Bibliography March 2008

1. Anonymous, Role of power Doppler sonography in evaluation of therapeutic response of the knee in juvenile rheumatoid arthritis, Ultrasound Q., Vol. 24(1), 43, 2008
   Organism:

2. Anonymous, Methotrexate: new indication. Idiopathic juvenile arthritis: the standard immunosuppressant, Prescrire.Int., Vol. 17(93), 15, 2008
   Organism:Lengthy follow-up, but mediocre comparative assessment

3. 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:Department of Pediatrics, University of Genoa, G Gaslini Institute, Genoa, ItalyFAU - Alpigiani, M G
   Abstract:

4. ALSAADI G, QUIRYNEN M, MICHILES K, TEUGHELS W, KOMAREK Aet VAN STEENBERGHE D: Impact of local and systemic factors on the incidence of failures up to abutment connection with modified surface oral implants, J.Clin.Periodontol., Vol. 35(1), 51-57., 2008
   Organism:Department of Periodontology, School of Dentistry, Oral Pathology and Maxillofacial Surgery, Faculty of Medicine, Catholic University of Leuven, Leuven, BelgiumFAU - Alsaadi, Ghada
   Abstract:AIM: This study aimed to assess the influence of systemic and local bone and intra-oral factors on the occurrence of early TiUnite implant failures. MATERIAL AND METHODS: A total of 283 consecutive patients (187 females; mean age 56.2), who received a total of 720 TiUnite implants, at the Department of Periodontology of the University Hospital of the Catholic University of Leuven, were prospectively followed. The following aspects were particularly assessed: hypertension, cardiac problems, gastric problems, osteoporosis, hypo- or hyperthyroid, hypercholesterolaemia, asthma, diabetes types I or II, Crohn's disease, rheumatoid arthritis, chemotherapy, hysterectomy and intake of medication (antidepressants, steroids, hormone replacement), radiotherapy of the concerned area, breach of sterility during surgery, implant parameters, bone (quality, quantity, dehiscence or perforation), type of edentulism, antibiotics prescription, fenestration of the implant in the sinus/nasal cavity, immediate implant placement, apical lesion detection and insertion torque. RESULTS AND CONCLUSION: A global failure rate of 1.9% was recorded. Owing to the very few failures, no definitive conclusion concerning statistical significance can be achieved. However, a tendency for more failures was noticed for apical lesions, vicinity with natural dentition, smoking, hormone replacement, gastric problems, Crohn's disease, diabetes I and radical hysterectomy

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. BARTOLI M, TARO M, MAGNI-MANZONI S, PISTORIO A, TRAVERSO F, VIOLA S, MAGNANI A, GASPARINI C, MARTINI Aet RAVELLI A: The magnitude of early response to methotrexate therapy predicts long-term outcome of patients with juvenile idiopathic arthritis, Ann.Rheum.Dis., Vol. 67(3), 370-374., 2008
   Organism:Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S Matteo, Pavia, ItalyFAU - Bartoli, M
   Abstract:OBJECTIVE: To investigate the relationship between the magnitude of clinical response in the first 6 months of methotrexate (MTX) therapy and long-term outcome in children with juvenile idiopathic arthritis (JIA). METHODS: The clinical charts of 125 JIA patients who were started with MTX and then followed for at least 5 years were reviewed. Based on the level of American College of Rheumatology (ACR) Pediatric response at 6 months, patients were divided in four mutually exclusive groups: (1) non-responders, (2) responders at 30%, (3) responders at 50%, and (4) responders at 70%. The long-term outcome in each response group was evaluated by calculating the percentage change in active and restricted joint counts from baseline to 1, 2 and 5 years and the frequency of inactive disease at 5 years. RESULTS: At 6 months, 42 patients were classified as non-responders, 24 as 30% responders, 26 as 50% responders, and 33 as 70% responders. Patients who had achieved a 70% response showed a significantly greater percentage improvement in active joint count between baseline to 5 years compared with non-responders and 30% responders, and a significantly greater percentage improvement in restricted joint count between baseline to 5 years compared with 30% responders. The 70% responders also had a greater frequency of inactive disease at 5 years compared with 30% responders, CONCLUSIONS: Our results show that the achievement of an ACR Pediatric 70 response at 6 months after start of MTX therapy predicts a more favorable long-term outcome of patients with JIA

7. BESTARD MO, POVEDA MR, IBERNON VM, CARRERA PMet GRINYO BOIRA JM: Systemic AA amyloidosis induced by benign neoplasms, Nefrologia., Vol. 28(1), 93-98., 2008
   Organism:Amyolidosis is a systemic disorder characterized by the extracelllular tissue deposition of insoluble, toxic aggregates in bundles of beta- sheet fibrillar proteins. These deposits are typically identified on the bases of their apple-green birrefringence under a polarized light microscope after staining with Congo red, and by the presence of rigid, nonbranching fibrils 8 to 10 nm in diameter on electron microscopy. The type of amyloid fibril unit can be further defined by immunohistology or by immunoelectron microscopy. It has been described at least 25 different human protein precursors of amyloid fibrils, which will describe its corresponding amyloid disease. The most common types of amyloidosis are AL (primary) and AA (secondary) types; the former, is the most frequent and is due to deposition of proteins derived from immunoglobulin light chain fragments, occurring alone or in association with multiple myeloma. The later (AA), is caused by deposition of fibrils composed of fragments of the acute phase reactant serum amyloid A (SAA) and complicates chronic diseases with ongoing or recurring inflammation, namely; rheumatoid arthritis (RA), juvenile chronic polyarthritis, ankylosing spondylitis, familial periodic fever syndromes (Familial Mediterranean Fever), chronic infections and furthermore, some neoplasms (mainly renal cell carcinoma and Hodking's disease). Despite its less frequent association, some benign neoplasms can subsequently complicate to AA amyloidosis, therefore, an early diagnose and successful treatment may lead indeed, to regression of the amyloid disease. Herein, we present two cases of AA amyloidosis, both of them caused by 2 different benign neoplasms: 1. A 34 year-old woman, after chronic oral contraceptive use, developed an hepatic adenoma (fig. 1) which finally lead to AA amyloidosis with primary kidney presentation (pure nephrotic syndrome) (table 1). Post-surgical complications yield to acute renal failure from which unfortunately could not be recovered. After being on hemodialysis therapy during 10 months she received a first renal allograft without any complication. 2. A 20 yearold woman, was diagnosed of AA amyloidosis after a renal biopsy (fig. 2) because of nephrotic syndrome (table 1). Further investigation lead to the finding of a hialyne-vascular type Castleman's disease located in the retroperotoneum (fig. 2). Despite surgical resection and medical treatment (colchicine) she developed progressive renal failure requiring initialization of hemodialysis therapy. After 6 years being on hemodialysis, she received a first renal allograft which is currently functioning after one year of follow- up. Although other chronic inflamatory diseases complicate more frequently to AA amyloidosis, benign tumors have to be taken into account as a potential ethiological cause for secondary amyloidosis

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. BRUNNER JK, SCHOLL-BURGI S, HOSSINGER D, WONDRAK P, PRELOG Met ZIMMERHACKL LB: Chitotriosidase activity in juvenile idiopathic arthritis, Rheumatol.Int., Vol. ., 2008
   Organism:Division of Rheumatology, Nephrology, Infectious diseases and Endocrinology, Department of Pediatrics, Innsbruck Medical University, Anichstrasse 35, 6020, Innsbruck, Austria, juergenbrunner@ukiat
   Abstract:Juvenile idiopathic arthritis (JIA) is an inflammatory joint disease of unknown etiology. The pathogenesis is driven by T and B cells. The role of macrophages remains unclear. Chitotriosidase belongs to the chitinase protein family and is secreted by activated macrophages. The chitinases are able to catalyze the hydrolysis of chitin or chitin-like substrates such as 4-methylumbelliferyl chitotrioside. Chitotriosidase activity was determined using the substrate 4-methylumbelliferyl beta-DNN'N''-triacetylchitotrioside (4-MU-TCT, SIGMA Chemical Co.). The substrate and serum were incubated with the serum in a citrate/phosphate buffer. The reaction was stopped by adding a buffer (Na(2)CO(3)). The fluorescence of 4-methylumbelliferone was evaluated by fluorimeter at excitation 360 nm and emission 450 nm. We report about chitotriosidase measurements in patients with JIA. The chitotriosidase level in synovial fluid was up to approximately 1,000 nmol/(h ml) at disease onset before therapy. The level in the sera was below 600 nmol/(h ml). Serum chitotriosidase levels could represent the activity of macrophages in the synovial fluid in JIA

10. CESPEDES-CRUZ A, GUTIERREZ-SUAREZ R, PISTORIO A, RAVELLI A, LOY A, MURRAY KJ, GERLONI V, WULFFRAAT N, OLIVEIRA S, WALSH J, PENADES IC, ALPIGIANI MG, LAHDENNE P, SAAD-MAGALHAES C, CORTIS E, LEPORE L, KIMURA Y, WOUTERS C, MARTINI Aet RUPERTO N: Methotrexate improves the health-related quality of life of children with juvenile idiopathic arthritis, Ann.Rheum.Dis., Vol. 67(3), 309-314., 2008
    Organism:IRCCS G Gaslini, Pediatria II, Reumatologia, PRINTO, Largo Gaslini, 5, 16147 Genova, ItalyFAU - Cespedes-Cruz, A
    Abstract:OBJECTIVES: To examine the change in health-related quality of life (HRQOL) and its determinants in children with juvenile idiopathic arthritis (JIA) treated with methotrexate (MTX). METHODS: Patients were extracted from the PRINTO clinical trial which aimed to evaluate the efficacy and safety profile of MTX administered in standard, intermediate or higher doses (10, 15 and 30 mg/m(2)/week respectively). Children with polyarticular-course JIA, who were less than 18 years and had a complete HRQOL assessment were included. RESULTS: A total of 521 children were included. At baseline, patients with JIA showed poorer HRQOL (p<0.01) than healthy children. In 207/412 (50%) and 63 (15%) children, HRQOL values were 2 standard deviations below the mean of healthy controls in the physical and psychosocial summary scale, respectively. After 6 months of treatment with standard dose MTX, there was a statistically significant improvement in all HRQOL health concepts, particularly the physical ones. Similar improvements were observed in those who did not respond to a standard dose of MTX and were subsequently randomised to a higher dose. The presence of marked disability at baseline was associated with a fivefold increased risk of retaining poor physical health after 6 months of active treatment with standard dose MTX. Other less important determinants of retaining poor physical well-being were the baseline level of systemic inflammation, pain intensity and an antinuclear-antibody-negative status. CONCLUSIONS: MTX treatment produces a significant improvement across a wide range of HRQOL components, particularly in the physical domains, in patients with JIA

11. DELL'ERA L, VERCELLESI P, FORZENIGO LV, CARNELLI Vet CORONA F: Synovial cyst in juvenile idiopathic arthritis, Clin.Rheumatol., Vol. ., 2008
    Organism:Pediatric Rheumatologic Centre, II Pediatric Clinic, Fondazione Policlinico, Mangiagalli e Regina Elena, Milan, Italy, lauradellera@yahooit
    Abstract:Small synovial cysts are a common manifestation of juvenile idiopathic arthritis; large brachial cysts, however, are a rare sign of the disease and they must be differentiated from other soft tissue swelling which are not related to articular involvement. We describe the case of three children with juvenile idiopathic arthritis who came to our attention with large synovial cysts. Ultrasonographic examination and MRI were performed in all cases, showing the real nature of the swelling and the connection to the joint. In all cases, swelling reduced and then disappeared with control of disease activity; in two cases, they reappeared in coincidence with a severe relapse of juvenile idiopathic arthritis.Brachial swellings represent a diagnostic challenge because they can be the clinical expression of a variety of diseases. In children with juvenile idiopathic arthritis who present with a sudden swelling of the upper arm, synovial cysts must be considered in the diagnostic workout, because they are a possible rare manifestation of juvenile idiopathic arthritis

12. DEWITT EM: Medication safety in children with arthritis, N.C.Med.J., Vol. 68(6), 427-429., 2007
    Organism:Duke University Medical Center Durham, NC 27710, USA esimorgandewitt@dukeeduFAU - DeWitt, Esi Morgan
    Abstract:

13. DING T, HALL A, JACOBS Ket DAVID J: Psychological functioning of children and adolescents with juvenile idiopathic arthritis is related to physical disability but not to disease status, Rheumatology (Oxford)., Vol. %20;., 2008
    Organism:Department of Rheumatology, Nuffield Orthopaedic Centre and Department of Paediatric Psychology, Oxford Children's Hospital, Oxford, UK
    Abstract:Objectives. This study investigates the psychological functioning of children with polyarticular joint disease and its association with disease activity and disability. Methods. Sixty children aged 7-18 yrs with juvenile idiopathic arthritis and >4 joints involved were recruited. Children underwent a physical examination. The Childhood HAQ was completed by both the children and their parents. Children also completed questionnaires for depression (Birleson Depression Inventory; BDI), anxiety (Revised Children's Manifest Anxiety Scale; RCMAS) and peer, emotional and behavioural problems (Strengths and Difficulties Questionnaire; SDQ). Clinical information was extracted from the hospital records. Results. Self-reported psychological functioning (depression, anxiety, and behaviour) was not different from the normal population. Parent-reported emotional difficulties on the SDQ were somewhat elevated. There were no significant correlations between psychological functioning and physician-rated disease activity score or the number of active joints at the time of assessment. Furthermore, no differences in psychological functioning were found between children with or without significantly raised inflammatory markers. All aspects of psychological function (depression, anxiety and behaviour) correlated moderately with physical function (r(s) = 0.49, 0.41, 0.46, respectively; all P < 0.01). Conclusions. Children and adolescents with polyarthritis are not at significantly elevated risk of psychological difficulties. Poor psychological outcome was associated with more severe physical disability but not with the level of disease activity

14. DOLMAN KM, BROUWER N, FRAKKING FN, FLATO B, TAK PP, KUIJPERS TW, FORRE Oet SMERDEL-RAMOYA A: Mannose-binding lectin deficiency is associated with early onset of polyarticular juvenile rheumatoid arthritis: a cohort study, Arthritis Res.Ther., Vol. 10(2), R32, 2008
    Organism:ABSTRACT: BACKGROUND: Mannose-binding lectin (MBL) is an innate immune protein. The aim of our study was to determine whether 1. genetically-determined MBL deficiency is associated with susceptibility to juvenile rheumatoid arthritis (JRA) and 2. MBL2 genotypes are associated with JRA severity. METHODS: In a retrospective cohort study of 218 patients with polyarthritis (n=67) and oligoarthritis (n=151), clinical and laboratory disease variables were obtained by clinical examination and chart reviews. Healthy Caucasian adults (n=194) served as controls. MBL2 gene mutations were determined by Taqman analysis to identify genotypes with high, medium and low MBL expression. Functional MBL plasma concentrations were measured by enzyme-linked immunosorbent assay. Associations between clinical and laboratory variables and MBL2 genotypes were determined by Kruskal-Wallis and Chi-square tests. RESULTS: MBL2 genotype frequencies were similar in polyarthritis and oligoarthritis patients as compared to controls. MBL plasma concentrations were associated with the high, medium and low MBL genotype expression groups (p<0.01). In polyarthritis patients, the presence of low-expressing (deficient) MBL2 genotypes was associated with early age of onset of disease (p=0.03). In oligoarthritis patients, patients with low-expressing MBL2 genotypes were more often in remission (81%) than patients in the medium (54%) and high (56%) genotype groups (p=0.02). The remaining clinical and laboratory variables, such as arthritis severity index, presence of radiographic erosions and antinuclear antibody positivity, were not associated with MBL2 genotypes. CONCLUSIONS: Genetically determined MBL deficiency does not increase susceptibility to JRA, but MBL-deficiency is associated with a younger age of onset of juvenile polyarthritis. On the other hand, MBL-deficient children with juvenile oligoarthritis are more often in remission. Therefore, MBL appears to play a dual role in JRA

15. 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:University of Manchester, Manchester, UKFAU - 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

16. DUARTE-SALAZAR C, GUZMAN-VAZQUEZ S, SOTO-MOLINA H, CHAIDEZ-ROSALES P, ILIZALITURRI-SANCHEZ V, NIEVES-SILVA J, VALERO-GONZALEZ Fet AGUILERA-ZEPEDA JM: Disability impact on quality of life in Mexican adults with juvenile idiopathic arthritis and juvenile ankylosing spondylitis, Clin.Exp.Rheumatol., Vol. 25(6), 922-927., 2007
    Organism:Department of Rheumatology, Instituto Nacional de Rehabilitacion, Mexico City caro20@ prodigynetmxFAU - Duarte-Salazar, C
    Abstract:OBJECTIVE: Our aim was to determine the disability impact on quality of life (QOL) in Mexican adults with juvenile idiopathic arthritis polyarticular course (JIAPA) and juvenile ankylosing spondylitis (JAS). METHODS: A cross-sectional study was performed on 32 adult patients with juvenile idiopathic arthritis. Functional outcome was evaluated using Global Functional Status (GFS) according to American College of Rheumatology (ACR) and Spanish Health Assessment Questionnaire-Disability Index (HAQ-DI) arthritis-specific measurements for functional disability in patients with polyarticular course and Bath Ankylosing Spondylitis Functional Index (BASFI) for those who developed JAS. Quality of life (QOL) was assessed using SF-36 and EuroQol 5D (EQ-5D). Descriptive statistics and associations among clinical, functional, and QOL measurements were examined using Spearman's correlation test. Multiple regression analysis was used to estimate predictor factors for impaired QOL. Differences between groups were evaluated by Fisher exact and Mann-Whitney U tests, and p values of <0.05 were considered statistically significant. RESULTS: JIAPA and JAS had GFS III/IV in 65 and 50%, respectively. A HAQ-DI score of > 1.5 was found in 35% of JIAPA, and a BASFI score of > 5 in 92% of JAS. Patients with JIAPA and JAS reported lower scores for all physical domains and for mental domains (physical role, social functioning, and emotional role) compared with Mexican population scores (p < 0.005). Health status between both groups studied does not show significant differences (p > 0.05). EQ-5D showed impairment in all five dimensions for both groups studied. Multiple regression analysis showed that GFS was the only variable that affects QOL assessed by SF36. CONCLUSIONS: In our study population, JIAPA and JAS exhibited a great disability impact on QOL and poor functional outcome during the patients' adult life. GFS has a significant impact on quality of life

17. EL MASRY MA, EL ASSUITY WI, SADEK FZet SALAH H: Two methods of atlantoaxial stabilisation for atlantoaxial instability, Acta Orthop.Belg., Vol. 73(6), 741-746., 2007
    Organism:Cairo University Teaching Hospitals, Cairo, Egypt drmedoelmasry@yahoocoukFAU - El Masry, Mohamed Adel
    Abstract:Thirty-seven patients, 19 males and 18 females, with a mean age of 37.6 years (range 9-62), underwent atlantoaxial fusion for atlantoaxial instability associated with pseudarthrosis of the odontoid, fixed rotary subluxation, rheumatoid arthritis, and mongolism. Two operative techniques were used: transarticular C1-C2 screws and posterior bone grafts according to Magerl, but without posterior wiring, in 24 patients (group 1), and C1 lateral mass screws/C2 pedicle screws, plates and posterior bone grafts, according to Goel, in 13 patients (group 2). The mean follow-up period was 27.6 months. In both groups 92% of the patients were free of neck pain. In group 1, 4 out of 9 patients with neurological involvement improved one Frankel grade and in group 2, 3 out of 5. The fusion rates were 96% and 100%, respectively; they were superior to the rates mainly seen after a Gallie fusion: 67 to 86%. One vertebral artery injury without sequelae occurred in group 1, and one wound infection, that healed with debridement, in group 2. In conclusion, the results were excellent in both groups, but slightly better in group 2

18. FROSCH Met ROTH J: New insights in systemic juvenile idiopathic arthritis--from pathophysiology to treatment, Rheumatology (Oxford)., Vol. 47(2), 121-125., 2008
    Organism:University of Muenster, Department of Paediatrics, Albert-Schweitzer-Str 33, D-48149 Muenster, Germany froschm@uni-muensterdeFAU - Frosch, M
    Abstract:Systemic juvenile idiopathic arthritis (SJIA) is characterized by the clinical features of remitting fever, a typical skin rash and arthritis. Many patients show frequent flares or persistent disease activity with significant morbidity and serious complications. Recent investigations in the pathophysiology of SJIA have focused on mediators of the innate immune system. Especially IL-1beta, IL-6 and IL-18 as well as phagocyte-specific S100-proteins (S100A8, S100A9 and S100A12) are correlated with disease activity and secondary complications. Beside IL-6 all these molecules are secreted by a so-called alternative pathway. A loss of control of the alternative secretory pathway seems to be involved in release of pro-inflammatory proteins leading to the inflammatory process of SJIA. These insights lead to new promising treatment approaches, like application of recombinant anti-IL-1 receptor antagonist or anti-IL-6 receptor antibodies in patients resistant to conventional anti-inflammatory treatment. First case studies show improvement and remission on therapy in a substantial portion of these patients. In this review, we summarize the current knowledge of pathophysiology and experiences in the treatment of SJIA

19. GARCIA-CONSUEGRA MJ, MERINO MRet DE INOCENCIO AJ: [Macrophage Activation Syndrome and Juvenile Idiopathic Arthritis. A multicenter study.], An.Pediatr.(Barc.)., Vol. 68(2), 110-116., 2008
    Organism:Seccion de Reumatologia Pediatrica Hospital Universitario La Paz Madrid Espana reumapedhulp@saludmadridorgFAU - Garcia-Consuegra Molina, J
    Abstract:INTRODUCTION: Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile idiopathic arthritis (sJIA). OBJECTIVE: To describe the clinical characteristics and outcome of patients diagnosed with MAS in Spanish pediatric rheumatology units. PATIENTS AND METHOD: A protocol for data collection was designed and distributed to pediatricians and rheumatologists attending children with rheumatic diseases. RESULTS: Information was available from 31 patients (16 boys and 15 girls) who had 37 MAS episodes. Twenty-seven children had only one episode, three had two episodes and one had four episodes. The interval between episodes ranged from 1 to 33 months. The median age was 5.9 years (range 1-23). MAS was the initial manifestation of sJIA in nine patients. The most frequent symptom was fever (97 %), followed by skin rash (49 %), central nervous system dysfunction (41 %), and gastrointestinal abnormalities (15 %). Abnormal laboratory findings included thrombopenia (78 %) and elevated levels of hepatic enzymes (70 %). Hemophagocytosis was confirmed in 16 of 30 bone marrow samples evaluated, 15 with cyclosporine A and six with etoposide. All episodes but one were treated with steroids. One patient received a liver transplant before diagnosis. The mortality rate was 6.5 % (2/31). CONCLUSION: MAS is a severe, potentially lethal, complication of sJIA. The clinical and laboratory abnormalities characteristic of sJIA complicate its diagnosis. The earliest and most frequent findings were decreased in platelet count and elevation of hepatic enzymes. A high degree of suspicion as well as early diagnosis and prompt treatment are essential in this disease

20. HALVORSEN EH, POLLMANN S, GILBOE IM, VAN DER HD, LANDEWE R, ODEGARD S, KVIEN TKet MOLBERG O: Serum IgG antibodies to peptidylarginine deiminase 4 in rheumatoid arthritis and associations with disease severity, Ann.Rheum.Dis., Vol. 67(3), 414-417., 2008
    Organism:Institute of Immunology, University of Oslo, Rikshospitalet University Hospital, 0027 Oslo, Norway eirikhha@medisinuionoFAU - Halvorsen, E H
    Abstract:BACKGROUND: Antibodies targeting citrullinated antigens are specific for rheumatoid arthritis (RA). Citrullination is catalysed by the peptidylarginine deiminase (PAD) enzyme family. Critical enzymes are often targeted by disease-specific antibodies in complex immune-mediated diseases. Here, we have tested for autoantibodies against human recombinant PAD4 (hPAD4) in Caucasian RA patients. METHODS: A time-resolved fluorometric immunoassay based on hPAD4 was developed to analyse sera from two RA cohorts (n = 237 and n = 177), one systemic lupus erythaematosus (SLE) cohort (n = 84) and 148 healthy controls. Simple and multiple analyses were performed to examine possible associations between anti-hPAD4 and disease variables. RESULTS: Raised levels of anti-hPAD4 IgG were found in both RA cohorts compared to the controls, and 23% of the RA patients were anti-hPAD4 IgG positive. Anti-hPAD4 was associated with anti-cyclic citrullinated peptide (CCP) and rheumatoid factor (RF), as well as increased physical disability. Anti-hPAD4 was also associated with higher longitudinal radiographic damage scores and increased clinical joint pathology, but weaker than anti-CCP. No associations were found between anti-hPAD4 and selected Human leukocyte antigen (HLA)-DRB1 variants. CONCLUSIONS: Approximately 23% of Caucasian RA patients have serum IgG antibodies against hPAD4.The presence of serum anti-hPAD4 IgG was in simple analyses associated with a more severe disease phenotype, and the association with physical disability was maintained in multiple analyses

21. HELMICK CG, FELSON DT, LAWRENCE RC, GABRIEL S, HIRSCH R, KWOH CK, LIANG MH, KREMERS HM, MAYES MD, MERKEL PA, PILLEMER SR, REVEILLE JDet STONE JH: Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I, Arthritis Rheum., Vol. 58(1), 15-25., 2008
    Organism:CDC, Atlanta, Georgia 30341-3717, USA CHelmick@cdcgovFAU - Helmick, Charles G
    Abstract:OBJECTIVE: To provide a single source for the best available estimates of the US prevalence of and number of individuals affected by arthritis overall, rheumatoid arthritis, juvenile arthritis, the spondylarthritides, systemic lupus erythematosus, systemic sclerosis, and Sjogren's syndrome. A companion article (part II) addresses additional conditions. METHODS: The National Arthritis Data Workgroup reviewed published analyses from available national surveys, such as the National Health and Nutrition Examination Survey and the National Health Interview Survey (NHIS). For analysis of overall arthritis, we used the NHIS. Because data based on national population samples are unavailable for most specific rheumatic conditions, we derived estimates from published studies of smaller, defined populations. For specific conditions, the best available prevalence estimates were applied to the corresponding 2005 US population estimates from the Census Bureau, to estimate the number affected with each condition. RESULTS: More than 21% of US adults (46.4 million persons) were found to have self-reported doctor-diagnosed arthritis. We estimated that rheumatoid arthritis affects 1.3 million adults (down from the estimate of 2.1 million for 1995), juvenile arthritis affects 294,000 children, spondylarthritides affect from 0.6 million to 2.4 million adults, systemic lupus erythematosus affects from 161,000 to 322,000 adults, systemic sclerosis affects 49,000 adults, and primary Sjogren's syndrome affects from 0.4 million to 3.1 million adults. CONCLUSION: Arthritis and other rheumatic conditions continue to be a large and growing public health problem. Estimates for many specific rheumatic conditions rely on a few, small studies of uncertain generalizability to the US population. This report provides the best available prevalence estimates for the US, but for most specific conditions, more studies generalizable to the US or addressing understudied populations are needed

22. HERLIN Tet THASTUM M: [Juvenile idiopathic arthritis--pain and coping strategies], 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

23. HING CB, BACK DL, BAILEY M, YOUNG DA, DALZIEL REet SHIMMIN AJ: The results of primary Birmingham hip resurfacings at a mean of five years. An independent prospective review of the first 230 hips, J.Bone Joint Surg.Br., Vol. 89(11), 1431-1438., 2007
    Organism:Melbourne Orthopaedic Group, 33 The Avenue, Windsor, Victoria 3181, AustraliaFAU - Hing, C B
    Abstract:We report an independent prospective review of the first 230 Birmingham hip resurfacings in 212 patients at a mean follow-up of five years (4 to 6). Two patients, one with a loose acetabular component and the other with suspected avascular necrosis of the femoral head, underwent revision. There were two deaths from unrelated causes and one patient was lost to follow-up. The survivorship with the worst-case scenario was 97.8% (95% confidence interval 95.8 to 99.5). The mean Harris hip score improved significantly (paired t-test, p < 0.05) from 62.54 (8 to 92) pre-operatively to 97.7 (61 to 100) at a mean of three years (2.1 to 4.3), then deteriorated slightly to a mean of 95.2 (47 to 100) at a mean of five years. The mean flexion improved from 91.5 degrees (25 degrees to 140 degrees) to 110.4 degrees (80 degrees to 145 degrees) at a mean of three years with no further improvement at five years (111.2 degrees; 70 degrees to 160 degrees). On radiological review at five years, one patient had a progressive lucent line around the acetabular component and six had progressive lucent lines around the femoral component. A total of 18 femoral components (8%) had migrated into varus and those with lucent lines present migrated a mean of 3.8 degrees (1.02 degrees to 6.54 degrees) more than the rest. Superolateral notching of the femoral neck and reactive sclerosis at the tip of the peg of the femoral component were associated with the presence of lucent lines (chi-squared test, p < 0.05), but not with migration of the femoral component, and are of unknown significance. Our results with the Birmingham hip resurfacing continue to be satisfactory at a mean follow-up of five years

24. JOLLES BMet BOGOCH ER: Quality of life after TKA for patients with juvenile rheumatoid arthritis, Clin.Orthop.Relat Res., Vol. 466(1), 167-178., 2008
    Organism:Hopital Orthopedique de la Suisse Romande, Centre Hospitalier Universitaire Vaudois, University of Lausanne, 4 avenue Pierre Decker, 1005 Lausanne, Switzerland BrigitteJolles-Haeberli@chuvchFAU - Jolles, Brigitte M
    Abstract:Total knee arthroplasty frequently is required during early adulthood in patients with advanced juvenile rheumatoid arthritis. We queried patients on issues of importance to them, asked whether they were satisfied with surgical outcomes, and ascertained their postoperative status. We retrospectively reviewed 14 adult patients (22 knees) with severe juvenile rheumatoid arthritis who were treated with primary total knee arthroplasty between 1989 and 2001. All patients were evaluated by pain and stiffness visual analog scales, range of motion, the Patient-Specific Index, Hospital for Special Surgery knee score, WOMAC Osteoarthritis Index, EuroQuol in five dimensions, and SF-36 Health Survey. Preoperative scores were assessed by recall. Patients had a minimum followup of 2 years (mean, 8 years; range, 2-13 years). Quality of life improved after TKA as measured by the Patient-Specific Index. Eighteen of 22 patients rated themselves satisfied with the functional outcome of their surgery; all patients were satisfied with pain relief. Final SF-36, EuroQuol in five dimensions, and WOMAC scores were low compared with age-matched population norms. A mean postoperative flexion arc of 77 degrees (range, 30 degrees -130 degrees ) was observed. Total knee arthroplasty had a major positive impact on quality of life as reported by patients. LEVEL OF EVIDENCE: Level IV, therapeutic study

25. KALLIOLIAS GDet LIOSSIS SN: The future of the IL-1 receptor antagonist anakinra: from rheumatoid arthritis to adult-onset Still's disease and systemic-onset juvenile idiopathic arthritis, Expert.Opin.Investig.Drugs., Vol. 17(3), 349-359., 2008
    Organism:Hospital for Special Surgery, Arthritis and Tissue Degeneration Program, Department of Medicine, New York, NY 10021, USAFAU - Kalliolias, George D
    Abstract:BACKGROUND: IL-1 receptor antagonist (IL-1Ra) is a naturally occurring IL-1RI-binding molecule that blocks the biologic effects of the proinflammatory cytokine IL-1. A recombinant form of human IL-1Ra, anakinra (Kineret), has been approved for use in rheumatology initially to manage rheumatoid arthritis (RA) patients that are refractory to more conventional forms of treatment. OBJECTIVE: This review summarizes the experience with anakinra in the treatment of patients with rheumatic diseases emphasizing its beneficial effects in novel applications. METHODS: English-language trials of anakinra were searched using MEDLINE and abstracts from rheumatology scientific meetings. RESULTS/CONCLUSIONS: In the treatment of patients with RA anakinra is effective but inferior to TNF-alpha blocking agents. Over the last few years it has become increasingly evident that anakinra is highly effective and safe in patients with systemic-onset juvenile idiopathic arthritis, adult-onset Still's disease, hereditary autoinflammatory syndromes, Schnitzler's syndrome and recently in gouty attacks

26. KAPITANY A, SZABO Z, LAKOS G, ALEKSZA M, VEGVARI A, SOOS L, KARANYI Z, SIPKA S, SZEGEDI Get SZEKANECZ Z: Associations between serum anti-CCP antibody, rheumatoid factor levels and HLA-DR4 expression in Hungarian patients with rheumatoid arthritis, Isr.Med.Assoc.J., Vol. 10(1), 32-36., 2008
    Organism:Laboratory of Immunology, Third Department of Medicine, University of Debrecen Medical Centre, Debrecen, HungaryFAU - Kapitany, Aniko
    Abstract:BACKGROUND: The presence of anti-cyclic citrullinated peptide autoantibody is highly specific for rheumatoid arthritis. Certain HLA-DR4 (HLA-DRB1*04) alleles, also known as the "shared epitope," are associated with increased susceptibility to RA. In addition, these alleles may also have relevance for disease outcome. Anti-CCP antibody positivity has been associated with the presence of HLA-DR4 alleles in patients with RA. However, there is little information regarding a relationship between quantitative anti-CCP production (serum anti-CCP concentrations) and the shared epitope. OBJECTIVES: To determine the association between anti-CCP antibody production and various HLA-DRB1 alleles. METHODS: Serum anti-CCP, rheumatoid factor and C-reactive protein levels were assessed in 53 RA patients. All these patients underwent HLA-DRB1 genotyping. RESULTS: Of the 53 patients 33 (62%) were positive for anti-CCP antibody. We found significant correlations between anti-CCP and RF positivity (chi-square = 6.717, P < 0.01), as well as between anti-CCP and HLA-DRB1*04 positivity (chi-square = 5.828, P < 0.01). There was no correlation between RF positivity and serum levels, CRP serum levels and HLA-DRB1*04 positivity. When quantitatively comparing serum anti-CCP levels with shared epitope positivity, patients carrying one or two copies of HLA-DRB1*04 alleles had significantly higher anti-CCP concentrations (530.0 +/- 182.6 U/ml) compared to DRB1*04-negative patients (56.8 +/- 27.4 U/ml) (P < 0.01). There was no difference in serum anti-CCP antibody concentrations between patients carrying only one HLA-DRB1*01 allele but no HLA-DRB1*04 allele (12.0 +/- 8.6 U/ml) compared to SE-negative patients (76.8 +/- 56.2 U/ml). Regarding non-SE HLA-DRB1 genotypes, all 6 patients (100%) carrying DRB1*15 alleles and 6 of 7 (85%) patients carrying DRB1*13 were anti-CCP positive. In addition, patients with HLA-DRB1*13 (282.5 +/- 23.8 U/ml) and DRB1*15 (398.7 +/- 76.2 U/ml) produced significantly more anti-CCP than did any other non-SE HLA-DRB1 subtypes (P < 0.01). CONCLUSIONS: There is significant association between anti-CCP and RF, as well as between anti-CCP and SE positivity in RA. In addition, the presence of one or two copies of HLA-DRB1*04 alleles has been associated with higher serum anti-CCP antibody levels. Thus, patients carrying HLA-DRB1*04 alleles exhibited an overall tenfold increase in serum anti-CCP antibody levels in comparison to HLA-DRB1*04-negative subjects. Increased anti-CCP production may also be associated with other non-SE HLA-DRB1 genotypes, such as DRB1*13 or DRB1*15. In reports by other investigators, both anti-CCP concentrations and SE positivity were related to more rapid disease progression and unfavorable outcome

27. KASHEF S, SAKI F, KARAMIZADEH Zet KASHEF MA: Bone mineral density in children wth systemic lupus erythematosus and juvenile rheumatoid arthritis, Ann.Saudi.Med., Vol. 27(6), 427-431., 2007
    Organism:Department of Pediatrics, Allergy Research Center, Division of Endocrinology, Shiraz University of Medical Sciences, Shiraz, Iran kashefs@sumsacirFAU - Kashef, Sara
    Abstract:BACKGROUND: Although there is increasing in bone metabolism in patients with rheumatic disorders, few data exist on bone mineral density (BMD) in children with rheumatic disorders or on the association of BMD with disease-related variables. We determined BMD in Iranian children with systemic lupus erythematosus (SLE) and juvenile rheumatoid arthritis (JRA) to evaluate the relationship between disease-related variables and BMD. PATIENTS AND METHODS: Twenty patients (13 girls and 7 boys) with SLE (n=15) and JRA (n=5) with a mean age of 13.10+/-3.29 years (range, 6-17 years), attending a pediatric rheumatology clinic and 20 healthy controls (matched for age and sex with each patient) were enrolled in a cross-sectional study between 2001 and 2003. BMD (g/cm(2)) of the femoral neck (BMD-F) and lumbar vertebrae (BMD-L) were measured by dual energy X-ray absorptiometry (DEXA). The correlation between BMD and cumulative dose of steroids, daily dose of steroid, disease duration, disease activity, height, weight, and age was investigated. RESULTS: BMD in the patients (BMD-F=0.72+/-0.15, BMD-L=0.70+/-0.19) was significantly lower than controls (BMD-F=0.95+/-0.17, BMD-L=0.98+/-0.20, P=<0.001). The severity of descreased BMD was more prominent in lumbar vertebrae than the femoral neck (P=0.04). None of the variables were consistently related to a decrease in BMD. CONCLUSION: BMD was significantly lower in patients compared with controls. It was more prominent in lumbar vertebrae (trabecular bone). Although cumulative dose of steroids and diseaese appeared to have some influence on BMD, none were independently correlated with BMD

28. KAVANAUGH A, ROSENGREN S, LEE SJ, HAMMAKER D, FIRESTEIN GS, KALUNIAN K, WEI Net BOYLE DL: Assessment of rituximab's immunomodulatory synovial effects (ARISE trial). 1: clinical and synovial biomarker results, Ann.Rheum.Dis., Vol. 67(3), 402-408., 2008
    Organism:University of California, San Diego, Division of Rheumatology, Allergy, and Immunology, 9500 Gilman Drive, Mail Code 0943, La Jolla, CA 92093-0943, USA akavanaugh@ucsdeduFAU - Kavanaugh, A
    Abstract:OBJECTIVE: Treatment with the anti-CD20 monoclonal antibody (mAb) rituximab is effective in rheumatoid arthritis (RA). Marked depletion of circulating B cells, seen in almost all patients, does not correlate with efficacy. The potential synovial immunomodulatory effects of rituximab have not been fully defined. METHODS: The ARISE trial is an open label, serial synovial biopsy (pre-treatment and 8 weeks) study of rituximab, given 1 g intravenously on days 0 and 14 without peri-infusional steroids, in active RA patients on concomitant methotrexate (MTX). Synovial tissue was analysed by immunohistochemistry with digital image analysis and gene expression by real-time PCR. RESULTS: The mean (SD) baseline DAS28 score was 6.5 (0.4), and mean MTX dose 17.3 mg/week. Of 13 patients, 11 had failed prior tumour necrosis factor (TNF) inhibitor therapy. With treatment, all patients experienced near complete depletion of circulating B cell numbers. During the 6 months after treatment, 7/13 patients achieved an American College of Rheumatology (ACR) 20% improvement (ACR20) response, 3/13 an ACR50 response and 2/13 an ACR70 response. There was a significant decrease in synovial B cells after treatment, but only a small trend towards greater reduction among clinical responders. Among the three patients with ACR50 responses there was a significant decrease in synovial immunoglobulin synthesis. CONCLUSIONS: These data suggest that unlike those in circulation, synovial B cells are decreased but are not eliminated by rituximab therapy. Patients with higher levels of response may have more consistent depletion of synovial B cells, and may also have an alteration in synovial B cell function, as indicated by decreases in synovial immunoglobulin synthesis. Thus, effects on synovial B cells may be necessary but not sufficient for inducing clinical efficacy. Other effects, such as on primary lymph organ B cell antigen presentation or cytokine production, may be operative

29. KAWAI S, TAKAGI K, KUSUNOKI Y, NISHIO Set MATSUMOTO N: [Systemic autoimmune diseases], Nippon Rinsho., Vol. 66(1), 130-136., 2008
    Organism:Division of Rheumatology, Department of Internal Medicine (Omori), Toho University School of MedicineFAU - Kawai, Shinichi
    Abstract:Since Hench successfully treated a patient with rheumatoid arthritis (RA) with glucocorticoid (GC) in 1948, the clinical usefulness of GC in the management of systemic autoimmune diseases has been established. However, serious adverse reactions of GC are the severe clinical problems. In addition, some of clinical evidences of GC therapy for these diseases are still controversial due to the difficulties for conducting clinical trials. In this review, we summarize the significance of GC therapy in autoimmune diseases such as RA and systemic lupus erythematosus, based upon the clinical reports for these diseases

30. KINOUCHI R, HIROKAWA H, IGARASHI S, FUKUI K, HIRANO Y, TAKAI Yet YOSHIDA A: [A case of panuveitis with optic disc neovascularization associated with juvenile idiopathic arthritis which progressed during a clinical trial of etanercept], Nippon Ganka Gakkai Zasshi., Vol. 111(12), 970-975., 2007
    Organism:Department of Ophthalmology, Asahikawa Medical College, Japan rkino@asahikawa-medacjpFAU - Kinouchi, Reiko
    Abstract:PURPOSE: To report a case of uveitis associated with juvenile idiopathic arthritis that progressed from iritis to panuveitis with disc neovascularization during a clinical trial of etanercept, a tumor necrosis factor a (TNF-alpha) blocker. CASE: A 12-year-old girl with juvenile idiopathic arthritis, which had begun at the age of 1 year 7 months. The patient was enrolled in a clinical trial of etanercept at 11 years of age. The methotrexate which she has been taking was stopped, and prednisolone was decreased gradually from 7 mg. The iritis worsened and progressed to panuveitis with disc neovascularization when the prednisolone dose had been tapered to 2 mg. The uveitis was controlled by treatment with a steroid pulse and a liposteroid. CONCLUSION: When starting etanercept therapy in a patient with juvenile idiopathic arthritis who has uveitis and the antirheumatic drug is stopped and steroid treatment tapered, special care is needed to avoid the exacerbation of uveitis

31. KOENIG MK, PEREZ M, ROTHENBERG Set BUTLER IJ: Juvenile onset central nervous system folate deficiency and rheumatoid arthritis, J.Child Neurol., Vol. 23(1), 106-107., 2008
    Organism:University of Texas Medical School at Houston, TX 77030, USAFAU - Koenig, Mary Kay
    Abstract:Isolated cerebral folate deficiency was detected in a 13-year-old girl with cognitive and motor difficulties and juvenile rheumatoid arthritis. Her serum contains autoantibodies that block membrane-bound folate receptors that are on the choroid plexus and diminish the uptake of folate into the spinal fluid. Whereas her serum folate exceeded 21 ng/mL, her spinal fluid contained 3.2 ng/mL of 5-methyltetrahydrofolate as a consequence of the autoantibodies diminishing the uptake of this folate

32. KOSTINOV MP, TARASOVA AAet ZAITSEV EM: [Contents of antibodies to Bordetella pertussis antigens in patients with rheumatic diseases], Zh.Mikrobiol.Epidemiol.Immunobiol., Vol. (6), 61-64., 2007
    Organism:Study of presence of antibodies against pertussis in 72 rheumatic patients (with uvenile rheumatoid arthritis, systemic lupus erythematosus, etc.) aged 1-18 year old without history of pertussis was performed. Mean age of the patients was 10.6 +/- 0.48 year old, duration of illness--51.2 +/- 4.42 months. Immunosupressive therapy at the time of the study was conducted in 68 (94.4%) children. Using ELISA method, IgG to pertussis toxin (PT) and to antigens of acellular pertussis vaccine (aPV) were detected in 98.6% and 100% of children. High titers of antibodies were detected more frequently in 7-18 year old age group, which can indicate recent pertussis disease or infection. Vaccination history was studied in 131 children with rheumatic diseases. Incidence of pertussis in 43 unvaccinated children was 116.3 per 1000, and in 16 children with incomplete vaccination--62.5 per 1000. Out of 75 patients, who received vaccination series and revaccination, clinically distinct pertussis was not diagnosed

33. LATOS-BIELENSKA A, MARIK I, KUKLIK M, MATERNA-KIRYLUK A, POVYSIL Cet KOZLOWSKI K: Pachydermoperiostosis-critical analysis with report of five unusual cases, Eur.J.Pediatr., Vol. 166(12), 1237-1243., 2007
    Organism:Department of Medical Genetics, University of Medical Sciences, Poznan, PolandFAU - Latos-Bielenska, Anna
    Abstract:Pachydermoperiostosis (idiopathic hypertrophic arthropathy) {MIM 167100} is an uncommon disease characterized by unique phenotype (digital clubbing and pachydermia) and distinctive radiographic appearances (periostosis). Two families are reported that, in additional to the typical phenotype and radiographic characteristics of pachydermoperiostosis, show some rare and/or unusual, not yet reported, clinical findings. In the first family, distinctive features were severe progressive arthritis with villonodular involvement of the knees. The clinical course of the disease was much more severe than usually reported. The older brother was disabled at the age of 29 years. In the second family, the clinical history was exceptional, with unique early appearance of clinical signs. Pachydermoperiostosis is usually inherited as a dominant trait, but probable autosomal recessive inheritance has been reported. Also in the present families, autosomal recessive inheritance is likely, possibly explaining the severe clinical course of the disease. Differential diagnosis and the confusing nomenclature of pachydermoperiostosis are discussed

34. LEQUERRE T, QUARTIER P, ROSELLINI D, ALAOUI F, DE BANDT M, MEJJAD O, KONE-PAUT I, MICHEL M, DERNIS E, KHELLAF M, LIMAL N, JOB-DESLANDRE C, FAUTREL B, LE L, Xet SIBILIA J: Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France, Ann.Rheum.Dis., Vol. 67(3), 302-308., 2008
    Organism:Rheumatology Department, Rouen University Hospital & Inserm 905, 76031 Rouen, France thierrylequerre@univ-rouenfrFAU - Lequerre, T
    Abstract:BACKGROUND: Anakinra treatment has been reported to be effective in some patients with systemic-onset juvenile idiopathic arthritis (SoJIA) or adult-onset Still disease (AoSD). OBJECTIVES: To assess the efficacy and the safety of anakinra treatment in SoJIA and AoSD. METHODS: SoJIA and AoSD patients were treated with anakinra (1-2 mg/kg/day in children, 100 mg/day in adults); we analysed its effect on fever, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, numbers of swollen and tender joints, the assessment of disease activity (by physician and parent/patient) and pain (by parent/patient), and American College of Rheumatology (ACR) pediatric core set criteria for JIA activity. RESULTS: A total of 35 patients were included, 20 with SoJIA and 15 with AoSD. Their mean age (range) at the onset of treatment was 12.4 (3-23) and 38.1 (22-62) years, respectively; disease duration was 7.0 (1-16) and 7.8 (2-27) years, respectively. Active arthritis was present in all cases but one. Of the 20 SoJIA patients, 5 achieved ACR 50% improvement in symptoms (ACR50) response criteria at 6 months. Steroid dose had been decreased by 15% to 78% in 10 cases. A total of 11 of the 15 AoSD patients achieved at least a 50% improvement for all disease markers (mean follow-up: 17.5 (11-27) months). Steroids had been stopped in two cases and the dose was decreased by 45% to 95% in 12 patients. Two patients stopped anakinra due to severe skin reaction, and two patients due to infection: one visceral leishmaniasis and one varicella. CONCLUSION: Anakinra was effective in most AoSD patients, but less than half SoJIA patients achieved a marked and sustained improvement

35. MAMAN E, BICKELS J, EPHROS M, PARAN D, COMANESHTER D, METZKOR-COTTER E, AVIDOR B, VARON-GRAIDY M, WIENTROUB Set GILADI M: Musculoskeletal manifestations of cat scratch disease, Clin.Infect.Dis., Vol. 45(12), 1535-1540., 2007
    Organism:Department of Pediatric Orthopaedics, Dana Children's HospitalFAU - Maman, Eran
    Abstract:BACKGROUND: Musculoskeletal manifestations (MMs) are considered to be rare in cat scratch disease (CSD) and are not well characterized. We aimed to study MMs of CSD. METHODS: A surveillance study performed over 11 years identified patients with CSD on the basis of compatible clinical presentation and confirmatory serological test or PCR results for Bartonella henselae. Patients with CSD who had MMs (i.e., myalgia, arthritis, arthralgia, tendinitis, osteomyelitis, and neuralgia) were compared with patients with CSD who did not have MMs (control subjects). RESULTS: Of 913 patients with CSD, 96 (10.5%) had MMs. Myalgia (in 53 patients [5.8%]) was often severe, with a median duration of 4 weeks (range, 1-26 weeks). Arthropathy (arthralgia and/or arthritis; in 50 patients [5.5%]) occurred mainly in the medium and large joints and was classified as moderate or severe in 26 patients, with a median duration of 5.5 weeks (range, 1-240 weeks). In 7 patients, symptoms persisted for >or=1 year; 5 developed chronic disease. Tendinitis, neuralgia, and osteomyelitis occurred in 7, 4, and 2 patients, respectively. Patients with MMs were significantly older than patients in the control group (median age, 31.5 years vs. 15.0 years). In multivariate analysis, age >20 years was associated with having any MM (relative risk [RR], 4.96; 95% confidence interval [CI], 2.79-8.8), myalgia (RR, 4.69; 95% CI, 2.22-9.88), and arthropathy (RR, 11.0; 95% CI, 4.3-28.2). Arthropathy was also associated with female sex (RR, 1.89; 95% CI, 1.01-3.52) and erythema nodosum (RR, 4.07; 95% CI, 1.38-12.02). CONCLUSIONS: MMs of CSD are more common than previously thought and affect one-tenth of patients with CSD. MMs occur mostly in patients aged >20 years and may be severe and prolonged. Osteomyelitis, the most well known MM of CSD is, in fact, the rarest

36. 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

37. NAKANO S, MORIMOTO S, SUZUKI J, NOZAWA K, AMANO H, TOKANO Yet TAKASAKI Y: Role of pathogenic auto-antibody production by Toll-like receptor 9 of B cells in active systemic lupus erythematosus, Rheumatology (Oxford)., Vol. 47(2), 145-149., 2008
    Organism:Department of Rheumatology and Internal Medicine, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421, Japan soubey@medjuntendoacjpFAU - Nakano, S
    Abstract:OBJECTIVES: Toll-like receptor 9 (TLR9) is a pattern-associated receptor functioning in innate immunity that may be involved in the recognition of self-antigens and the production of pathogenic auto-antibodies. Therefore, we examined the expression of TLR9 in systemic lupus erythematosus (SLE) to determine whether TLR9 is involved in the production of pathogenic auto-antibodies. METHODS: B cells were collected from patients with active SLE, and subjected to analysis of the TLR9 molecule using flow cytometry fluorescence activated cell sorting (FACS) and TLR9 mRNA by reverse-transcriptase polymerase chain reaction. SLE B cells were stimulated with CpG-ODN, and subsequent cytokine and anti-dsDNA antibody production was measured by enzyme-linked immunosorbent assay. RESULTS: The expression and mRNA level of TLR9 on B cells was up-regulated in SLE patients, and SLE disease activity index (SLEDAI) and CH50 were correlated with TLR9 expression on CD20+ B cells. Moreover, TLR9-CpG interaction enhanced the production of anti-dsDNA antibody and IL-10. CONCLUSIONS: The present study demonstrated that higher expression of TLR9 on peripheral blood B cells from patients with active SLE was significantly correlated with CH50 and SLEDAI to TLR9, and induced the production of anti-dsDNA antibody and IL-10 by TLR9-CpG ligation. These results suggest that an abnormality of innate immunity plays a crucial role in the pathology of SLE, and that blockade of CpG-TLR9 interaction may be a new therapeutic approach for SLE

38. 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

39. OHLSSON V, BAILDAM E, FOSTER H, JANDIAL S, PAIN C, STRIKE Het RAMANAN AV: Anakinra treatment for systemic onset juvenile idiopathic arthritis (SOJIA), Rheumatology (Oxford)., Vol. 47(4), 555-556., 2008
    Organism:

40. OKUMUS O, ERGUVEN M, DEVECI M, YILMAZ Oet OKUMUS M: Growth and bone mineralization in patients with juvenile idiopathic arthritis, Indian J.Pediatr., Vol. 75(3), 239-243., 2008
    Organism:Department of Pediatrics, Goztepe Training and Research Hospital, Istanbul, Turkey muferete@yahoocomFAU - Okumus, Ozgur
    Abstract:Objective: To investigate growth, development and bone mineralization of children with juvenile idiopathic arthritis (JIA). Methods: Thirty patients between 4-17 years of age (mean 11.34 +/- 3.88) resistant to therapy were studied. Enrollment began in November 1999 and continued through November 2004 and children with chronic disease were excluded. Data like height, weight, medications and acute phase reactants were obtained from medical records. On study-visit, puberty was assessed by physical examination and bone mineral density (BMD) was measured. Serum Ca, P, ALP, insulin-like growth factor-1 (IGF-1) and urinary Ca/Cr and hydroxyproline /Cr levels were measured. Results were compared with the control group that consisted of 30 cases of similar age and gender. Results: Patients with JIA had decreased height standard deviation score (SDS) and growth retardation. BMD of the cases in the study group was lower than the control group (p< 0.05). Patients who were at younger age at the onset of the disease had lower BMD. Among the drugs, only steroids had a negative effect on growth. Serum IGF-1 levels of the study group were significantly lower than the control group (p< 0.0001). Conclusion: Early diagnosis and suppression of disease activity is important in prevention of osteoporosis and growth retardation in children with JIA. BMD has to be measured yearly in patients for accurate diagnosis of osteoporosis. Vitamin D and Ca-rich nutrition with promotion of physical activity and controlled use of steroids may protect the children against bone loss

41. OTERMIN I, ELIZONDO G, ZABALETA Jet AMIGOT A: [Etanercept and pregnancy], An.Sist.Sanit.Navar., Vol. 30(3), 491-493., 2007
    Organism:Servicio de Medicina Interna, Clinica Ubarmin, Elcano, Navarra, Spain inakioterminmaya@cfnavarraesFAU - Otermin, I
    Abstract:We present the case of a pregnant woman with arthritis in treatment with etanercept. After becoming pregnant she continued treatment with standard doses of this drug, 25 mg twice a week, without complications

42. OW ATet CHEUNG LK: Meta-analysis of mandibular distraction osteogenesis: clinical applications and functional outcomes, Plast.Reconstr.Surg., Vol. 121(3), 54e-69e., 2008
    Organism:Discipline of Oral and Maxillofacial Surgery, Faculty of Dentistry, University of Hong Kong, Prince Philip Dental Hospital, Hong Kong, SARFAU - Ow, Andrew T C
    Abstract:BACKGROUND: Mandibular distraction osteogenesis has been used effectively to treat syndromic craniofacial deformities. In recent years, its scope of application has widened to include treatment of airway obstruction in adults and children and nonsyndromic class II mandibular hypoplasia. So far, there has been no evidence-based review of mandibular distraction osteogenesis for mandibular lengthening. METHODS: Two rounds of searches were performed by two independent assessors. The first-round PubMed search used the keywords "mandible" and "distraction osteogenesis." In the second-round search, the reference lists of the articles were retrieved. For both rounds, abstracts and then full articles were reviewed and selected on the basis of a set of inclusion and exclusion criteria. RESULTS: The 178 retrieved articles yielded 1185 mandibular distraction osteogenesis patients: 539 received unilateral mandibular distraction osteogenesis and 646 received bilateral mandibular distraction osteogenesis. Mandibular distraction osteogenesis was reported to improve facial asymmetry and retrognathia (50.1 percent), correct the slanted lip commissure (24.7 percent), and improve or level the mandibular occlusal plane (11.1 percent) in unilateral asymmetry cases, whereas bilateral mandibular distraction osteogenesis was shown to be effective in preventing tracheostomies for 91.3 percent of neonates or infants with respiratory distress, and in relieving symptoms of obstructive sleep apnea for 97.0 percent of children and 100 percent of adult patients. CONCLUSIONS: Mandibular distraction osteogenesis is effective in treating craniofacial deformities, but further clinical trials are required to assess the long-term stability and to compare the treatment with conventional treatment methods, especially in cases of obstructive sleep apnea or class II mandibular hypoplasia

43. 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

44. RUSSO RA, ROSENZWEIG SDet KATSICAS MM: Hepatitis A-associated macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: report of 2 cases, J.Rheumatol., Vol. 35(1), 166-168., 2008
    Organism:Servicio de Inmunologia y Reumatologia, Hospital de Pediatria Prof Dr Juan P Garrahan, Buenos Aires, ArgentinaFAU - Russo, Ricardo A G
    Abstract:OBJECTIVE: We describe two 3-year-old patients with systemic juvenile idiopathic arthritis (SJIA) who developed hepatitis A-associated macrophage activation syndrome (MAS). One patient showed MAS as the presenting manifestation of SJIA, while MAS complicated SJIA during the second year of the disease course in the other child. Both girls presented with fever, jaundice, hepatosplenomegaly, neurological involvement, mucosal hemorrhage, and purpura. Cytopenias, hypofibrinogenemia, and hemophagocytosis confirmed the diagnosis. After aggressive treatment with high-dose corticosteroids and immunosuppressants one patient entered remission while the other one died. Hepatitis A virus may induce severe MAS in SJIA

45. SHANAHAN EM, SMITH M, ROBERTS-THOMSON L, ESTERMAN Aet AHERN M: Influence of rheumatoid arthritis on work participation in Australia, Intern.Med.J., Vol. 38(3), 166-173., 2008
    Organism:Flinders University, and Rheumatology Research Unit, Repatriation General Hospital, Adelaide, South Australia, Australia michaelshanahan@rghsagovauFAU - Shanahan, E M
    Abstract:BACKGROUND: The aim of the study was to determine the prevalence of work disability in a cohort of Australians with rheumatoid arthritis. METHODS: A cross-sectional study of a sample of 497 individuals aged 18-65 years with rheumatoid arthritis in Adelaide, South Australia, was carried out. RESULTS: Of those employed, 130 (51%) were in full-time employment (> or= 35 h per week) work and 124 (49%) were in part-time employment (average 20 h per week). Overall, the observed/expected numbers working were 254/316 (relative risk 0.8 (0.69-0.91)). Using a comparator adjusted by removing those on the disability support pension, the relative risk of the working was 0.74. The observed/expected numbers working part time in the study group were 124/89 (relative risk 1.4 (1.25-1.65)). Those who continued to work had lower Health Assessment Questionnaire scores, less morning stiffness, superior scores for patient assessed level of function, lower pain scores, lower joint counts, a lower C-reactive protein, better measures of 'patient global assessment' and higher levels of education compared with the group who had ceased work. Overall, of those working at the time of diagnosis, 20% had ceased work within 5 years and approximately 40% had ceased work by 20 years. Of those who ceased work, the mean duration from time of diagnosis to work cessation was 7 years with half the subjects who ceased work doing so within 4 years of diagnosis. CONCLUSION: Work disability associated with rheumatoid arthritis in Australia is very significant and costly. Work disability occurs relatively early in the disease and is associated with several identifiable variables, many of which may be amenable to intervention strategies

46. SHISHOV M, HENRICKSON M, BURGOS-VARGAS R, RUBIO-PEREZ N, BACA V, ROMERO-FEREGRINO R, SOLIS-VALLEJO E, HUANG B, GROM AAet LOVELL DJ: Systemic features and early prognostic factors in Hispanic and non-Hispanic children from the United States of America and Mexico with systemic juvenile idiopathic arthritis, Clin.Exp.Rheumatol., Vol. 25(6), 907-914., 2007
    Organism:Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA mshishov@phoenixchildrenscomFAU - Shishov, M
    Abstract:OBJECTIVE: To investigate if the persistence of systemic features is longer in Hispanic children with systemic juvenile idiopathic arthritis (S-JIA) than in non-Hispanic children with S-JIA and to determine early predictors of systemic and articular disease. METHODS: We performed a multi-center retrospective chart review of patients followed in six pediatric rheumatology centers with onset of S-JIA from 1974 to 2004. Patients were included in the study if they had been followed for > or = 1 year after disease onset. Information collected included demographic, clinical, laboratory and treatment data. Systemic features included fever, rash, lymphadenopathy, hepatosplenomegaly, pericarditis, and pleuritis. RESULTS: Of the 159 S-JIA patients screened, 120 (75%) met our inclusion criteria. There were 65 boys and 55 girls. The mean follow-up period for Hispanic patients was 5.7 years (SD 4.0) and for non-Hispanic patients was 8.6 years (SD 7.2). There was no significant difference in the presence of systemic features between Hispanic and non-Hispanic patients at 0.5, 1, 2, 4, 6, 8, and 10 years of follow-up. Polyarthritis at the 6-month visit was predictive of systemic features (OR 9.7, 95% CI 1.16-81.35, p = 0.036) and polyarthritis (OR 5.6, 95% CI 1.42-21.8, p = 0.014) at last follow-up. CONCLUSION: In children with S-JIA, Hispanics did not demonstrate longer persistence of systemic features than non-Hispanics. Polyarthritis at 6 months strongly predicted the development of persistent systemic features and chronic polyarticular disease

47. SIDIROPOULOU-CHATZIGIANNI S, PAPADOPOULOS MAet KOLOKITHAS G: Mandibular condyle lesions in children with juvenile idiopathic arthritis, Cleft Palate Craniofac.J., Vol. 45(1), 57-62., 2008
    Organism:Department of Orthodontics, School of Dentistry, Aristotle University of Thessaloniki, Thessaloniki, Greece sonia@dentauthgrFAU - Sidiropoulou-Chatzigianni, Sossani
    Abstract:OBJECTIVE: To assess the prevalence of radiographically detectable destruction of the temporomandibular joints in children with juvenile idiopathic arthritis and to study the possible relationships between condylar destruction and type and duration of the disease, as well as the type of occlusion. MATERIAL AND METHOD: The study group consisted of 66 children with juvenile idiopathic arthritis (27 boys, 39 girls; mean age, 11.9 years). The possible presence of condylar destruction was examined in panoramic radiographs. The medical history and the type of malocclusion were registered also. The statistical evaluation was performed by means of descriptive statistics, Student's t test, Pearson's chi-square, and an analysis of variance test. The whole procedure was repeated after a 4-week interval to estimate the error of the method. RESULTS: Of the children with juvenile idiopathic arthritis, 50% showed some form of condylar destruction. Significant correlation was found between the type of the disease and the condyles affected. In the polyarticular type of juvenile idiopathic arthritis, 75% of the children presented affected condyles and 55.6% of them showed lesions bilaterally. The condylar affection was found to be independent of sex, although girls showed a tendency to bilateral lesions. In children with unilateral destruction, the right condyle was affected four times more frequently than the left. The duration of juvenile idiopathic arthritis seems to be significantly correlated to condylar destruction and especially to bilateral destruction. CONCLUSION: Children with juvenile idiopathic arthritis presented a remarkable prevalence of condylar destruction, which was correlated to the type and duration of the disease

48. SIMON D: Management of growth retardation in juvenile idiopathic arthritis, Horm.Res., Vol. 68 Suppl 5, 122-125., 2007
    Organism:Service d'Endocrinologie Pediatrique, Hopital Robert Debre, Paris, France Dominiquesimon@rdbaphpfrFAU - Simon, Dominique
    Abstract:BACKGROUND: Inflammation and glucocorticoid therapy are major factors in the growth retardation seen in children with severe forms of juvenile idiopathic arthritis (JIA). It has been recently shown that tumor necrosis factor (TNF)-alpha antagonist therapy can improve growth velocity in JIA patients; however, the recombinant human soluble TNF-alpha receptor fusion protein etanercept has had limited efficacy in systemic forms of JIA. For several years, growth hormone (GH) has been used to treat growth retardation in patients with JIA receiving glucocorticoids. GH treatment can normalize growth velocity and prevent the severe loss of height; however, catch-up growth markedly varies with the severity of the inflammatory state and the steroid doses used during GH treatment. Recently, early institution of GH treatment has been shown to maintain normal growth in children with JIA. CONCLUSIONS: These promising results show the need for careful monitoring of growth in children with JIA, the utility of GH therapy before the onset of severe growth delay and the potential for preservation of long-term growth during disease progression

49. SORENSEN LK, HAVEMOSE-POULSEN A, SONDER SU, BENDTZEN Ket HOLMSTRUP P: Blood Cell Gene Expression Profiling in Subjects With Aggressive Periodontitis and Chronic Arthritis, J.Periodontol., Vol. 79(3), 477-485., 2008
    Organism:* Department of Periodontology, School of Dentistry, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark, dagger Institute for Inflammation Research, Rigshospitalet National University Hospital, Copenhagen, Denmark
    Abstract:Background: Microarray analysis of local and peripheral cells in subjects with immune-inflammatory diseases may identify candidate genes associated with these diseases. The present study identified differentially expressed genes in peripheral blood mononuclear cells (PBMCs) from subjects with untreated localized aggressive periodontitis (LAgP) or generalized aggressive periodontitis (GAgP). Differentially expressed genes were validated in groups of subjects with LAgP, GAgP, juvenile idiopathic arthritis (JIA), or rheumatoid arthritis (RA) and controls. Methods: Candidate genes were identified by gene expression profiling of PBMCs using a microarray system in untreated gender-matched subjects with LAgP (N = 2) or GAgP (N = 3) and controls (N = 2) younger than 35 years of age. The microarray results were validated by real-time reverse transcription-polymerase chain reaction (RT-PCR) using PBMCs from 103 individuals, including groups of subjects with LAgP (N = 18), GAgP (N = 27), JIA (N = 10), or RA (N = 23) and controls (N = 25). Results: Of 53 differentially expressed candidate genes identified in subjects with LAgP, 14 were involved in immune responses and inflammatory processes. Of these, the RT-PCR validation confirmed that Toll-like receptor 2 gene (TLR2) and myomesin 2 gene had a significantly higher expression in subjects with LAgP than in controls. RT-PCR also showed increased expression of TLR2 in subjects with RA. Comparison of subjects with GAgP to controls using microarray analysis identified only three upregulated genes. Conclusion: Several genes upregulated in subjects with LAgP were related to immune responses including TLR2 and myomesin 2

50. STINSON JN, TOOMEY PC, STEVENS BJ, KAGAN S, DUFFY CM, HUBER A, MALLESON P, MCGRATH PJ, YEUNG RSet FELDMAN BM: Asking the experts: exploring the self-management needs of adolescents with arthritis, Arthritis Rheum., Vol. 59(1), 65-72., 2008
    Organism:University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada jenniferstinson@sickkidscaFAU - Stinson, Jennifer N
    Abstract:OBJECTIVE: To explore the self-management needs of adolescents with juvenile idiopathic arthritis and the acceptability of a Web-based program of self-management aimed at improving quality of life. METHODS: A descriptive qualitative design was used. A convenience sample of 36 adolescents (male and female) who varied in age, disease onset subtype, and disease severity were recruited from 4 Canadian tertiary care pediatric centers. Individual (n=25) and 3 focus-group (n=11) interviews were conducted with adolescents using semistructured interview guides. After each interview session, the audiotaped interview data were transcribed verbatim. NUD*IST 6.0 was used to assist with the sorting, organizing, and coding of the data. Data were organized into categories that reflected emerging themes. RESULTS: Adolescents articulated how they developed effective self-management strategies through the process of "letting go" from others who had managed their illness (health care professionals, parents) and "gaining control" over managing their illness on their own. The 2 strategies that assisted in this process were gaining knowledge and skills to manage the disease and experiencing understanding through social support. Five further subthemes emerged around skills to manage the disease, including knowledge and awareness about the disease, listening to and challenging care providers, communicating with the doctor, managing pain, and managing emotions. CONCLUSION: Adolescents were united in their call for more information, self-management strategies, and meaningful social support to better manage their arthritis. They believed that Web-based interventions were a promising avenue to improve accessibility and availability of these interventions

51. TER BRAAK BP, VINCKEN PW, VAN ERKEL AR, BLOEM RM, NAPOLEON LJ, COENE MN, VAN LUIJT PA, DE LANGE Set BLOEM JL: Are radiographs needed when MR imaging is performed for non-acute knee symptoms in patients younger than 45 years of age?, Skeletal Radiol., Vol. 36(12), 1129-1139., 2007
    Organism:Department of Radiology, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The NetherlandsFAU - Ter Braak, Bert P M
    Abstract:OBJECTIVE: The objective was to determine the value of radiographs in young adults with non-acute knee symptoms who are scheduled for magnetic resonance imaging (MRI). MATERIALS AND METHODS: Nine hundred and sixty-one consecutive patients aged between 16 and 45 years with knee symptoms of at least 4 weeks' duration were prospectively included in three participating hospitals. After applying exclusion criteria, 798 patients remained. Exclusion criteria were previous knee surgery (including arthroscopy) or MRI, history of rheumatoid arthritis, clinical diagnosis of retropatellar chondromalacia, contra-indication for MRI and recent trauma. We identified two groups: group A with no history of trauma (n = 332), and group B with an old (>4 weeks) history of trauma (n = 466). Patients had a standardized history taken, and underwent a physical exam, antero-posterior (AP) and lateral radiographs and MRI. We evaluated the radiographs and MRI for osseous lesions, articular surface lesions, fractures, osteoarthritis, loose bodies, bone marrow edema and incidental findings. Subsequently, patients with osseous abnormalities (Kellgren grade 1 and 2 excluded) on radiographs and a matched control group was evaluated again using MRI without radiographs. RESULTS: Median duration of symptoms was 20 weeks. In group A, radiographs showed 36 osseous abnormalities in 332 patients (10.8%). Only 13 of these, all Kellgren grade 1 osteoarthritis, were not confirmed on MRI. MRI showed 72 (21.7%) additional abnormalities not confirmed on radiographs. In group B, radiographs showed 40 osseous abnormalities (8.6%) in 466 patients. Only 15 of these, all Kellgren grade 1 osteoarthritis, were not confirmed on MRI. MRI showed 194 (41.6%) additional abnormalities not confirmed on radiographs. The second evaluation of MRI without radiographs in 34 patients was identical to the first MRI evaluation. Common lesions were significantly more often diagnosed with MRI than with radiographs. CONCLUSION: Radiographs should not be obtained routinely when MRI is being performed in young adults with non-acute knee complaints because the yield and added value to MRI are low

52. THORNTON J, BERESFORD MWet CLAYTON P: Improving the evidence base for treatment of juvenile idiopathic arthritis: the challenge and opportunity facing the MCRN/ARC Paediatric Rheumatology Clinical Studies Group, Rheumatology (Oxford)., Vol. ., 2008
    Organism:Greater Manchester, Lancashire and South Cumbria Medicines for Children Research Network, Royal Manchester Children's Hospital, Pendlebury, Manchester M27 4HA, Medicines for Children Research Network/Arthritis Research Campaign Paediatric Rheumatology Clinical Studies Group, University of Liverpool Division of Child Health, Royal Liverpool Children's Hospital, Alder Hey, Liverpool L12 2AP and Department of Child Health and Paediatric Endocrinology, University of Manchester, Manchester, UK
    Abstract:

53. TING TVet HASHKES PJ: Methotrexate/naproxen-associated severe hepatitis in a child with juvenile idiopathic arthritis, Clin.Exp.Rheumatol., Vol. 25(6), 928-929., 2007
    Organism:Section of Pediatric Rheumatology, Department of Rheumatic Diseases, Cleveland Clinic Foundation, Cleveland, OH 44195, USAFAU - Ting, T V
    Abstract:Methotrexate (MTX) is a cornerstone in the treatment of juvenile idiopathic arthritis (JIA). Although associated with many mild adverse effects, the short and long-term safety of MTX in JIA has been excellent. While many JIA children treated with MTX develop liver enzyme abnormalities, no cases of irreversible liver damage or of severe non-infectious hepatitis with Reye-like features have been reported in non-systemic JIA. We report a 2-year-old girl with oligoarthritis whose liver enzyme increased to greater than 45 times the upper limit of normal, and developed hypoglycemia and hyperammonemia after 10 months of MTX and naproxen therapy. An infectious and metabolic work-up for other causes was unremarkable. She recovered completely after folinic acid therapy; MTX and naproxen was not restarted. While very rare in JIA, MTX in synergism with naproxen can induce severe liver toxicity and it is important to screen children for liver enzyme abnormalities

54. TOLL ML, LIO P, SUNDEL RPet NIGROVIC PA: Comparison of Vancouver and International League of Associations for rheumatology classification criteria for juvenile psoriatic arthritis, Arthritis Rheum., Vol. 59(1), 51-58., 2008
    Organism:Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USAFAU - Toll, Matthew L S
    Abstract:OBJECTIVE: The International League of Associations for Rheumatology (ILAR) criteria constitute the current international diagnostic standard for juvenile psoriatic arthritis (PsA), replacing the less-restrictive Vancouver criteria. The impact of this change on the population diagnosed with juvenile PsA is unknown. METHODS: We reviewed the records of patients seen in a pediatric rheumatology clinic with International Classification of Diseases, Ninth Revision diagnosis codes for psoriasis, PsA, or spondylarthritis. Characteristics of children who met the Vancouver and ILAR criteria were compared. RESULTS: Of 139 children meeting the Vancouver criteria for juvenile PsA, ILAR criteria excluded 80 (58%). Grounds for exclusion were insufficiently definitive rash (44%), a competing diagnosis of enthesitis-related arthritis (23%), family history of psoriasis limited to second-degree relatives (16%), fulfillment of criteria for >1 subtype of juvenile idiopathic arthritis (JIA) (5%), and HLA-B27 in a male with arthritis onset after age 6 (2%). Remaining patients were not homogeneous but could be divided into younger and older subpopulations differing in clinical features as described previously among patients identified under the Vancouver standard. Of excluded patients, 76% were reclassified as having other forms of JIA yet were phenotypically comparable with those retained. CONCLUSION: Despite apparently modest changes from previous criteria, ILAR definitions strikingly restrict the diagnosis of PsA in childhood. Similarity between excluded and included patients suggests that these restrictions may not reflect substantive clinical differences. To the extent that excluded patients become reclassified within JIA, current criteria risk compromising other ILAR categories while reducing the number of patients available for the study of juvenile PsA

55. TOMA CD, MACHACEK P, BITZAN P, ASSADIAN O, TRIEB Ket WANIVENHAUS A: Fusion of the wrist in rheumatoid arthritis: a clinical and functional evaluation of two surgical techniques, J.Bone Joint Surg.Br., Vol. 89(12), 1620-1626., 2007
    Organism:Department of Orthopaedic SurgeryFAU - Toma, C D
    Abstract:We retrospectively compared wrist arthrodesis using the Mannerfelt technique in 19 or an AO-plate in 23 patients with long-standing rheumatoid arthritis. The mean follow-up was for 76 months. Compared with the Mannerfelt fusion group, patients in the AO-plate group reported greater satisfaction with their wrist function (74% vs 37%, p = 0.015). Complications were reported in six wrists in the AO-plate group and two wrists in the Mannerfelt fusion group (p = 0.258). At final follow-up, 95% of patients (41) reported either no pain or only mild pain. There was improvement in flexion of the finger joints in both groups but no significant improvement in the extension lag in either group. Both methods relieve pain and improve function. Overall, the activities of daily living scores and the patients' subjective assessment of outcome tended to be higher in the AO-plate group than in the Mannerfelt fusion group, although the difference was not statistically significant. Similarly, although more postoperative complications occurred in the AO-plate group, the difference between the two groups was not statistically significant

56. TYNJALA P, KOTANIEMI K, LINDAHL P, LATVA K, AALTO K, HONKANEN Vet LAHDENNE P: Adalimumab in juvenile idiopathic arthritis-associated chronic anterior uveitis, Rheumatology (Oxford)., Vol. 47(3), 339-344., 2008
    Organism:Department of Pediatric Rheumatology, Hospital for Children and Adolescents, Helsinki, Finland pirjotynjala@husfiFAU - Tynjala, P
    Abstract:OBJECTIVE: To evaluate the efficacy of adalimumab in juvenile idiopathic arthritis (JIA)-associated uveitis. METHODS: Retrospective observational study of 20 patients with JIA and chronic uveitis on adalimumab treatment. The ocular inflammation and improvement was assessed according to the Standardization of Uveitis Nomenclature criteria. RESULTS: At the initiation of adalimumab, the mean age of patients was 13.4 yrs and the mean duration of uveitis 8.7 yrs. Seventeen (85%) patients had polyarticular JIA and 19 (95%) had previously been on anti-TNF treatment. The mean duration of adalimumab therapy was 18.7 months. Of the 20 patients, 7 (35%) showed improved activity, 1 (5%) worsening activity and in 12 (60%) no change was observed in the activity of uveitis. Those with improved activity were younger and had shorter disease duration. The mean number of flares/yr decreased from 1.9 to 1.4 during adalimumab treatment. Serious adverse events or side-effects were not observed. Seven patients discontinued adalimumab during the follow-up: six because of inefficacy and one because of inactive uveitis. CONCLUSION: Adalimumab is a potential treatment option in JIA-associated uveitis, even in patients non-responsive to previous other anti-TNF therapy

57. TZARIBACHEV N, KUEMMERLE-DESCHNER J, EICHNER Met HORNEFF G: Safety and efficacy of etanercept in children with juvenile idiopathic arthritis below the age of 4 years, Rheumatol.Int., Vol. ., 2008
    Organism:University Children's Hospital, Hoppe-Seyler Str 1, 72076, Tuebingen, Germany, nikolaytzaribachev@meduni-tuebingende
    Abstract:Little is known about the safety and efficacy of etanercept in children below the age of 4 years. Twenty-five patients with juvenile idiopathic arthritis (JIA) below the age of 4 years, who received etanercept, were documented in the German JIA Etanercept Registry. Patients with the nonsystemic JIA disease-subtype responded more frequently to treatment with etanercept than systemic onset patients. At last observation, two (20%) of the nonsystemic patients did not reach a PedACR 30 response, while six (40%) of the systemic onset patients did not respond to the therapy. Complete resolution of symptoms was observed in two (20%) nonsystemic and in five (33%) systemic onset patients with JIA. Tolerability was good with only two infections occurring in the total patients group. Patients with JIA below the age of 4 years, with a methotrexate-resistant disease course, would also benefit from treatment with etanercept, showing a good tolerability. The restriction to children older than 4 years appears to be artificial without any good rationale

58. VAGLIO A, PALMISANO A, FERRETTI S, ALBERICI F, CASAZZA I, SALVARANI Cet BUZIO C: Peripheral inflammatory arthritis in patients with chronic periaortitis: report of five cases and review of the literature, Rheumatology (Oxford)., Vol. 47(3), 315-318., 2008
    Organism:Department of Clinical Medicine, Nephrology and Health Science, University of Parma, Parma, Italy augustovaglio@virgilioitFAU - Vaglio, A
    Abstract:OBJECTIVES: Chronic periaortitis (CP) is a rare disease with a potentially immune-mediated pathogenesis. The study aims to report the frequency and the clinical characteristics of peripheral inflammatory arthritis in a cohort of CP patients, and to review the literature regarding the association between arthritis and CP. METHODS: Forty-nine consecutive CP patients were seen at our department between 2000 and 2006; all of them underwent imaging (abdominal computed tomography and magnetic resonance imaging) and laboratory examinations, also including erythrocyte sedimentation rate, C-reactive protein and a panel of autoantibodies. The clinical history of the patients who developed peripheral inflammatory arthritis is reported in detail. A PubMed/Medline search without any date limits was performed for English-language articles reporting the association between CP and arthritis. RESULTS: Five of the 49 enrolled patients developed an inflammatory form of peripheral arthritis: three were diagnosed as having RA, one palindromic rheumatism and one acute reactive arthritis. In all but one case, arthritis became clinically overt months to years after the onset of CP, and its outcome was good, since almost all patients were asymptomatic at the end of follow-up. No patient suffered from ankylosing spondylitis. In the literature review, 20 cases of CP-associated arthritis were found, mainly in the form of case reports: 14 of them were spondyloarthropathies, whereas the remaining ones were RA, juvenile RA or undifferentiated arthritis. CONCLUSIONS: Peripheral inflammatory arthritis, particularly RA or RA-like forms, may develop in CP patients. This overlap strengthens the hypothesis of an autoimmune origin of CP

59. VEIT TD, VIANNA P, SCHEIBEL I, BRENOL C, BRENOL JC, XAVIER RM, DELGADO-CANEDO A, GUTIERREZ JE, BRANDALIZE AP, SCHULER-FACCINI Let CHIES JA: Association of the HLA-G 14-bp insertion/deletion polymorphism with juvenile idiopathic arthritis and rheumatoid arthritis, Tissue Antigens., Vol. ., 2008
    Organism:Genetics Department, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil
    Abstract:We tested the possible association of the 14-bp polymorphism of the HLA-G gene in the course of two inflammatory diseases, rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA). Patients and controls were genotyped for the 14-bp polymorphism by polymerase chain reaction with specific primers for the exon 8 of the human leukocyte antigen (HLA)-G gene and the amplified fragment was visualized in a 6% polyacrylamide gel. A total of 106 JIA patients, 265 RA patients, 356 healthy adults and 85 healthy children were genotyped for the 14-bp polymorphism. Female JIA patients presented a higher frequency of the -14 bp allele when compared with female healthy children (0.743 and 0.500, corrected P = 0.003), which reflected in the JIA group as a whole. This increased frequency of the -14-bp allele was observed in all JIA subtypes. In RA patients, no differences in allelic and genotypic frequencies were observed between patients and controls. No correlations were observed among genotype and disease severity or clinical manifestations. Our data suggest that the HLA-G -14 bp allele is probably a risk factor for JIA, mainly in females. Considering the differences observed in relation to gender, we suggest that hormonal differences can interfere with the development of JIA. Considering the RA patients, our data agree with results from the literature and highlight the differences in the etiology of RA and JIA

60. WEI CM, LEE JH, WANG LC, YANG YH, CHANG LYet CHIANG BL: Frequency and phenotypic analysis of CD4(+)CD25(+) regulatory T cells in children with juvenile idiopathic arthritis, J.Microbiol.Immunol.Infect., Vol. 41(1), 78-87., 2008
    Organism:Department of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanFAU - Wei, Chih Ming
    Abstract:BACKGROUND AND PURPOSE: The aim of this study was to investigate the frequency of CD4(+)CD25(+) regulatory T cells and their phenotypic expression in peripheral blood of children with active and non-active juvenile idiopathic arthritis (JIA) and healthy controls, to determine if their frequency or phenotypic expression is involved in the immunoregulation of this disease. METHODS: From October 2004 to October 2005, 55 JIA patients and 55 age- and gender-matched healthy controls were enrolled in the study at National Taiwan University Hospital. Flow cytometry was used to determine the frequency of CD4(+)CD25(+) and CD4(+)CD25(hi) in CD4(+) T cells and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) expression on CD4(+)CD25(+) by tricolor staining. Basic profiles, medication history, clinical symptoms and laboratory data were obtained by chart review and outpatient department interviews. RESULTS: There was no significant difference in expression of CD4(+)CD25(+) T cells between patients with inactive JIA and normal controls (13.74 +/- 3.25% vs 12.85 +/- 3.68%, p>0.05). The expression of CD4(+)CD25(hi) T cells was significantly lower in inactive JIA patients than in normal controls (1.89 +/- 1.01% vs 2.76 +/- 1.28%, p<0.01). The expression of CTLA-4 on CD4(+)CD25(+) T cells was also significantly lower in inactive JIA patients compared with controls (4.37 +/- 2.02% vs 6.33 +/- 2.57%, p<0.001). CONCLUSION: We speculate that a decreased frequency of CD4(+)CD25(hi) regulatory T cells and lower level of CTLA-4 expression on CD4(+)CD25(+) regulatory T cells might play a role in the immunoregulation of JIA

61. WOLLINA U, HANSEL G, KOCH A, SCHONLEBE J, KOSTLER Eet HAROSKE G: Tumor necrosis factor-alpha inhibitor-induced psoriasis or psoriasiform exanthemata: first 120 cases from the literature including a series of six new patients, Am.J.Clin.Dermatol., Vol. 9(1), 1-14., 2008
    Organism:Departments of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Dresden, Germany wollina-uw@khdfdeFAU - Wollina, Uwe
    Abstract:Tumor necrosis factor-alpha (TNFalpha) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients from the literature including six new patients (three women and three men) who developed pustular lesions during treatment with TNFalpha inhibitors. We identified 72 women and 36 men (several papers did not specify the gender of patients) with an age range of 13-78 years (mean 42.3 years). The primary diagnoses were rheumatoid arthritis (n = 61), ankylosing spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFalpha treatment (n = 73), followed by palmoplantar pustular psoriasis (n = 37) and psoriasis of the nail (n = 6), sometimes combined in the same patient. Palmoplantar pustulosis and psoriasiform exanthema was the diagnosis in ten patients each. A positive personal history of psoriasis was recorded in 25 patients. A positive family history was noted in eight patients. No data about personal (n = 7) or family history (n = 46) were available in a number of patients. Newly induced psoriasis was diagnosed in 74 patients whereas an exacerbation or aggravation of a pre-existing psoriasis was noted in another 25 patients. All three TNFalpha inhibitors available on the market were involved: infliximab (63 patients), etanercept (37 patients), and adalimumab (26 patients). Several patients were treated with more than a single TFNalpha inhibitor. The timing of cutaneous adverse effects (psoriasis and psoriasiform rash) varied considerably among patients, ranging from after a single application to a delayed response of up to 63 months after initiation of treatment. The mean time to appearance of the cutaneous adverse effect for all TNFalpha inhibitors was 9.5 months. Cessation of the responsible TNFalpha inhibitor was carried out in 47 patients either alone or in association with adjuvant anti-psoriatic therapy (mostly topical). This resulted in complete remission in 21 patients, partial remission in 20 patients, and stable disease in another three patients; in the other three patients, the outcome was not reported. TNFalpha inhibition was continued in 47 patients but anti-psoriatic adjuvant therapy was introduced. The outcome in this group was complete remission in 22 patients, partial remission in 25 patients, and stable disease in 2 patients. The response rate (complete remission plus partial remission) was 93.2% and 95.9%, respectively, in each group. In six patients, switching from one TNFalpha inhibitor to another one immediately after cutaneous adverse effects occurred resulted in an improvement in five patients. In nine patients, a second TNFalpha inhibitor was initiated after a break in TNFalpha inhibition. The response to a second or third drug in these patients was mixed. The underlying pathomechanisms of induction of psoriasis or psoriasiform exanthemata by TNFalpha inhibitors remain elusive but there is reason to assume that induction of such adverse events has more than one pathophysiology

62. WOO P: Anakinra treatment for systemic juvenile idiopathic arthritis and adult onset Still disease, Ann.Rheum.Dis., Vol. 67(3), 281-282., 2008
    Organism:

63. YILMAZ M, KENDIRLI SG, ALTINTAS DU, KARAKOC GB, INAL Aet KILIC M: Juvenile idiopathic arthritis profile in Turkish children, Pediatr.Int., Vol. 50(2), 154-158., 2008
    Organism:Division of Pediatric Allergy and Immunology, Cukurova University Faculty of Medicine, Adana, Turkey
    Abstract:Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders. Publications from different countries point to differences in the disease manifestation of JIA among different populations. The aim of the present paper was to evaluate the clinical and laboratory features of JIA in Turkish children. Methods: A total of 196 JIA patients who fulfilled International League of Associations for Rheumatology (ILAR) diagnostic criteria were included in this retrospective study. The data collected were age, gender, age at disease onset and at diagnosis, and follow-up duration. Antinuclear antibody (ANA), rheumatoid factor (RF), and human leukocyte antigen B-27 were evaluated for each patient. Results: There were 102 boys and 94 girls with a mean duration of disease of 4.1 years. The mean age at the first visit was 8.8 years, and the mean age at onset of disease was 6.8 years (range, 8 months-15 years). Polyarticular JIA was the most frequent onset type (37.2%). Other subtypes included oligoarthritis (34.2%), systemic arthritis (15.3%), psoriatic arthritis (1%), enthesitis-related arthritis (9.7%), and other arthritis (2.2%). ANA was positive in 28 patients (14.2%). Chronic uveitis occurred in two patients with oligoarthritis; and two patients with enthesitis-related arthritis had acute uveitis. Three patients (1.4%) developed amyloidosis. Conclusion: Compared to reports from Western countries, remarkably different features of JIA were found in Turkish children, which included higher frequency of polyarticular JIA, higher prevalence among boys, lower rate of ANA positivity and uveitis. Further studies are required to understand how genetic and environmental differences affect JIA expression

64. 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

65. ZHAO Yet CAO LF: [A case report of macrophage activation syndrome complicated by systemic onset juvenile idiopathic arthritis], Zhongguo Dang.Dai Er.Ke.Za Zhi., Vol. 9(6), 610, 2007
    Organism:Department of Pediatrics, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, ChinaFAU - Zhao, Yu
    Abstract:

66. ZULIAN F: Systemic sclerosis and localized scleroderma in childhood, Rheum.Dis.Clin.North Am., Vol. 34(1), 239-255., 2008
    Organism:Department of Pediatrics, Pediatric Rheumatology Unit, University of Padova, Via Giustiniani 3, 35128 Padova, ItalyFAU - Zulian, Francesco
    Abstract:Juvenile scleroderma syndromes, including the systemic and the localized varieties, represent the third most frequent chronic rheumatic conditions in pediatric rheumatology practice. In children, systemic sclerosis shows a significantly less frequent involvement of all organs, a higher prevalence of arthritis and myositis, and a better outcome than in adults. Recently, new classification criteria were proposed, which help improve patient care by enabling earlier, more definite diagnoses and by standardizing the conduct of clinical trials. Localized scleroderma is the more frequent subtype of scleroderma in childhood. It comprises a group of distinct conditions that involve mainly the skin and subcutaneous tissues. They range from small plaques of fibrosis involving only the skin to diseases causing significant functional deformity with various extracutaneous features

67. ZUNNUNOV ZR, IBRAGIMOVA SN, ZUNNUNOVA SZet SHOMURODOV NV: [Ammotherapy of rheumatoid arthritis], Vopr.Kurortol.Fizioter.Lech.Fiz Kult., Vol. (6), 25-27., 2007
    Organism: