic arthritis (JCA) in a Nigerian boy--a case report, Niger.J.Med., Vol. 17(1), 112-114., 2008
    Organism:Rheumatology Unit, Olabisi Onabanjo University Teaching Hospital Sagamu, Ogun State Oguntonasa@yahoocomFAU - Oguntona, A S
    Abstract:BACKGROUND: Juvenile chronic arthritis (JCA) is a chronic arthritis affecting children below age of 16 years. The systemic onset subgroup is also known as Still's disease. There are several distinct subgroups. There is paucity of literature of this disease entity in our environment due to under diagnosis of the disease. METHOD: The case note of this patient was retrieved. He was managed for 2 years in the adult rheumatology clinic after being transferred from the Paediatric unit. Relevant literature was reviewed. RESULT: There was a good response to immunosuppressive agents and low dose prednisolone. He was back at School and able to play with his mates after long withdrawal from School. CONCLUSION: Prompt referral of such cases to specialist centers will go a long way in determining the outcome of such patients. Prognosis is better with early presentation and appropriate management

42. OLSSON LM, LINDQVIST AK, KALLBERG H, PADYUKOV L, BURKHARDT H, ALFREDSSON L, KLARESKOG Let HOLMDAHL R: A case-control study of rheumatoid arthritis identifies an associated single nucleotide polymorphism in the NCF4 gene, supporting a role for the NADPH-oxidase complex in autoimmunity, Arthritis Res.Ther., Vol. 9(5), R98, 2007
    Organism:Medical Inflammation Research, Lund University, BMC I11, 221 84, Lund, Sweden linaolsson@medluseFAU - Olsson, Lina M
    Abstract:Rheumatoid arthritis (RA) is a chronic inflammatory disease with a heritability of 60%. Genetic contributions to RA are made by multiple genes, but only a few gene associations have yet been confirmed. By studying animal models, reduced capacity of the NADPH-oxidase (NOX) complex, caused by a single nucleotide polymorphism (SNP) in one of its components (the NCF1 gene), has been found to increase severity of arthritis. To our knowledge, however, no studies investigating the potential role played by reduced reactive oxygen species production in human RA have yet been reported. In order to examine the role played by the NOX complex in RA, we investigated the association of 51 SNPs in five genes of the NOX complex (CYBB, CYBA, NCF4, NCF2, and RAC2) in a Swedish case-control cohort consisting of 1,842 RA cases and 1,038 control individuals. Several SNPs were found to be mildly associated in men in NCF4 (rs729749, P = 0.001), NCF2 (rs789181, P = 0.02) and RAC2 (rs1476002, P = 0.05). No associations were detected in CYBA or CYBB. By stratifying for autoantibody status, we identified a strong association for rs729749 (in NCF4) in autoantibody negative disease, with the strongest association detected in rheumatoid factor negative men (CT genotype versus CC genotype: odds ratio 0.34, 95% confidence interval 0.2 to 0.6; P = 0.0001). To our knowledge, this is the first genetic association identified between RA and the NOX complex, and it supports previous findings from animal models of the importance of reactive oxygen species production capacity to the development of arthritis

43. PFEIL A, BOTTCHER J, SEIDL BE, HEYNE JP, PETROVITCH A, EIDNER T, MENTZEL HJ, WOLF G, HEIN Get KAISER WA: Computer-aided joint space analysis of the metacarpal-phalangeal and proximal-interphalangeal finger joint: normative age-related and gender-specific data, Skeletal Radiol., Vol. 36(9), 853-864., 2007
    Organism:Institute of Diagnostic and Interventional Radiology, Friedrich-Schiller-University Jena, Erlanger Allee 101, 07747 Jena, Germany alexanderpfeil@meduni-jenadeFAU - Pfeil, Alexander
    Abstract:PURPOSE: The purpose of the study was to provide reference data for computer-aided joint space analysis based on a semi-automated and computer-aided diagnostic system for the measurement of metacarpal-phalangeal and proximal-interphalangeal finger joint widths; additionally, the determination of sex differences and the investigation of changes in joint width with age were evaluated. PATIENTS AND METHODS: Eighty hundred and sixty-nine patients (351 female and 518 male) received radiographs of the hand for trauma and were screened for a host of conditions known to affect the joint spaces. All participants underwent measurements of joint space distances at the metacarpal-phalangeal articulation (JSD-MCP) from the thumb to the small finger and at the proximal-interphalangeal articulation (JSD-PIP) from the index finger to the small finger using computer-aided diagnosis technology with semi-automated edge detection. RESULTS: The study revealed an annual narrowing of the JSD of 0.6% for the JSD-MCP and for the JSD-PIP. Furthermore, the data demonstrated a notable age-related decrease in JSD, including an accentuated age-related joint space narrowing in women for both articulations. Additionally, males showed a significantly wider JSD-MCP (+11.1%) and JSD-PIP (+15.4%) compared with the female cohort in all age groups. CONCLUSION: Our data presented gender-specific and age-related normative reference values for computer-aided joint space analysis of the JSD-MCP and JSD-PIP that could be used to identify disease-related joint space narrowing, particularly in patients with osteoarthritis and rheumatoid arthritis commonly involving the peripheral small hand joints

44. PRINCE FH, TWILT M, TEN CATE R, VAN ROSSUM MA, ARMBRUST W, HOPPENREIJS EP, SANTEN-HOEUFFT M, KOOPMAN-KEEMINK Y, WULFFRAAT NMet SUIJLEKOM-SMIT LW: Long-term follow-up on effectiveness and safety of etanercept in JIA: the Dutch national register, Ann.Rheum.Dis., Vol. ., 2008
    Organism:Erasmus MC Sophia Children's Hospital, Netherlands
    Abstract:OBJECTIVE: Observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different Juvenile Idiopathic Arthritis (JIA) subtypes. METHODS: At baseline we collected patient and disease characteristics of all Dutch JIA patients who started treatment with etanercept. Disease activity was evaluated (at start of the study, after three months, and then yearly) according to the JIA core set of the American College of Rheumatology Paediatric definition of improvement (ACR Pedi). Use of etanercept and concomitant drugs was monitored. Adverse events were recorded. RESULTS: We included 146 JIA patients with a median follow-up of 2.5 years per patient (range 0.3-7.3). JIA subtypes represented: 27% systemic, 8% polyarticular rheumatoid-factor positive, 38% polyarticular rheumatoid-factor negative, 19% oligoarticular extended, 3% enthesitis-related, and 5% psoriatica. Most patients (77%) met the criteria of the ACR Pedi 30 in the first three months of treatment. For the majority of patients this improvement was sustained; 53 (36%) of all patients met the remission criteria. No other second-line agents were needed in 43 patients. Although systemic JIA patients responded initially less to etanercept therapy than patients from other subtypes, those who did respond showed equal effectiveness in the long-term. Serious adverse events rate was low (0.029 per patient-year). CONCLUSIONS: Etanercept is effective and safe in JIA, even for a great part of the systemic JIA patients. The greatest improvement occurred in the first three months of treatment, and was sustained for a long time in most patients (up to 75 months)

45. RATH SA, MOSZKO S, SCHAFFNER PM, CANTONE G, BRAUN V, RICHTER HPet ANTONIADIS G: Accuracy of pedicle screw insertion in the cervical spine for internal fixation using frameless stereotactic guidance, J.Neurosurg.Spine., Vol. 8(3), 237-245., 2008
    Organism:Department of Neurosurgery and Interventional Neuroradiology, Medical Center, Deggendorf, Germany stefanrath@klinikum-deggendorfdeFAU - Rath, Stefan A
    Abstract:OBJECT: Although transpedicular fixation is a biomechanically superior technique, it is not routinely used in the cervical spine. The risk of neurovascular injury in this region is considered high because the diameter of cervical pedicles is very small and their angle of insertion into the vertebral body varies. This study was conducted to analyze the clinical accuracy of stereotactically guided transpedicular screw insertion into the cervical spine. METHODS: Twenty-seven patients underwent posterior stabilization of the cervical spine for degenerative instability resulting from myelopathy, fracture/dislocation, tumor, rheumatoid arthritis, and pyogenic spondylitis. Fixation included 1-6 motion segments (mean 2.2 segments). Transpedicular screws (3.5-mm diameter) were placed using 1 of 2 computer-assisted guidance systems and lateral fluoroscopic control. The intraoperative mean deviation of frameless stereotaxy was < 1.9 mm for all procedures. RESULTS: No neurovascular complications resulted from screw insertion. Postoperative computed tomography (CT) scans revealed satisfactory positioning in 104 (90%) of 116 cervical pedicles and in all 12 thoracic pedicles. A noncritical lateral or inferior cortical breach was seen with 7 screws (6%). Critical malplacement (4%) was always lateral: 5 screws encroached into the vertebral artery foramen by 40-60% of its diameter; Doppler sonographic controls revealed no vascular compromise. Screw malplacement was mostly due to a small pedicle diameter that required a steep trajectory angle, which could not be achieved because of anatomical limitation in the exposure of the surgical field. CONCLUSIONS: Despite the use of frameless stereotaxy, there remains some risk of critical transpedicular screw malpositioning in the subaxial cervical spine. Results may be improved by the use of intraoperative CT scanning and navigated percutaneous screw insertion, which allow optimization of the transpedicular trajectory

46. RAVELLI A: The time has come to include assessment of radiographic progression in juvenile idiopathic arthritis clinical trials, J.Rheumatol., Vol. 35(4), 553-557., 2008
    Organism:Dipartimento di Pediatria G De Toni, Universita degli Studi di Genova and Istituto di Ricovero e Cura a Carattere Scientifico G Gaslini, Genova, ItalyFAU - Ravelli, Angelo
    Abstract:

47. RIISE OR, LEE A, CVANCAROVA M, HANDELAND KS, WATHNE KO, NAKSTAD B, GAUSTAD Pet FLATO B: Recent-onset childhood arthritis--association with Streptococcus pyogenes in a population-based study, Rheumatology (Oxford)., Vol. ., 2008
    Organism:Department of Rheumatology, Rikshospitalet Medical Centre, Department of Paediatrics, Ulleval University Hospital, Department of Biostatistics, Rikshospitalet Medical Centre, Oslo, Department of Paediatrics, Akershus University Hospital, University of Oslo, Akershus Faculty Division, Nordbyhagen, Institute of Microbiology, Rikshospitalet Medical Centre and University of Oslo, Oslo, Norway
    Abstract:Objectives. To assess the frequency of Streptococcus pyogenes in children with early arthritis, compare the characteristics in patients with post-streptococcal ReA (PSReA) with those in patients with other types of arthritis, and describe the occurrence of carditis in PSRA. Patients. In a population-based Norwegian study, the physicians were asked to refer all children with suspected arthritis. The arthritis patients were followed up at 6 weeks, 6 months and 18 months. The presence of S. pyogenes was based on throat smear or antibodies. Echocardiography was performed in the patients with ARF or PSRA. Results. Thirty-two (18%) of the 173 children with arthritis tested positive for S. pyogenes. The percentage of positive tests rose steadily with age and peaked at ages 8-11 (35%). Six weeks after admission arthritis was present in 33% of the PSRA patients, which was less frequent than in the juvenile idiopathic arthritis (JIA) patients (P < 0.001), but more frequent than in the transient arthritis patients (P = 0.012). Hip arthritis was more frequent and knee/ankle arthritis, ANA and HLA-B27 were less frequent in PSRA than in JIA (P < 0.001, P = 0.009 and P = 0.029, respectively). The PSRA patients were older than those with transient arthritis (P = 0.007). One child with ARF had carditis. Conclusions. Streptococcus pyogenes was present in 18% of children with arthritis. The patient characteristics, clinical presentation and early disease course in PSRA was different from that of JIA and transient arthritis

48. RINGOLD S, TORGERSON TR, EGBERT MAet WALLACE CA: Intraarticular Corticosteroid Injections of the Temporomandibular Joint in Juvenile Idiopathic Arthritis, J.Rheumatol., Vol. ., 2008
    Organism:From the Children's Hospital and Regional Medical Center, Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington, USA
    Abstract:OBJECTIVE: To describe the clinical and radiographic outcomes in a series of patients with juvenile idiopathic arthritis (JIA) who underwent one or more intraarticular corticosteroid (IAS) injections of the temporomandibular joint (TMJ) performed without imaging guidance. METHODS: Retrospective chart review was performed for all patients with JIA diagnosed and treated at our institution between January 1, 2000, and January 1, 2006, who underwent one or more IAS injections of their TMJ. IAS injections were performed by the same oral and maxillofacial surgeon without imaging guidance, using either triamcinolone acetonide or triamcinolone hexacetonide. The primary outcomes assessed were maximal incisal opening (MIO) measurements, patient-reported symptoms, physical examination findings, and imaging results. RESULTS: Twenty-five patients were identified. Twenty-one (84%) had radiographic evidence of TMJ disease when TMJ disease was first suspected by their physician. The 25 patients underwent 74 IAS injections on 47 separate occasions. When baseline MIO measurements were compared to the last MIO measurements of the study period, there was a mean increase in MIO of 6.9 mm (p = 0.002; 95% CI 3, 10.7). There was a mean increase in MIO of 3.8 mm following each IAS injection (p = 0.003; 95% CI 1.4, 6.2). Patients who underwent multiple IAS injections had a mean increase in MIO after first injection of 6.6 mm (p < 0.001; 95% CI 4.1, 9.1); however, the mean increase in MIO after subsequent injections was 0.4 mm (p = 0.8; 95% CI -3.5, 4.4). One patient developed subcutaneous atrophy at the injection site. Two patients developed small, asymptomatic intraarticular calcifications. No additional adverse events were reported. CONCLUSIONS: In this patient population, there was an overall increase in MIO measurements following initial IAS injection and during the study period. Patients tended to have minimal response to subsequent injections. IAS injections performed without imaging guidance by an experienced oral and maxillofacial surgeon were well tolerated with only rare adverse events. The presence of radiographic changes when the physician first suspected TMJ disease in 84% of patients emphasizes the need for better screening and early intervention for synovitis in this joint

49. ROHR P, VEIT TD, SCHEIBEL I, XAVIER RM, BRENOL JC, CHIES JAet KVITKO K: GSTT1, GSTM1 and GSTP1 polymorphisms and susceptibility to juvenile idiopathic arthritis, Clin.Exp.Rheumatol., Vol. 26(1), 151-155., 2008
    Organism:Departamento de Genetica e Programa de Pos-graduacao em Genetica e Biologia Molecular, Universidade Federal do Rio Grande do Sul, Porto Alegre, BrasilFAU - Rohr, P
    Abstract:OBJECTIVE: In this study we have analyzed GSTM1, GSTT1 and GSTP1 polymorphisms in patients with juvenile idiopathic arthritis (JIA), to investigate a possible role of these genes as genetic components of the disease. METHODS: A total of 103 individuals (49 oligoarticular, 41 polyarticular and 13 systemic) were analyzed for the three polymorphisms, using a PCR/RFLP methodology. RESULTS: We have observed significantly increased frequencies of individuals with GSTT1 null genotype in JIA patients comparing to controls (37% x 21%; p=0.0183). There was a 2-fold increased risk (OR 2.2, 95% CI 1.2-4.1) associating the disease with the GSTT1 null genotype. Considering the subgroups (oligoarticular, polyarticular and systemic), the results indicated an association between polyarticular and systemic patients and the GSTT1 null genotype. There was a 2-fold increased risk for polyarticular patients (OR 2.4, 95%, CI 1.1-5.4), and a 4-fold increased risk for systemic patients (OR 4.4, 95%, 1.3-14.5). CONCLUSION: The GSTT1 null genotype seems to be involved in polyarticular and systemic JIA

50. RUPERTO Net MARTINI A: Network in pediatric rheumatology: the example of pediatric rheumatology international trials organisation, Georgian.Med.News., Vol. (156), 68-74., 2008
    Organism:Paediatric Rheumatology International Trials Organisation (PRINTO); IRCCS G Gaslini, Universita di Genova, Pediatria II-Reumatologia, Genova, ItalyFAU - Ruperto, N
    Abstract:The pediatric rheumatic diseases (PRD) are rare conditions associated with important sequelae on the quality of life and long term outcome. The research aimed at studying new therapeutic approaches is difficult because of logistic, methodological and ethical problems. To face these problems 2 international networks; the Pediatric Rheumatology Collaborative Study Group (PRCSG) and the Paediatric Rheumatology International Trials Organization (PRINTO) have been founded. The 2 networks have the goal to promote, facilitate and conduct high quality research for the PRD. In particular they have been able to standardize the evaluation of response to therapy in juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus, and juvenile dermatomyositis, to draft clinical remission criteria in JIA, and to provide cross-cultural adapted and validated quality of life instruments like the Childhood Health Assessment Questionnaire, and the Child Health Questionnaire, into 32 different languages. In this paper we reviewed how the creation of large international trial networks such as PRINTO and PRCSG, the definition of internationally recognized and standardized outcome measures and definitions of improvement, the validation of quality of life instruments, the adoption of adequate legislative measures (pediatric rule), have created the basic premises for the best future assessment of the PRD. This progress now offers children with PRD the same opportunities as adults to be treated with drugs whose safety and efficacy have been assessed through legitimate scientifically valid investigations

51. RUSSO RAet KATSICAS MM: Global Damage in Systemic Juvenile Idiopathic Arthritis: Preliminary Early Predictors, J.Rheumatol., Vol. ., 2008
    Organism:From Servicio de Inmunologia y Reumatologia, Hospital de Pediatria "Prof Dr Juan P Garrahan," Buenos Aires, Argentina
    Abstract:OBJECTIVE: To assess damage in systemic juvenile idiopathic arthritis (sJIA) by the use of the Juvenile Arthritis Damage Index (JADI) and to identify early predictors of global, articular, and extraarticular damage. METHODS: Forty-seven consecutive patients with sJIA with a disease duration > 24 months were assessed for damage in a cross-sectional evaluation. The JADI was administered by 2 pediatric rheumatologists. Damage was defined as JADI score >/= 1. Early clinical variables were retrieved from clinical records, and they included demographic, clinical, and laboratory characteristics. Univariate analysis was used to select candidate predictors to be included in multiple logistic regression. RESULTS: Twenty (43%) patients exhibited damage: 18 (38%) patients had articular and 9 (19%) extraarticular damage. JADI score ranged between 0 and 24. Cervical spine arthritis and corticosteroid usage occurring in the first 6 months of the disease course were found as predictors of damage. Damage scores correlated with number of joints with limited motion, and with functional disability. CONCLUSION: Articular damage is the main component of global damage in patients with sJIA. Early cervical spine involvement and corticosteroid usage may identify patients with sJIA at risk of developing damage

52. SAITO Y, TORIUMI S, OTAKE Ret SUZUKI M: [Adult-onset Still's disease following severe sore throat and fever. Case report], Nippon Jibiinkoka Gakkai Kaiho., Vol. 111(1), 21-24., 2008
    Organism:Department of Otolaryngology, Takeda General Hospital, FukushimaFAU - Saito, Yuki
    Abstract:Adult-onset Still's disease (AOSD) is characterized by fever, rash, and joint pain and may lead to chronic arthritis. The cause of AOSD is unknown, and it is rare. In children, Still's Disease is called systemic juvenile rheumatoid arthritis. We encountered a patient with adult-onset Still's disease following a severe sore throat and fever. The patient was a 17-year-old woman who consulted our hospital because of a sore throat and fever. She was admitted and treated with antibiotics, but the fever persisted. Laboratory parameters of inflammatory activity increased at an accelerated rate, and after ruling out sepsis, EBV-associated disease, and malignant lymphoma, a diagnosis of AOSD was made. Steroid therapy was very effective. When acute pharyngitis is observed in association with significant changes in laboratory parameters despite mild local symptoms, or when pharyngitis is observed in association with joint pain, continuous fever, and a rash, it is important to consider AOSI)

53. SARMA PK, MISRA Ret AGGARWAL A: Elevated serum receptor activator of NFkappaB ligand (RANKL), osteoprotegerin (OPG), matrix metalloproteinase (MMP)3, and ProMMP1 in patients with juvenile idiopathic arthritis, Clin.Rheumatol., Vol. 27(3), 289-294., 2008
    Organism:Department of Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, IndiaFAU - Sarma, Pradip Kumar
    Abstract:We studied the serum levels of receptor activator of nuclear factor-kappaB ligand (RANKL), osteoprotegerin (OPG), pro-matrix metalloproteinase (MMP) 1, MMP3, and tissue inhibitor of metalloproteinase (TIMP) 1 in patients with juvenile idiopathic arthritis (JIA) and correlated these with different disease variables. Sera of 70 patients with JIA (ILAR 2001 criteria) and 33 age- and sex-matched controls were assayed by enzyme-linked immunosorbent assay. Nonparametric tests were used for analysis of data. The subtype distribution of the JIA patients was: enthesitis-related arthritis (ERA) 24, polyarticular 22, systemic onset 13, oligoarticular 8, and others 3. The median level of RANKL, OPG, pro-MMP1, MMP3, and TIMP-1 were elevated in JIA patients as compared to controls (p < 0.001). There was no difference in levels among different types of JIA. RANKL/OPG ratio was elevated in all subtypes of JIA. MMP3/TIMP-1 ratio correlated with measures of disease activity including swollen and tender joint count, erythrocyte sedimentation rate, and disease activity score (rS 0.28, p < 0.05). Ours is the first study to show elevated RANKL in serum of patients with JIA. Further, our data suggest that patients with ERA have similar levels to other forms of JIA. Association of the MMP3/TIMP-1 ratio with disease activity suggests that it may be a useful biomarker for follow-up

54. SCHAFRANSKI MD, PEREIRA FL, SCHERNER D, TORRES Ret MESSIAS-REASON IJ: Functional MASP2 gene polymorphism in patients with history of rheumatic fever, Hum.Immunol., Vol. 69(1), 41-44., 2008
    Organism:Departamento de Patologia Medica, Hospital de Clinicas da Universidade Federal do Parana (UFPR), Curitiba, Parana, BrazilFAU - Schafranski, Marcelo Derbli
    Abstract:In this study we investigated whether partial or total MASP-2 deficiencies resulting from Asp105Gly mutation are associated with rheumatic fever (RF) and chronic rheumatic heart disease (RHD). The Asp105Gly MASP2 mutation (D105G) was detected by polymerase chain reaction-restriction fragment length polymorphism in 148 patients (43 men and 105 women; mean age 39.1 +/- 14.4 years) with a history of RF, including 106 (73%) with RHD and 42 (27%) without cardiac sequelae, and 129 control subjects (52 men and 77 women, mean age 38.4 +/- 12.2 years). The D105G mutation was detected in four patients with RHD (3.77%) and in five control subjects (3.88%), all in the heterozygous state. None of the patients without cardiac sequelae had the mutation. No significant difference was found in the frequency of the mutant allele between the groups (p < 0.6). These results suggest that the D105G mutation in the MASP2 gene does not play a major role in the pathogenesis of RF. Whether D105G plays a role in the development of RHD should be ascertained in future studies

55. SCHWARTZ HCet RELLE RJ: Distraction osteogenesis for temporomandibular joint reconstruction, J.Oral Maxillofac.Surg., Vol. 66(4), 718-723., 2008
    Organism:Southern California Permanente Medical Group, Oral and Maxillofacial Surgery, University of California Los Angeles, Los Angeles, CA, USA HCSchwartz@scalkporgFAU - Schwartz, Harry C
    Abstract:PURPOSE: This clinical study evaluated the use of transport distraction osteogenesis in reconstruction of the ramus-condyle unit (RCU) of the temporomandibular joint (TMJ). PATIENTS AND METHODS: Thirteen TMJ reconstructions were carried out in 12 patients. Diagnoses included tumors, trauma, ankylosis, and degenerative joint disease. The follow-up period has ranged from 7 to 59 months. RESULTS: Successful distraction was carried out in all cases, with development of solid regenerate bone and an effective new articulation. There were no complications. A good functional level was achieved in all cases. One patient with bilateral rheumatoid arthritis has experienced ongoing degenerative changes in the reconstructed condyles, with reappearance of an anterior open bite. The occlusion has remained stable in all other cases. CONCLUSIONS: Distraction osteogenesis is a promising treatment option in reconstruction of the RCU of the TMJ

56. SMITH AD, KIM YIet REFSUM H: Is folic acid good for everyone?, Am.J.Clin.Nutr., Vol. 87(3), 517-533., 2008
    Organism:Oxford Project to Investigate Memory and Ageing, Department of Physiology, Anatomy & Genetics, University of Oxford, Oxford, United Kingdom davidsmith@pharmoxacukFAU - Smith, A David
    Abstract:Fortification of food with folic acid to reduce the number of neural tube defects was introduced 10 y ago in North America. Many countries are considering whether to adopt this policy. When fortification is introduced, several hundred thousand people are exposed to an increased intake of folic acid for each neural tube defect pregnancy that is prevented. Are the benefits to the few outweighed by possible harm to some of the many exposed? In animals, a folic acid-rich diet can influence DNA and histone methylation, which leads to phenotypic changes in subsequent generations. In humans, increased folic acid intake leads to elevated blood concentrations of naturally occurring folates and of unmetabolized folic acid. High blood concentrations of folic acid may be related to decreased natural killer cell cytotoxicity, and high folate status may reduce the response to antifolate drugs used against malaria, rheumatoid arthritis, psoriasis, and cancer. In the elderly, a combination of high folate levels and low vitamin B-12 status may be associated with an increased risk of cognitive impairment and anemia and, in pregnant women, with an increased risk of insulin resistance and obesity in their children. Folate has a dual effect on cancer, protecting against cancer initiation but facilitating progression and growth of preneoplastic cells and subclinical cancers, which are common in the population. Thus, a high folic acid intake may be harmful for some people. Nations considering fortification should be cautious and stimulate further research to identify the effects, good and bad, caused by a high intake of folic acid from fortified food or dietary supplements. Only then can authorities develop the right strategies for the population as a whole

57. STENBERG AE, SYLVEN L, HEDSTRAND H, KAMPE Oet HULTCRANTZ M: Absence of autoantibodies connected to autoimmune polyendocrine syndrome type I and II and Addison's disease in girls and women with Turner syndrome, J.Negat.Results Biomed., Vol. 6, 10, 2007
    Organism:Dept of Otorhinolaryngology, Karolinska University Hospital, Solna, Sweden annikastenberg@kiseFAU - Stenberg, Annika E
    Abstract:BACKGROUND: A disturbance in the immune system has been described in Turner syndrome (45,X), with an association to low levels of IgG and IgM and decreased levels of T- and B-lymphocytes. Also different autoimmune diseases have been connected to Turner syndrome (45,X), thyroiditis being the most common. Other autoimmune diseases seen are inflammatory bowel disease, insulin dependent diabetes mellitus, Addison's disease, rheumatoid arthritis, myasthenia gravis, vitiligo, alopecia, pernicious anaemia and hypoparathyroidism, but the association to Turner syndrome is not definite.Besides the typical features of Turner syndrome (short stature, failure to enter puberty spontaneously and infertility due to ovarian insufficiency) ear problems are common. Otitis media and a progressive sensorineural hearing disorder are commonly seen. In the normal population there are known inner ear disorders related to autoimmune diseases. The aim of this study was to investigate patients with Turner syndrome regarding autoantibodies connected to the autoimmune disorders; autoimmune polyendocrine syndrome type I and II and Addison's disease, to screen for overlapping profile of autoantibodies.Blood samples from 110 Turner patients (7-65 years) were investigated using in vitro transcription, translation and immunoprecipitation techniques regarding autoantibodies connected to autoimmune polyendocrine syndrome type I and II and Addison's disease (21-hydroxylase, 17alpha-hydroxylase, side-chain cleavage enzyme, aromatic L-amino acid decarboxylase, tyrosine hydroxylase and tryptophan hydroxylase). RESULTS: The autoantibodies investigated were not overrepresented among the Turner patients. CONCLUSION: The autoimmune disorders associated with Turner syndrome do not seem to be of the same origin as Addison's disease, the type I or II autoimmune polyendocrine syndrome

58. STOCK CJ, OGILVIE EM, SAMUEL JM, FIFE M, LEWIS CMet WOO P: Comprehensive association study of genetic variants in the IL-1 gene family in systemic juvenile idiopathic arthritis, Genes Immun., Vol. ., 2008
    Organism:1Centre for Paediatric and Adolescent Rheumatology, Windeyer Institute for Medical Sciences, University College London, London, UK
    Abstract:Patients with systemic juvenile idiopathic arthritis (sJIA) have a characteristic daily spiking fever and elevated levels of inflammatory cytokines. Members of the interleukin-1 (IL-1) gene family have been implicated in various inflammatory and autoimmune diseases, and treatment with the IL-1 receptor antagonist, Anakinra, shows remarkable improvement in some patients. This work describes the most comprehensive investigation to date of the involvement of the IL-1 gene family in sJIA. A two-stage case-control association study was performed to investigate the two clusters of IL-1 family genes using a tagging single nucleotide polymorphism (SNP) approach. Genotyping data of 130 sJIA patients and 151 controls from stage 1 highlighted eight SNPs in the IL1 ligand cluster region and two SNPs in the IL1 receptor cluster region as showing a significant frequency difference between the populations. These 10 SNPs were typed in an additional 105 sJIA patients and 184 controls in stage 2. Meta-analysis of the genotypes from both stages showed that three IL1 ligand cluster SNPs (rs6712572, rs2071374 and rs1688075) and one IL1 receptor cluster SNP (rs12712122) show evidence of significant association with sJIA. These results indicate that there may be aberrant control of the activity of the IL-1 family in sJIA patients causing the increased susceptibility to the disease.Genes and Immunity advance online publication, 17 April 2008; doi:10.1038/gene.2008.24

59. SYNODINOS PNet POLYZOIS I: Oral health and orthodontic considerations in children with juvenile idiopathic arthritis: review of the literature and report of a case, J.Ir.Dent.Assoc., Vol. 54(1), 29-36., 2008
    Organism:Dublin Dental School & Hospital, Lincoln Place, DublinFAU - Synodinos, Philippos N
    Abstract:Juvenile idiopathic arthritis (JIA) is a severe disease of childhood, which comprises a diverse group of distinct clinical entities of unclear aetiology. Some abnormality of the immune system is present in all JIA cases. In its most severe clinical form, JIA may show localised and/or systemic complications, including functional impairment of the affected sites. This may result in variable growth and developmental anomalies. In many JIA cases, where the temporomandibular joint (TMJ) is affected, mandibular growth may be restricted, thus leading to the development of mandibular hypoplasia and/or retrognathism. As a result, it is not uncommon for JIA patients to present with skeletal Class II and open bite malocclusions. Furthermore, in JIA cases with unilateral TMJ involvement, craniofacial asymmetry may occur. In such cases, early orthodontic intervention facilitates both the skeletal and the occlusal rehabilitation. Increased prevalence of dental caries and periodontal disease in JIA cases may be attributed to a combination of aetiological factors, including difficulties in executing good oral hygiene, unfavourable dietary practices and side effects from the long-term administration of medication. In addition, an association between periodontal disease and JIA has been reported based on their similar pattern of clinical disregulation of the inflammatory process. This paper presents a brief description of JIA, with special reference to dental health and orthodontic treatment considerations. In addition, a case is presented where the appropriate orthodontic intervention led to the establishment of a normally functioning, as well as an aesthetically pleasing, occlusion

60. TAKAGAKI H, NISHIMURA S, ONDA J, HARADA K, TAKAYASU Tet KAZEKAWA K: [Vertebral arteriovenous fistula as a complication of atlantoaxial transarticular screw fixation--case report], Brain Nerve., Vol. 60(3), 291-294., 2008
    Organism:Department of Neurosurgery, Kitakyusyu General Hospital, 5-10-10 Yugawa, Kokuraminami-ku, Kitakyusyu-shi, Fukuoka 800-0256, JapanFAU - Takagaki, Hisashi
    Abstract:We report a rare case of a vertebral arteriovenous fistula that developed as a complication of atlantoaxial transarticular screw fixation. The patient was a 44-year-old male with a history of juvenile rheumatoid arthritis. He had undergone an atlantoaxial transarticular screw fixation for an atlantoaxial dislocation. At 2 months after the surgery, he complained of right-side tinnitus. A selective left vertebral angiography showed a high-flow arteriovenous fistula of the right V2 segment and occulusion of the right vertebral artery at the level of the C3 vertebral body. Endovascular embolization of the arteriovenous fistula was successfully performed using detachable coils. No deficits were observed after the treatment, and the tinnitus disappeared completely. Endovascular coil embolization is currently an effective and safe treatment for the vertebral arteriovenous fistula

61. TAKKEN T, VAN BRUSSEL M, ENGELBERT R, VAN DER NJ, KUIS Wet HELDERS P: Exercise therapy in juvenile idiopathic arthritis, Cochrane.Database.Syst.Rev., Vol. (2), CD005954, 2008
    Organism:BACKGROUND: Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). OBJECTIVES: To assess the effects of exercise therapy on functional ability, quality of life and aerobic capacity in children with JIA. SEARCH STRATEGY: The Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Database of Systematic Reviews (The Cochrane Library), MEDLINE (January 1966 to April 2007), CINAHL (January 1982 to April 2007), EMBASE (January 1966 to October 2007), PEDro (January 1966 to October 2007), SportDiscus (January 1966 to October 2007), Google Scholar (to October 2007), AMED (Allied and Alternative Medicine) (January 1985 to October 2007), Health Technologies Assessment database (January 1988 to October 2007), ISI Web Science Index to Scientific and Technical Proceedings (January 1966 to October 2007) and the Chartered Society of Physiotherapy website (http://www.cps.uk.org) were searched and references tracked. SELECTION CRITERIA: Randomised controlled trials (RCTs) of exercise treatment in JIA. DATA COLLECTION AND ANALYSIS: Potentially relevant references were evaluated and all data were extracted by two review authors working independently. MAIN RESULTS: Three out of 16 identified studies met the inclusion criteria, with a total of 212 participants. All the included studies fulfilled at least seven of 10 methodological criteria. The outcome data of the following measures were homogenous and were pooled in a meta-analysis: functional ability (n = 198; WMD -0.07, 95% CI -0.22 to 0.08), quality of life (CHQ-PhS: n = 115; WMD -3.96, 95% CI -8.91 to 1.00) and aerobic capacity (n = 124; WMD 0.04, 95% CI -0.11 to 0.19). The results suggest that the outcome measures all favoured the exercise therapy but none were statistically significant. None of the studies reported negative effects of the exercise therapy. AUTHORS' CONCLUSIONS: Overall, based on 'silver-level' evidence (www.cochranemsk.org) there was no clinically important or statistically significant evidence that exercise therapy can improve functional ability, quality of life, aerobic capacity or pain. The low number of available RCTs limits the generalisability. The included and excluded studies were all consistent about the adverse effects of exercise therapy; no short-term detrimental effects of exercise therapy were found in any study. Both included and excluded studies showed that exercise does not exacerbate arthritis. The large heterogeneity in outcome measures, as seen in this review, emphasises the need for a standardised assessment or a core set of functional and physical outcome measurements suited for health research to generate evidence about the possible benefits of exercise therapy for patients with JIA. Although the short-term effects look promising, the long-term effect of exercise therapy remains unclear

62. THORNTON J, LUNT M, ASHCROFT DM, BAILDAM E, FOSTER H, DAVIDSON J, GARDNER-MEDWIN J, BERESFORD MW, SYMMONS D, THOMSON Wet ELLIOTT RA: Costing juvenile idiopathic arthritis: examining patient-based costs during the first year after diagnosis, Rheumatology (Oxford)., Vol. ., 2008
    Organism:Arthritis Research Campaign Epidemiology Unit, Division of Epidemiology and Health Sciences, School of Pharmacy and Pharmaceutical Sciences, University of Manchester, Manchester, Royal Liverpool Children's Hospital, Department of Rheumatology, Liverpool, Department of Rheumatology, Medical School, Newcastle upon Tyne, Department of Rheumatology, Royal Hospital for Sick Children, Department of Child Health, Royal Hospital for Sick Children, Glasgow, University of Liverpool and Royal Liverpool Children's Hospital, Department of Rheumatology, Liverpool and Division of Social Research in Medicines and Health, School of Pharmacy, University of Nottingham, Nottingham, UK
    Abstract:Objectives. There are few data on the treatment patterns and associated cost of treating children with inflammatory arthritis including juvenile idiopathic arthritis (JIA), in the short or long term. The aim of this study was to obtain patient-based costs for treating children with JIA in the UK, in the first year from diagnosis and from the secondary health care payer perspective. Methods. The Childhood Arthritis Prospective Study (CAPS) is an ongoing longitudinal study recruiting children with inflammatory arthritis from four UK hospital centres. Included children are newly diagnosed, </=16 years old with inflammatory arthritis of one or more joints, which has persisted for at least 2 weeks. Health service resource use data were collected as part of routine clinical care at study entry, 6 months and 1 year. Reference unit costs were applied to these data and the cost of treatment per child calculated for the first year from diagnosis. Results. A total of 297 children attended a 12-month follow-up visit. The mean annual total cost per child was pound1649 (s.d. pound1093, range pound401- pound6967). The highest cost component was for appointments with paediatric rheumatologists. Mean total costs were highest for children with enthesitis-related, systemic JIA or extended oligoarthritis. Conclusions. In the first 12 months after diagnosis, children with all JIA disease subtypes consume large, but highly variable quantities of health service resources. Individual patient costs are required to reflect the wide variation in cost between patients and allow appropriate recouping of costs for contracted services and for assessing the economic impact of interventions

63. TRACHANA M, PRATSIDOU-GERTSI P, KANAKOUDI-TSAKALIDOU F, CHRISTODOULOU A, BANTOURAKI M, ARSOS Get BENIZELOS J: Secondary osteonecrosis mimicking a femur bone tumor in an adolescent with juvenile idiopathic arthritis, Pediatr.Blood Cancer., Vol. ., 2008
    Organism:Pediatric Immunology and Rheumatology Center, First Department of Pediatrics, Ippokration General Hospital, Aristotle University, Thessaloniki, Greece
    Abstract:

64. TURAN Y, DURUOZ MT, BAL S, GUVENC A, CERRAHOGLU Let GURGAN A: Assessment of fatigue in patients with ankylosing spondylitis, Rheumatol.Int., Vol. 27(9), 847-852., 2007
    Organism:Department of Physical Medicine and Rehabilitation, Ataturk Research and Education Hospital, Izmir, Turkey yasemin_dincer@yahoocomFAU - Turan, Yasemin
    Abstract:In this study, we evaluated fatigue by using the multidimensional assessment of fatigue (MAF) index in 68 ankylosing spondylitis (AS) patients. To determine the disease activity, functional status and quality of life, bath ankylosing spondylitis disease activity index (BASDAI), bath ankylosing spondylitis functional index (BASFI) and Short Form 36 (SF36) were used respectively. Mander enthesis index (MEI) was used for evaluation of enthesitis. The mean age of the patients was 37.7 (11.1) years. The prevalence of fatigue was 76.5%. There were significant correlations between MAF and BASDAI (P < 0.001), BASFI (P < 0.001), MEI (P = 0.048), pain (P = 0.001), hemoglobin (P = 0.001), ESR (P = 0.035), dorsal Schober's (P = 0.009), occiput-wall distance (P = 0.048). Also MAF was correlated with all dimensions of SF36 except for social function and emotional role. BASFI was found to be the most significant correlated (P = 0.002) parameter with MAF. This study suggests that fatigue is an important symptom in AS and it seemed to occur in severe AS patients. It should appropriately be measured with respect to its intensity with appropriate measures, such as MAF. Moreover, fatigue may increase functional disability, which is already present as a feature of the disease

65. TWILT M, SCHULTEN AJ, VERSCHURE F, WISSE L, PRAHL-ANDERSEN Bet SUIJLEKOM-SMIT LW: Long-term followup of temporomandibular joint involvement in juvenile idiopathic arthritis, Arthritis Rheum., Vol. 59(4), 546-552., 2008
    Organism:Erasmus MC Sophia Children's Hospital, Rotterdam, The NetherlandsFAU - Twilt, Marinka
    Abstract:OBJECTIVE: Temporomandibular joint (TMJ) involvement is a frequent feature in cross-sectional prevalence studies among patients with juvenile idiopathic arthritis (JIA). In this followup study, patients were reviewed after 5 years to study the course of TMJ involvement in relation to disease characteristics. METHODS: Children with JIA from a previous study on TMJ involvement were included. A rheumatologic evaluation including the 6 parameters of the JIA core set and an orthodontic evaluation including an orthopantomogram (OPT) were performed. OPTs were scored according to Rohlin's grading system (grades 0-5). RESULTS: The overall prevalence of patients with condylar alterations decreased from 49% to 40%. Improvement of the alterations was seen in 69% of the initially affected condyles, and consequently improvement was seen in 83% of the initially affected patients. Normalization of the alterations was seen in 67% of the improved condyles, and consequently in 44% of the patients. This proves that the condyle has a regenerative capacity. Improvement was related to low disease activity and a less extensive therapeutic regimen. CONCLUSION: In patients with JIA, condylar alterations can improve and even regenerate. Condylar improvement is associated with a low disease activity

66. VIVARELLI M, D'URBANO LE, INSALACO A, LUNT M, JURY F, TOZZI AE, RAVELLI A, MARTINI A, DONN Ret DE BENEDETTI F: Macrophage migration inhibitory factor (MIF) and oligoarticular juvenile idiopathic arthritis (o-JIA): association of MIF promoter polymorphisms with response to intra-articular glucocorticoids, Clin.Exp.Rheumatol., Vol. 25(5), 775-781., 2007
    Organism:Direzione Scientifica, Pediatria II Reumatologia, IRCCS Ospedale Pediatrico Bambino Gesu, Rome, ItalyFAU - Vivarelli, M
    Abstract:OBJECTIVES: To address the clinical relevance of macrophage migration inhibitory factor (MIF) promoter polymorphisms in oligoarticular juvenile idiopathic arthritis (o-JIA) by evaluating their associations with serum and SF MIF levels, with response to intra-articular glucocorticoid injections and with outcome of the disease. METHODS: Seventy-five Caucasian patients with o-JIA were studied. Alleles of the -794 CATT variable number of tandem repeats (VNTR) and of the -173 G/C single nucleotide polymorphism (SNP) were identified by capillary electrophoresis following fluorescently labelled PCR and by allelic discrimination assay, respectively. MIF levels were measured by ELISA. The association of MIF promoter polymorphisms with polyarticular extension, Childhood Health Assessment Questionnaire (CHAQ) score at the last follow-up visit and occurrence of chronic anterior uveitis was evaluated only in patients with a follow up > 5 years. RESULTS: Neither of the MIF promoter polymorphisms was associated with serum MIF levels, nor with the long-term outcome of o-JIA. The -173 G/C SNP was significantly associated with both SF MIF levels and duration of response to intra-articular glucocorticoid injection. Carriers of a MIF -173 C allele were 4 times more likely to relapse within 3 months. No association was found between the different MIF CATT alleles and both SF MIF levels and duration of response to intra-articular glucocorticoids. CONCLUSION: Our study shows the clinical relevance of the MIF -173 G/C SNP in o-JIA and suggests that the -173 C allele may represent a predictor of poor response to intra-articular glucocorticoid treatment

67. WAGNER-WEINER L: Pediatric rheumatology for the adult rheumatologist, J.Clin.Rheumatol., Vol. 14(2), 109-119., 2008
    Organism:Rheumatology Grand Rounds at Rush University Medical Center, Chicago, IL, USA lww@uchicagoeduFAU - Wagner-Weiner, Linda
    Abstract:Although many manifestations of rheumatic diseases in children are similar to those in adults, there exist important differences. These include variations in presentation and complications, differential diagnoses to consider, medication side effects, impact on growth, and psychosocial issues. As there is a US and worldwide shortage of pediatric rheumatologists, many children with rheumatic illnesses are cared for by other physicians, especially by adult rheumatologists. This article is aimed toward educating the adult rheumatologist in 4 key areas of childhood rheumatic disease: juvenile idiopathic arthritis, other rheumatic and inflammatory illnesses seen primarily in the pediatric population, differences in rheumatic diseases common to both adults and children, and psychosocial issues

68. WEISS PF, ARABSHAHI B, JOHNSON A, BILANIUK LT, ZARNOW D, CAHILL AM, FEUDTNER Cet CRON RQ: High prevalence of temporomandibular joint arthritis at disease onset in children with juvenile idiopathic arthritis, as detected by magnetic resonance imaging but not by ultrasound, Arthritis Rheum., Vol. 58(4), 1189-1196., 2008
    Organism:Children's Hospital of Philadelphia and University of Pennsylvania Center for Clinical Epidemiology and Biostatistics, Philadelphia, PennsylvaniaFAU - Weiss, Pamela F
    Abstract:OBJECTIVE: To determine the prevalence of temporomandibular joint (TMJ) disease in a cohort of children with new-onset juvenile idiopathic arthritis (JIA), and to compare magnetic resonance imaging (MRI) with ultrasound (US) for the detection of acute and chronic changes of TMJ arthritis. METHODS: Between January 2005 and April 2007, children with newly diagnosed JIA were prospectively evaluated for TMJ arthritis. Prior to imaging, jaw pain and disability were assessed with questionnaires and physical examination. The TMJs of all patients were imaged with MRI and US within 8 weeks of diagnosis. RESULTS: Of the 32 patients enrolled, 78% were female, and the median age was 8.6 years (range 1.5-17.2 years). Acute TMJ arthritis was diagnosed in 75% of the children by MRI and in none by US; chronic arthritis was diagnosed in 69% by MRI and in 28% by US. Findings of both acute and chronic TMJ disease were detected by MRI in 53% of the patients. Of those with acute TMJ arthritis, 71% were asymptomatic, and 63% had normal findings on jaw examination. Fifty-six percent of patients with acute disease had an improved maximal incisal opening after corticosteroid injection. Among these responders, 56% had been asymptomatic and had normal jaw examination findings. CONCLUSION: TMJ arthritis was present in the majority of patients with new-onset JIA. Findings on MRI along with responses to treatment among asymptomatic patients with normal jaw examination findings suggest that a history review and physical examination are not sufficient to screen for TMJ disease. Our results also suggest that MRI and US findings are not well correlated, and that MRI is preferable for the detection of TMJ disease in new-onset JIA

69. WEISSBRICH B, SUSS-FROHLICH Yet GIRSCHICK HJ: Seroprevalence of parvovirus B19 IgG in children affected by juvenile idiopathic arthritis, Arthritis Res.Ther., Vol. 9(4), R82, 2007
    Organism:Institute of Virology and Immunobiology, University of Wurzburg, Versbacher Str 7, 97078 Wurzburg, Germany weissbrich@vimuni-wuerzburgdeFAU - Weissbrich, Benedikt
    Abstract:Parvovirus (PV) B19 is the causative agent of the childhood disease erythema infectiosum. An association of PV B19 with chronic arthropathies, sometimes resembling rheumatoid arthritis or juvenile idiopathic arthritis (JIA), has repeatedly been described. Other studies, however, have failed to identify any such relationship. In order to study further whether there is a link between PV B19 and JIA, we determined the prevalence of PV B19 specific IgG antibodies in serum samples from children with rheumatoid diseases and compared it with the prevalence in unaffected children We reasoned that if there is an association between PV B19 and JIA, then the prevalence of PV B19 IgG in the children with JIA should be higher than in the control group. PV B19 IgG status was tested in 406 children with JIA and related diseases, and in 146 children constituting a control group. The percentage of PV B19 IgG positive children was not significantly elevated in the disease subgroups compared with age-matched control groups. In conclusion, our findings do not support the hypothesis that human parvovirus B19 is involved in the pathogenesis of JIA

70. WONG SC, MACRAE VE, GRACIE JA, MCINNES IB, GALEA P, GARDNER-MEDWIN Jet AHMED SF: Inflammatory cytokines in juvenile idiopathic arthritis: Effects on physical growth and the insulin-like-growth factor axis, Growth Horm.IGF.Res., Vol. ., 2008
    Organism:Bone and Endocrine Research Group, Department of Child Health, Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8SJ, United Kingdom
    Abstract:OBJECTIVE: To investigate the relationship between markers of inflammation with physical growth and systemic markers of GH secretion in JIA. DESIGN: This is a cross sectional prospective study of patients with JIA recruited during therapeutic arthrocentesis of 17 children with JIA (F,10): 8 oligoarticular (OJIA) and 9 polyarticular (PJIA). RESULTS: Median adjusted height (AHt) SDS was -0.3 (-2.2 to 1.6). Serum ALS SDS (median -1.3, range -2.7 to -0.6) was reduced compared with serum IGFBP-3 SDS (median -0.5, range -7.7 to 2.3) and IGF-1 SDS (median -0.2, range -0.5 to 0.5). Log serum IL5 (95% CI -3.25, -0.81) and log serum IL15 (95% CI -9.58, -4.10) were independent factors associated with AHt SDS. Inflammatory cytokines individually showed no association with IGF-1, IGFBP-1, -2, -3 and ALS. CONCLUSION: Children with JIA and mild degree of growth retardation show decreased ALS and IGFBP-3. Cytokines did not show an association to systemic markers of GH secretion. However, this study reports the novel, preliminary association between serum levels of IL5 and IL15 and the extent of short stature

71. WU JF, YANG YH, WANG LC, LEE JH, SHEN EYet CHIANG BL: Comparative usefulness of C-reactive protein and erythrocyte sedimentation rate in juvenile rheumatoid arthritis, Clin.Exp.Rheumatol., Vol. 25(5), 782-785., 2007
    Organism:Department of Pediatrics, National Taiwan University Hospital, TaipeiFAU - Wu, J-F
    Abstract:OBJECTIVE: To compare serial C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) levels in juvenile rheumatoid arthritis (JRA) patients and investigate their application as diagnostic parameters and prognostic predictive factors. METHODS: We carried out retrospective chart review among JRA patients who were followed-up at the National Taiwan University Hospital (NTUH) between 1994 and 2005. RESULTS: Thirty-nine girls and 68 boys were included in this study. At the time of diagnosis, the prevalence of ESR was significantly greater than that of CRP (86.8% vs. 47.2%, p < 0.05). ESR revealed more responsiveness to treatment compared to CRP (SRMs were -0.69 and -0.31, respectively). At the time of diagnosis, high CRP levels (>or= 5mg/dL) correlated with poor therapeutic response, as do positive CRP (> 0.8 mg/dL) and high ESR levels (> 40 mm/h) after treatment for six months. Overall, initial high CRP levels (>or= 5mg/dL) demonstrated the strongest predictive role of failure of the first remission. CONCLUSION: For disease diagnosis, ESR can be a better parameter than CRP but a high initial CRP level can strongly predict treatment failure of the first remission

72. YILDIZ Bet KURAL N: IgG1 deficiency and high IgA level with juvenile idiopathic arthritis, Eur.J.Pediatr., Vol. 166(11), 1179-1180., 2007
    Organism:Department of Paediatrics, Faculty of Medicine, Eskisehir Osmangazi University, 26480, Eskisehir, Turkey bilalyn@yahoocomFAU - Yildiz, Bilal
    Abstract:

73. YOKOTA S: [Interleukin-6 as a pathogenic factor of systemic-onset juvenile idiopathic arthritis], Nihon Rinsho Meneki.Gakkai Kaishi., Vol. 31(2), 99-103., 2008
    Organism:Department of Pediatrics, Yokohama City University School of MedicineFAU - Yokota, Shumpei
    Abstract:Systemic-onset juvenile idiopathic arthritis (JIA) is a subtype of chronic childhood arthritis of unknown cause, manifested by spiking fever, erythematous rash, arthritis, pericarditis, and hepatosplenomegaly. It has been believed a disease developed due to an excessive production of pro-inflammatory cytokines, especially interleukin (IL)-6. We organized trials of a new biologic response modifier, Toclizimuab, anti-IL-6 receptor monoclonal antibody, to patients with systemic-onset JIA. Tocilizumab directs to solely IL-6 receptor, and is called as a one-point hit drug. We successfully administered Tocilizumab to stabilize the inflammation, and inflammatory symptoms and signs of the patients were abruptly gone. Previously, corticosteroids were the only life-saving drugs, but we proved Tocilizumab will be the possible alternative for treating these children. Basic science will help us to save the children, and clinical science also will promote the basic science in turn

74. YOKOTA S, IMAGAWA T, MORI M, MIYAMAE T, AIHARA Y, TAKEI S, IWATA N, UMEBAYASHI H, MURATA T, MIYOSHI M, TOMIITA M, NISHIMOTO Net KISHIMOTO T: Efficacy and safety of tocilizumab in patients with systemic-onset juvenile idiopathic arthritis: a randomised, double-blind, placebo-controlled, withdrawal phase III trial, Lancet., Vol. 371(9617), 998-1006., 2008
    Organism:Department of Paediatrics, Yokohama City University School of Medicine, Yokohama, Japan syokota@medyokohama-cuacjpFAU - Yokota, Shumpei
    Abstract:BACKGROUND: Systemic-onset juvenile idiopathic arthritis does not always respond to available treatments, including antitumour necrosis factor agents. We investigated the efficacy and safety of tocilizumab, an anti-interleukin-6-receptor monoclonal antibody, in children with this disorder. METHODS: 56 children (aged 2-19 years) with disease refractory to conventional treatment were given three doses of tocilizumab 8 mg/kg every 2 weeks during a 6-week open-label lead-in phase. Patients achieving an American College of Rheumatology Pediatric (ACR Pedi) 30 response and a C-reactive protein concentration (CRP) of less than 5 mg/L were randomly assigned to receive placebo or to continue tocilizumab treatment for 12 weeks or until withdrawal for rescue medication in a double-blind phase. The primary endpoint of the double-blind phase was an ACR Pedi 30 response and CRP concentration of less than 15 mg/L. Patients responding to tocilizumab and needing further treatment were enrolled in an open-label extension phase for at least 48 weeks. The analysis was done by intention to treat. This study is registered with ClinicalTrials.gov, numbers NCT00144599 (for the open-label lead-in and double-blind phases) and NCT00144612 (for the open-label extension phase). FINDINGS: At the end of the open-label lead-in phase, ACR Pedi 30, 50, and 70 responses were achieved by 51 (91%), 48 (86%), and 38 (68%) patients, respectively. 43 patients continued to the double-blind phase and were included in the efficacy analysis. Four (17%) of 23 patients in the placebo group maintained an ACR Pedi 30 response and a CRP concentration of less than 15 mg/L compared with 16 (80%) of 20 in the tocilizumab group (p<0.0001). By week 48 of the open-label extension phase, ACR Pedi 30, 50, and 70 responses were achieved by 47 (98%), 45 (94%), and 43 (90%) of 48 patients, respectively. Serious adverse events were anaphylactoid reaction, gastrointestinal haemorrhage, bronchitis, and gastroenteritis. INTERPRETATION: Tocilizumab is effective in children with systemic-onset juvenile idiopathic arthritis. It might therefore be a suitable treatment in the control of this disorder, which has so far been difficult to manage

75. ZHAO YXet LIU Y: [Clinical observation on effects of leflunomid and total glucosides of paeony on rheumatoid arthritis], Zhongguo Zhong.Xi.Yi.Jie.He.Za Zhi., Vol. 26(4), 355-357., 2006
    Organism:The Third Affiliated Hospital of Xinxiang Medical College , Henan 453003 ZYX080808@163comFAU - Zhao, Yong-xin
    Abstract:OBJECTIVE: The observe the clinical effect of leflunomide (LEF) and total glucosides of Paeony (TGP) on rheumatoid arthritis (RA) and their influences on laboratory findings. METHODS: Eighty patients with RA were randomly divided into 2 groups, 40 in each group: the treated group treated with TGP and leflunomide, and the control group treated with LEF alone, the therapeutic course for both groups was 12 weeks. Clinical effect after treatment, changes of symptoms and physical signs before and after treatment were observed and the relative laboratory indexes were detected as well. RESULTS: The total effective rate in the treated group was higher than that in the control group (97.5% vs 85.0%, P < 0.05). The clinical and laboratory indexes in the treated group were improved significantly after treatment (P < 0.05, P < 0.01), with the improvement superior to those in the control group (P < 0.05, P < 0.01) respectively. There was no significant difference in adverse reaction between the two groups. CONCLUSION: Combined application of TGP and LEF is superior to using of LEF alone in treating RA, owing to its quicker initiating action and less adverse reaction