Bibliography December 2007

1. Anonymous, Intra-articular triamcinolone: new indication. Juvenile idiopathic arthritis: several months of relief, Prescrire.Int., Vol. 16(92), 242, 2007
   Organism:Effective in more than three-quarters of children for at least 6 months after the injection. Caution about the risk of systemic adverse effects, especially in cases of repeated use

2. AKINCI A, CAKAR N, UNCU N, KARA Net ACAROGLU G: Keratoconjunctivitis sicca in juvenile rheumatoid arthritis, Cornea., Vol. 26(8), 941-944., 2007
   Organism:Department of Pediatric Ophthalmology, Diskapi Children's Hospital, Ankara, Turkey arsenakinci@yahoocomFAU - Akinci, Arsen
   Abstract:PURPOSE: To compare the symptoms, signs, and results of objective tests for keratoconjunctivitis sicca (KCS) in patients with juvenile rheumatoid arthritis (JRA) and controls. METHODS: Sixty-four patients with JRA and 64 age- and sex-matched controls were compared in terms of symptoms, signs, and results of objective tests for KCS. Relation between tear film breakup time (TBUT), Schirmer test results, and JRA-related variables such as age of onset, duration, and type of JRA; presence of antinuclear antibodies (ANAs); and history of uveitis were evaluated. Analysis of variance, multivariate regression analysis, Kruskall-Wallis, Student t tests, and chi tests were used for statistical analysis. RESULTS: Twelve and a half percent of patients with JRA complained of dry eye symptoms compared with 1.5% of the controls (P = 0.031). Dry eye signs were detected in 10.9% of patients with JRA compared with 1.5% of controls (P = 0.038). TBUT and Schirmer test results were lower in the JRA group than in controls (P = 0.032 and P = 0.029, respectively). Seven patients (10.9%) had definite and 1 (1.5%) had probable diagnosis of KCS in the JRA group compared with no children in the control group (P = 0.034). Within the JRA group, Schirmer test and TBUT results were significantly lower in male patients and ones with longer duration of disease. CONCLUSIONS: The prevalence of symptoms, signs, and definite diagnosis of KCS is higher and basal tear secretion and tear film stability are lower in children with JRA than in controls. Among children with JRA, male sex and longer duration of disease are independent risk factors for having decreased basal tear secretion and tear film stability

3. ALIZADEH BZ, VALDIGEM G, COENEN MJ, ZHERNAKOVA A, FRANKE B, MONSUUR A, VAN RIEL PL, BARRERA P, RADSTAKE TR, ROEP BO, WIJMENGA Cet KOELEMAN BP: Association analysis of functional variants of the FcgRIIa and FcgRIIIa genes with type 1 diabetes, celiac disease and rheumatoid arthritis, Hum.Mol.Genet., Vol. 16(21), 2552-2559., 2007
   Organism:Complex Genetics Section, Department of Medical Genetics, University Medical Center Utrecht, Utrecht, The Netherlands bzalizadeh@umcutrechtnlFAU - Alizadeh, Behrooz Z
   Abstract:FcgRIIa and FcgRIIIa are potent modulators of the immune system which bind (auto)antibodies and activate immune cells. The FcgRIIa*A519G and FcgRIIIa*A559C functional variants have been associated with several immune-related diseases. We studied FcgRIIa*A519G and FcgRIIIa*A559C SNPs in type 1 diabetes (T1D), celiac disease (CD) and rheumatoid arthritis (RA) patients and controls and included a meta-analysis of all recent studies of FcgRIIIa*A559C and RA. Our cohorts comprised 350 T1D, 519 CD, 639 RA patients and 1359 controls, who were genotyped for FcgRIIa*A519G and FcgRIIIa*A559C variants. Regression and expectation maximization (EM) algorithm-based haplotype analyses were used for the data analysis. We found significant differences in genotype frequencies of FcgRIIa between controls and patients with T1D (P = 0.04), CD (P = 0.000005) and RA (P = 0.04). The FcgRIIa*519GG genotype showed an increased risk for both T1D [odds ratio (OR) = 1.51; 95% confidence interval (95% CI) 1.08-2.12; P = 0.015] and CD (OR = 1.81; 95% CI 1.35-2.37; P = 0.000004), but not for RA. There was no difference in the frequency of FcgRIIIa*A559C genotypes or allelotypes between controls with T1D, CD and RA. We found that FcgRIIa and FcgRIIIa haplotype frequencies differed significantly between controls and patients with T1D (P = 0.05) and with CD (P = 0.00038) but not with RA. Our meta-analysis showed a significant 1.37(95% CI 1.14-1.66)-fold increased risk of RA for the FcgRIIIa*559CC (158VV) genotype (P = 0.001). This is the first report that the FcgRIIa*519GG genotype predisposes to T1D and CD. We confirmed that the FcgRIIIa*559CC genotype is associated with RA. If replicated, our findings would suggest FcgRIIa*519G as a common risk factor for auto-immune diseases. This may have clinical implications with regard to efficacy or safety of antibody-based immuno-modulator therapies

4. AMER R, BAMONTE Get FORRESTER JV: Resolution of juvenile idiopathic arthritis-associated uveitis after development of common variable immunodeficiency, Eur.J.Ophthalmol., Vol. 17(4), 666-668., 2007
   Organism:Department of Ophthalmology, Aberdeen Royal Infirmary, Foresterhill, Scotland, UK radgondeamir@abdnacukFAU - Amer, R
   Abstract:PURPOSE: To describe the occurrence of common variable immunodeficiency (CVID) in a patient with juvenile idiopathic arthritis (JIA) and JIA-associated uveitis. METHODS/RESULTS: Case report. A 29-year-old woman was followed-up since the age of 10 years because of right eye JIA-associated recurrent anterior uveitis. She was treated with steroids and immunosuppressants with good control of uveitis and arthritis. At the age of 17 years, she did not experience any further relapse of uveitis or arthritis and both diseases were considered to be in remission. Concomitantly, she started to have recurrent infections and later she underwent splenectomy because of autoimmune hemolytic anemia and thrombocytopenia. Liver biopsy disclosed granulomatous hepatitis. She was ultimately diagnosed with CVID at the age of 23 years when her blood tests revealed neutropenia and severe panhypogammaglobulinemia. She has been treated since then with intravenous immunoglobulins with good control of the disease. Since the development of CVID, she has had no relapses of uveitis or arthritis during a follow-up period of 12 years. CONCLUSIONS: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency where defective antibody formation is the most common feature with B-cell differentiation failure. Ocular complications have been rarely documented and included bacterial conjunctivitis, retinal vasculitis and multifocal choroiditis. We herein report on the occurrence of JIA-associated uveitis as a comorbid manifestation of CVID. We speculate a role for B cells in the pathogenesis of JIA and JIA-associated uveitis here, as this patient had total remission of both conditions with the onset of CVID

5. AROSTEGUI JI, ARNAL C, MERINO R, MODESTO C, ANTONIA CM, MORENO P, GARCIA-CONSUEGRA J, NARANJO A, RAMOS E, DE PAZ P, RIUS J, PLAZA Set YAGUE J: NOD2 gene-associated pediatric granulomatous arthritis: clinical diversity, novel and recurrent mutations, and evidence of clinical improvement with interleukin-1 blockade in a Spanish cohort, Arthritis Rheum., Vol. 56(11), 3805-3813., 2007
   Organism:Servicio de Inmunologia, Hospital Clinic, Barcelona, Spain jiaroste@clinicubesFAU - Arostegui, Juan I
   Abstract:OBJECTIVE: Blau syndrome and early-onset sarcoidosis are NOD2 gene-associated chronic autoinflammatory diseases characterized by skin rash, arthritis, and/or eye involvement, with noncaseating granulomata as their pathologic hallmark. This study was undertaken to describe the expanded clinical phenotype, treatment outcomes, and NOD2 gene mutation analysis in a Spanish cohort with pediatric granulomatous arthritis, a chronic disease resembling Blau syndrome/early-onset sarcoidosis. METHODS: Clinical, laboratory, and treatment data on the 12 patients in the cohort were obtained through direct interviews. NOD2 gene analysis was performed in a central laboratory, by bidirectional sequencing. Cytokine levels were measured using the human Flex-Set cytokine bead array. RESULTS: The classic Blau syndrome/early-onset sarcoidosis triad of skin rash, arthritis, and recurrent uveitis was identified in 5 patients (41.7%), whereas 7 patients (58.3%) presented with fewer than 3 of the classic features. Novel atypical manifestations such as persistent fever and myocardiopathy were also observed. NOD2 analysis revealed 1 heterozygous mutation in each patient, and familial studies confirmed its full penetrance. Of the 12 cases, 58.3% were sporadic, due to de novo mutations. Four different missense mutations on exon 4 were detected. Two of them (R334W and R334Q) were recurrent mutations and were found in 77.8% of the Spanish families, whereas the other 2 (C495Y and R587C) were novel. In the patient who received anakinra treatment, all clinical inflammatory symptoms improved and plasma cytokine levels normalized. CONCLUSION: These findings indicate that the expanding clinical heterogeneity of the disease (that is, the presentation of incomplete forms of the classic triad and atypical manifestations) and the high prevalence of sporadic cases should alert clinicians to the possible genetic basis of the condition and support the inclusion of DNA analysis as a diagnostic test. The positive response to anakinra observed in 1 patient suggests a new potential therapeutic approach that merits further investigation, and suggests that the pathogenesis of pediatric granulomatous arthritis may involve interleukin-1-mediated events

6. BARTNICKA M, GORSKA A, URBAN Met GORSKI S: [Growth disorders in the course of chronic juvenile arthritis], Endokrynol.Diabetol.Chor.Przemiany.Materii.Wieku.Rozw., Vol. 13(3), 116-119., 2007
   Organism:Zaklad Medycyny Rodzinnej i Pielegniarstwa Srodowiskowego AM w BialymstokuFAU - Bartnicka, Marta
   Abstract:INTRODUCTION: Juvenile idiopathic arthritis (JIA) is the most common chronic arthropathy, in witch an inflammatory process may lead not only to fixed deformities but also to developmental disturbances, including growth inhibition. The study objective of the current study was to assess the relationship of the degree of physical development disturbances in children with JIA with disease duration, clinical type, the Steinbrocker class of the disease and the therapy applied. MATERIAL AND METHODS: Anthropometric parameters were analysed in 97 children aged 2-18 years (45 girls and 52 boys) with JIA. Bone densitometry (DXA) was performed in 51 children to assess the mineral mass of the whole skeleton (Total BMD) and L2-L4 vertebrae. Results Growth deficiency below -2.0 SDHS--Standard Deviation Height Score (group I) was found in 25 children (25.8%), with the predominance of older children (p<0.05) suffering from a polyarticular type. Children with considerable joint destruction (class III and IV according to Steinbrocker) exhibited lower height (p<0.002) as compared to the group of children without growth disorders (group II) and with early or moderate anatomical joint damage. Significant differences in BMI were noted between group I and II (16.05 and 18.12, respectively; p<0.02). A significant reduction in total bone mineral mass were found in group I as compared to group II (total BMD 0.736 g/cm2 and 0.922 g/cm2; p<0.05). CONCLUSIONS: Growth deficiencies which strongly correlated with the degree of joint destruction and thus with inflammatory activity were found in 25% of the study children and adolescents with JIA. Growth retardation was accompanied by a significant bone mass reduction

7. BEHRENS EM, BEUKELMAN T, GALLO L, SPANGLER J, ROSENKRANZ M, ARKACHAISRI T, AYALA R, GROH B, FINKEL THet CRON RQ: Evaluation of the Presentation of Systemic Onset Juvenile Rheumatoid Arthritis: Data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR), J.Rheumatol., Vol. ., 2007
   Organism:From the Department of Pediatrics, Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia; Department of Pediatrics, Division of Rheumatology, Children's Hospital of Pittsburgh, Pittsburgh; and Department of Pediatrics, Division of Rheumatology, Pennsylvania State University Hershey Medical Center, Hershey, Pennsylvania, USA
   Abstract:OBJECTIVE: To characterize the initial clinical and laboratory features of patients with systemic onset juvenile rheumatoid arthritis (soJRA) through a Web-based registry. METHODS: Patients diagnosed with soJRA in the last 15 years at 3 medical centers in Pennsylvania were identified. Data were collected retrospectively using a Web-based interface in compliance with patient privacy standards. Inferential statistics were used to compare features of patients with and without macrophage activation syndrome. RESULTS: We identified 136 patients; 88% of patients presented with arthritis (8% mono-, 45% oligo-, 47% polyarticular). The most common joints involved were the knee (68% of patients with arthritis), wrist (68%), and ankle (57%). The International League of Associations for Rheumatology criteria for systemic juvenile idiopathic arthritis (SJIA) identified only 30% of patients at presentation. CONCLUSION: We successfully characterized the presenting features of a relatively rare disease, soJRA, through the use of a Web-based registry. Current classification criteria for SJIA may not be particularly sensitive for diagnosis at presentation

8. BENTIN J: [Early rheumatoid arthritis (ERA) diagnosis], Rev.Med.Brux., Vol. 28(4), 295-300., 2007
   Organism:Clinique de Rhumatologie, CHU Brugmann, BruxellesFAU - Bentin, J
   Abstract:The rheumatoid arthritis (RA) diagnosis is based on the ARA criteria, even though the radiological joint erosions are often a requirement to make a definite diagnosis. The early rheumatoid arthritis (ERA) concept was thought of following the poor therapeutic response of the established RA. The "window of opportunity" is defined as a time frame in the early phase of the disease in which the therapeutic response is favoured, and thus giving a real chance to modify the course and the prognosis of RA. To achieve such a goal, new imaging modalities have been developed (MRI and Musculoskeletal ultrasonography--MSU), together with new serologic, inflammatory markers, genetic tests and taking into account the environmental impact (such as tobacco smokers). Such an issue can be achieved with a tight collaboration between the primary care physician and the rheumatology speciality care

9. BEUKELMAN T, GUEVARA JP, ALBERT DA, SHERRY DDet BURNHAM JM: Variation in the initial treatment of knee monoarthritis in juvenile idiopathic arthritis: a survey of pediatric rheumatologists in the United States and Canada, J.Rheumatol., Vol. 34(9), 1918-1924., 2007
   Organism:Department of Pediatrics, Division of Rheumatology and Division of General Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USAFAU - Beukelman, Timothy
   Abstract:OBJECTIVE: To characterize variations in initial treatment for knee monoarthritis in the oligoarthritis subtype of juvenile idiopathic arthritis (OJIA) by pediatric rheumatologists and to identify patient, physician, and practice-specific characteristics that are associated with treatment decisions. METHODS: We mailed a 32-item questionnaire to pediatric rheumatologists in the United States and Canada (n = 201). This questionnaire contained clinical vignettes describing recent-onset chronic monoarthritis of the knee and assessed physicians' treatment preferences, perceptions of the effectiveness and disadvantages of nonsteroidal antiinflammatory drugs (NSAID) and intraarticular corticosteroid injections (IACI), proficiency with IACI, and demographic and office characteristics. RESULTS: One hundred twenty-nine (64%) questionnaires were completed and returned. Eighty-three percent of respondents were board certified pediatric rheumatologists. Respondents' treatment strategies for uncomplicated knee monoarthritis were broadly categorized: initial IACI at presentation (27%), initial NSAID with contingent IACI (63%), and initial NSAID with contingent methotrexate or sulfasalazine (without IACI) (10%). Significant independent predictors for initial IACI were believing that IACI is more effective than NSAID, having performed > 10 IACI in a single patient at one time, and initiating methotrexate via the subcutaneous route for OJIA. Predictors for not recommending initial or contingent IACI were believing that the infection risk of IACI is significant and lacking comfort with performing IACI. CONCLUSION: There is considerable variation in pediatric rheumatologists' initial treatment strategies for knee monoarthritis in OJIA. This variation is primarily associated with perceptions of medication effectiveness and proficiency with IACI. Further studies are warranted to clarify the optimal treatment of OJIA

10. BILLIAU AD, HU Y, VERDONCK A, CARELS Cet WOUTERS C: Temporomandibular joint arthritis in juvenile idiopathic arthritis: prevalence, clinical and radiological signs, and relation to dentofacial morphology, J.Rheumatol., Vol. 34(9), 1925-1933., 2007
    Organism:Department of Pediatric Rheumatology and Department of Orthodontics, University Hospital Leuven, BelgiumFAU - Billiau, An D
    Abstract:OBJECTIVE: To perform a prospective, comprehensive, clinical, and radiological evaluation of temporomandibular joint (TMJ) involvement and its influence on craniofacial growth, in a cohort of patients with juvenile idiopathic arthritis (JIA), representing all JIA subtypes. METHODS: Clinical rheumatologic and orthodontic evaluations were performed in 100 patients with JIA [12 systemic arthritis, 24 rheumatoid factor (RF)-negative polyarthritis, 1 RF-positive polyarthritis, 39 oligoarthritis, 22 enthesitis-related arthritis, 2 psoriatic arthritis]. An orthopantomogram and lateral cephalogram were performed in 46 patients. The prevalence of TMJ arthritis was studied in relation to JIA subtype and disease characteristics; cephalometric measurements were compared to those from age- and sex-matched healthy controls. RESULTS: Whereas 55% of patients with JIA had at least one symptom/sign of TMJ arthritis, 78% of the radiographed group exhibited condylar lesions. The presence of condylar damage was not related to clinical orthodontic findings or to JIA subtype, disease activity, severity, or duration. Patients with JIA exhibited larger mandibular plane and A-nasion-B angles, larger total anterior facial height, smaller interincisal and sella-nasion-B angles, and shorter mandibular ramus lengths than their age- and sex-matched controls. Craniofacial alterations were clearly related to the presence of condylar damage, even when present at a minimal degree. CONCLUSION: Our data show that TMJ condylar damage occurs very frequently in JIA, and irrespective of JIA subtype; condylar lesions can present early, progress insidiously, and -- even at a minimal degree -- can severely alter the craniofacial profile. We propose that the followup of patients with JIA should include early and regular evaluation by an orthodontist, supplemented with radiographic TMJ imaging

11. BLOOM BJ: Macrophage activation is a key component in the pathogenesis of systemic juvenile idiopathic arthritis: comment on the article by Blessing et al, Arthritis Rheum., Vol. 56(11), 3877-3878., 2007
    Organism:

12. BOUT-TABAKU S, RIVAS-CHACON RRet RESTREPO RR: Systemic lupus erythematosus in a patient treated with etanercept for polyarticular juvenile rheumatoid arthritis, J.Rheumatol., Vol. 34(12), 2503, 2007
    Organism:

13. BRIK R, GEPSTEIN V, SHAHAR E, GOLDSHER Det BERKOVITZ D: Tumor necrosis factor blockade in the management of children with orphan diseases, Clin.Rheumatol., Vol. 26(10), 1783-1785., 2007
    Organism:Department of Pediatrics, Meyer Children's Hospital of Haifa, PO Box 9602, Technion, Haifa 31096, Israel r_brik@rambamhealthgovilFAU - Brik, Riva
    Abstract:Tumor necrosis factor (TNF) blockade has been used successfully to treat a number of rheumatic disorders that have a substantial burden of illness. In children, the TNF antagonists are used mainly for the treatment of juvenile idiopathic arthritis (JIA). There are, however, a variety of rare systemic inflammatory diseases, in which TNF blockade appears promising. Preliminary data in adults suggest that several forms of vasculitis appear to be responsive to TNF antagonists-Behcet's disease, polyarteritis nodosa, Wegener granulomatosis, among others. Some of them respond better to infliximab, a chimeric monoclonal anti-TNF antibody, than to etanercept, a recombinant p75 TNF receptor. We describe our limited experience with infliximab in the treatment of three children with rare vasculitic conditions

14. CARCELLER A, TAPIERO B, RUBIN Eet MIRO J: [Acute rheumatic fever: 27 year experience from the Montreal's pediatric tertiary care centers], An.Pediatr.(Barc.)., Vol. 67(1), 5-10., 2007
    Organism:Divisiones de Pediatria, Hospital Sainte-Justine, Canada ana_carceller@ssssgouvqccaFAU - Carceller, A
    Abstract:OBJECTIVES: To examine the epidemiology, clinical characteristics and outcomes in a cohort of children with acute rheumatic fever (RF) over the past 27 years in Montreal. METHODS: The medical records of patients younger than 18 years of age hospitalized and diagnosed with RF in Montreal between January 1979 and December 2005 were reviewed. RESULTS: Among the initial 134 charts selected, 36 children were already followed-up for chronic RF and the remaining 98 patients (51 % females) who fulfilled the Jones criteria for acute RF were included in the analysis. The mean age at diagnosis was 10.1 +/- 3.0 years (range: 3-17). Over the 27-year study period, there was a mean incidence of 3.6 patients/year without peaks, but onset occurred in the last 15 years in almost two-thirds of the patients. Forty-nine percent of the patients were Canadian-born non-aboriginal (CbnA) and the remaining patients were Canadian-born aboriginal (CbA) or foreign-born (Fb). Carditis was diagnosed in 73 % of the patients and Sydenham's chorea in 49 %. Of the CbnA children, 39 % had carditis compared with 61 % of children from other ethnic groups (P = 0.003). However, the form of presentation was chorea in 69 % of CbnA children vs. 31 % of children from other ethnic groups (P < 0.001). No deaths were attributable to acute RF although 2 % of the patients relapsed during the study period. Severe cardiac sequelae requiring valve replacements occurred in 6.1 %. CONCLUSION: The incidence of acute RF in Montreal was low but consistent over the 27-year study period. Clinical presentation varied depending on ethnicity

15. CHANG Jet GIRGIS L: Clinical use of anti-TNF-alpha biological agents--a guide for GPs, Aust.Fam.Physician., Vol. 36(12), 1035-1038., 2007
    Organism:Rheumatology Department, St Vincent's Hospital, Sydney, New South WalesFAU - Chang, John
    Abstract:BACKGROUND: Tumour necrosis factor-alpha (TNF-alpha) inhibitors are a new class of injectable drugs, under the umbrella term 'biological agents', now available for the treatment of rheumatoid arthritis and other chronic inflammatory conditions including juvenile idiopathic arthritis, Crohn disease, psoriasis and psoriatic arthritis. OBJECTIVE: The aim of this review is to provide an overview of TNF-alpha inhibitors and highlight the key practical issues of relevance to general practitioners. DISCUSSION: TNF-alpha inhibitors may have a potent effect in reducing inflammation and possibly inducing remission where conventional disease modifying drugs have failed to do so. These drugs are associated with an increased risk of infection as well as other potentially serious side effects. Their use is restricted to the relevant specialist prescribing the drug and are only available on the Pharmaceutical Benefits Scheme under strict prescribing criteria. The role of the GP is critical in identifying patients suitable for referral to consider commencing treatment and in monitoring patients on long term therapy

16. CHEN HH, KUO HC, WANG L, YU HR, SHEN JM, KWANG KPet YANG KD: Childhood macrophage activation syndrome differs from infection-associated hemophagocytosis syndrome in etiology and outcome in Taiwan, J.Microbiol.Immunol.Infect., Vol. 40(3), 265-271., 2007
    Organism:Division of Pediatric Allergy, Immunology and Rheumatology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University, Kaohsiung, TaiwanFAU - Chen, Hsin Hsu
    Abstract:BACKGROUND AND PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome composed of macrophage activation syndrome (MAS), infection-associated hemophagocytosis syndrome (IAHS), malignancy-associated HLH and genetic HLH. Differentiation of MAS from IAHS and other HLH is important for early appropriate treatment. METHODS: A retrospective analysis was used to differentiate childhood MAS from IAHS and other HLH in Chang Gung Memorial Hospital (CGMH), Kaohsiung. All relevant clinical features, laboratory data, treatments and outcomes were analysed. RESULTS: Seventeen patients with childhood HLH were found at CGMH, Kaohsiung in the past decade, and could be classified into 3 categories: IAHS (9 patients), MAS (5 patients), and HLH of unknown etiology (3 patients). The diagnosis of MAS first appeared in this hospital in 2001. Patients with IAHS tended to be younger than those with MAS. Boys were more frequently found in the IAHS group whereas girls (with systemic lupus erythematosus or juvenile idiopathic arthritis) were more frequently found in the MAS group. The majority of mortality cases were noted in the IAHS group (44%, 4/9). All patients with MAS survived with early cyclosporine A treatment. CONCLUSIONS: Childhood MAS is different from IAHS in terms of age, gender, etiology and mortality. Early administration of cyclosporine A for MAS results in a lower mortality. Further prospective studies are required to confirm these findings

17. CHUNG HV, RILEY M, HO JK, LEUNG B, JEVON GP, ARBOUR LT, BARKER C, SCHREIBER Ret YOSHIDA EM: Retrospective review of pediatric and adult autoimmune hepatitis in two quaternary care centres in British Columbia: increased prevalence seen in British Columbia's First Nations community, Can.J.Gastroenterol., Vol. 21(9), 565-568., 2007
    Organism:Department of Medicine, University of British Columbia, Vancouver, CanadaFAU - Chung, Henry V
    Abstract:BACKGROUND: It has been previously reported that British Columbia's (BC's) First Nations (Aboriginal) community has an increased risk of autoimmune diseases, including rheumatological conditions (rheumatoid arthritis, systemic lupus) and primary biliary cirrhosis. The researchers hypothesized that this community may also be at increased risk for autoimmune hepatitis (AIH). METHODS: Independent, retrospective reviews of the databases of two separate tertiary/quaternary British Columbia university-affiliated health care institutions, the Adult Liver Transplant Program of the BC Transplant Society and the Division of Pediatric Gastroenterology, BC Children's Hospital (Vancouver, BC), were performed. All patients referred with a diagnosis of probable or definite AIH who identified themselves as being of First Nations descent from 1988 to 2004 were reviewed. The liver transplant database records all adult patients in the province referred for transplant assessment. The pediatric database records all children referred to the BC Children's Hospital. RESULTS: A total of 68 adult patients with a definite or probable diagnosis of AIH were referred to the liver transplant program. Twelve patients (17.6%) were Aboriginal, 11 of which were female. Similarly, a total of 30 children with probable or definite AIH were identified from the pediatric database. Six of these cases (20%) were identified in Aboriginal children. CONCLUSIONS: The findings suggest an increased prevalence of AIH among BC's First Nations community. A disproportionate First Nations representation was found on independent review of two databases. Future studies are needed to determine the true prevalence of AIH in this community, and to uncover the genetic predisposition and the environmental triggers explaining this phenomenon

18. CONSOLARO A, VITALE R, PISTORIO A, LATTANZI B, RUPERTO N, MALATTIA C, FILOCAMO G, VIOLA S, MARTINI Aet RAVELLI A: Physicians' and parents' ratings of inactive disease are frequently discordant in juvenile idiopathic arthritis, J.Rheumatol., Vol. 34(8), 1773-1776., 2007
    Organism:Istituto di Ricovero e Cura a Carattere Scientifico G Gaslini, Genova, ItalyFAU - Consolaro, Alessandro
    Abstract:OBJECTIVE: To investigate discrepancies between physicians' and parents' ratings of inactive disease in children with juvenile idiopathic arthritis (JIA) and the determinants of the discrepancy. METHODS: Study data were obtained from the clinical database generated at the study unit. Each patient visit included a standardized assessment of JIA outcome measures. One visit for each patient was selected for analysis. Three definitions of inactive disease were applied to the data: a physician-based definition (physician global assessment = 0); a parent-based definition (parent global assessment = 0); and a formal definition, based on fulfillment of newly developed criteria for inactive disease in JIA. RESULTS: Of 1237 visits made by 537 patients that included both physician and parent global assessments, 265 fulfilled the physician-based definition and/or the parent-based definition of inactive disease. Concordance between physicians and parents in rating the disease as inactive was seen in 40% of the visits, whereas in 60% of visits the 2 assessments were discordant. Parents tended to disagree with physicians in rating the disease as inactive if the child had pain or functional impairment, whereas physicians tended to disagree with parents in the presence of active joint symptoms. Only 2/3 of the 79 visits that fulfilled the formal definition of inactive disease also met the parent-based definition of inactive disease. CONCLUSION: We found frequent discordance between physicians' and parents' ratings of inactive disease in children with JIA, which suggests that the parent's rating of a child's disease activity should be considered for inclusion in the definition of clinical remission for JIA

19. DUBNER SE, SHULTS J, LEONARD MB, ZEMEL BS, SEMBHI Het BURNHAM JM: Assessment of Spine Bone Mineral Density in Juvenile Idiopathic Arthritis: Impact of Scan Projection, J.Clin.Densitom., Vol. ., 2007
    Organism:University of Pennsylvania School of Medicine
    Abstract:Although children with juvenile idiopathic arthritis (JIA) are at risk for vertebral fractures, recent conventional posterior-anterior (PA) spine dual-energy X-ray absorptiometry studies reported minimal areal bone mineral density (aBMD, g/cm(2)) deficits. Width-adjusted BMD (WA-BMD, g/cm(3)) represents the bone mineral content (BMC) from the lateral projection, excluding the dense cortical spinous processes, divided by the estimated vertebral body volume based on paired PA-lateral bone dimensions. Therefore, WA-BMD may be more sensitive to JIA effects on the predominantly trabecular vertebral body. Age- and sex-specific Z-scores for spine aBMD and WA-BMD were generated in 84 JIA subjects compared with healthy controls, aged 5-21yr. JIA was associated with lower mean WA-BMD Z-scores (-0.78, 95% CI: -1.03, -0.53; p<0.001) and aBMD Z-scores (-0.26, 95% CI: -0.49, -0.02; p<0.05), compared with controls. WA-BMD Z-scores were significantly lower than aBMD Z-scores in JIA (p<0.001). A significant JIA by age interaction (p<0.001) indicated that the magnitude of the difference between WA-BMD and aBMD Z-scores was greater in younger subjects. In conclusion, WA-BMD may be more sensitive to disease effects in children because it selectively measures the trabecular-rich vertebral body and is independent of growth-related changes in BMC of the dense spinous processes

20. EIKE MC, NORDANG GB, KARLSEN TH, BOBERG KM, VATN MH AND THE IB, DAHL-JORGENSEN K, RONNINGEN KS, JONER G, FLATO B, BERGQUIST A, THORSBY E, FORRE O, KVIEN TK, UNDLIEN DEet LIE BA: The FCRL3 -169T>C polymorphism is associated with rheumatoid arthritis and shows suggestive evidence of involvement with juvenile idiopathic arthritis in a Scandinavian panel of autoimmune diseases, Ann.Rheum.Dis., Vol. ., 2007
    Organism:Rikshospitalet HF/University of Oslo, Norway
    Abstract:BACKGROUND: and objectives: The Fc receptor-like 3 (FCRL3) -169T>C single nucleotide polymorphism (SNP) has been reported associated with several autoimmune diseases (AIDs) in Japanese populations. However, association results in other populations have been conflicting. Therefore, we have investigated this SNP in a Scandinavian panel of AIDs. METHODS: We genotyped patients with rheumatoid arthritis (RA; n=708), juvenile idiopathic arthritis (JIA; n=524), systemic lupus erythematosus (SLE; n=166), ulcerative colitis (UC; n=335), primary sclerosing cholangitis (PSC; n=365), Crohn's disease (CD; n=149), a healthy control group (n=1030) and 425 trio families with type 1 diabetes (T1D). Statistical analysis consisted of both case-control and family-based association tests. RESULTS: RA was associated both with the C allele (OR=1.16, 95 % CI 1.01-1.33) and the CC genotype (OR=1.30, 95 % CI 1.01-1.67) of the FCRL3 -169T>C SNP in our material. Suggestive evidence for association was also found for JIA (CC genotype: OR=1.30, 95% CI 0.99-1.70), and clinical subgroup analysis indicated that this was connected to the polyarticular subgroup. No significant association was found with SLE, UC, CD, PSC or T1D. In RA patients, we found no significant interaction between the FCRL3 -169T>C and PTPN22 1858C>T SNPs, nor between the FCRL3 -169CC genotype and IgM-rheumatoid factor or anti-cyclic citrullinated peptide titer levels. CONCLUSION: We found an association between the FCRL3 -169T>C SNP and RA, and suggestive evidence for involvement with JIA, in a Norwegian population. These findings lend support for a role for this SNP in RA across ethnically diverse populations, and warrant follow-up studies in JIA

21. FALL N, BARNES M, THORNTON S, LUYRINK L, OLSON J, ILOWITE NT, GOTTLIEB BS, GRIFFIN T, SHERRY DD, THOMPSON S, GLASS DN, COLBERT RAet GROM AA: Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome, Arthritis Rheum., Vol. 56(11), 3793-3804., 2007
    Organism:Children's Hospital Medical Center, Cincinnati, Ohio 45229, USAFAU - Fall, Ndate
    Abstract:OBJECTIVE: Systemic juvenile idiopathic arthritis (JIA) is frequently associated with the development of macrophage activation syndrome. This study was undertaken to better understand the relationship between systemic JIA and macrophage activation syndrome. METHODS: Gene expression profiles were examined in 17 patients with untreated new-onset systemic JIA, 5 of whom showed evidence of subclinical macrophage activation syndrome (of whom 2 eventually developed overt macrophage activation syndrome). Peripheral blood mononuclear cells (PBMCs) were separated on Ficoll gradients, and purified RNA was analyzed using Affymetrix GeneChip expression arrays. A fraction of the PBMCs were used for flow cytometry to define the cellular composition of the samples. RESULTS: Two hundred twenty-five differentially expressed genes (P < 0.05) that distinguished patients with systemic JIA from healthy controls (n = 30) were identified. Clustering analysis indicated that expression patterns correlated with serum ferritin levels. Three main clusters distinguished systemic JIA patients with highly elevated ferritin levels (including those with subclinical macrophage activation syndrome) from those with normal or only moderately elevated ferritin levels. The first cluster comprised genes involved in the synthesis of hemoglobins and structural proteins of erythrocytes. This transcriptional profile was consistent with immature nucleated red blood cells, likely reflective of high red blood cell turnover. Also included were transcripts indicating immature granulocytes. The second cluster was enriched for genes involved in cell cycle regulation. The third cluster was enriched for genes involved in innate immune responses, including those involved in the negative regulation of Toll-like receptor/interleukin-1 receptor-triggered inflammatory cascades and markers of the alternative pathway of macrophage differentiation. Additional differentially expressed genes of interest were those involved in the cytolytic pathway, including SH2D1A and Rab27a. CONCLUSION: These data indicate that gene expression profiling can be a useful tool for identifying early macrophage activation syndrome in patients with systemic JIA

22. FONOLLOSA A, SEGURA A, GIRALT Jet GARCIA-ARUMI J: Tuberculous uveitis after treatment with etanercept, Graefes Arch.Clin.Exp.Ophthalmol., Vol. 245(9), 1397-1399., 2007
    Organism:Hospital Vall d'Hebron, Barcelona, Spain 36427afc@combsesFAU - Fonollosa, Alex
    Abstract:BACKGROUND: Etanercept is a tumor necrosis factor (TNF) inhibitor that has been licensed in the United States for the treatment of adult and juvenile rheumatoid arthritis as well as psoriatic arthritis. Reactivation of tuberculosis is a complication of its use. We report the first case of tuberculous uveitis due to etanercept. METHODS: We performed a clinical chart review. CASE: A 58-year-old Caucasian woman was referred to our hospital for chronic unilateral granulomatous panuveitis of the right eye (RE). She was on etanercept and methotrexate for rheumatoid arthritis. Since the patient was immunosuppressed with etanercept and since the uveitis was granulomatous we considered tuberculosis as a possible etiology. An aqueous humor tap was performed and sent for polymerase chain reaction analyses of Herpes simplex, Herpes zoster, and Mycobacterium tuberculosis (MT). This last test was positive. Another aqueous humor sample was taken and sent for microscopic examination of sputum for acid-fast bacilli and culture, both of which were positive for MT. A diagnosis of tuberculous uveitis was established; the patient was treated with rifampin, isoniazid pyrazinamide, and ethambutol and etanercept was stopped. Four months later there were no cells in the anterior chamber and the vitreous was clear. DISCUSSION: To our knowledge this is the first reported case of tuberculous uveitis following treatment with etanercept. This etiology has to be considered in patients taking this drug who present with intraocular inflammation

23. FURST DE, BREEDVELD FC, KALDEN JR, SMOLEN JS, BURMESTER GR, SIEPER J, EMERY P, KEYSTONE EC, SCHIFF MH, MEASE P, VAN RIEL PL, FLEISCHMANN R, WEISMAN MHet WEINBLATT ME: Updated consensus statement on biological agents for the treatment of rheumatic diseases, 2007, Ann.Rheum.Dis., Vol. 66 Suppl 3, iii2-22., 2007
    Organism:David Geffen School of Medicine, UCLA - RM 32-59, 1000 Veteran Avenue, Los Angeles, CA 90025, USA defurst@mednetuclaeduFAU - Furst, D E
    Abstract:

24. GATTORNO M, TASSI S, CARTA S, DELFINO L, FERLITO F, PELAGATTI MA, D'OSUALDO A, BUONCOMPAGNI A, ALPIGIANI MG, ALESSIO M, MARTINI Aet RUBARTELLI A: Pattern of interleukin-1beta secretion in response to lipopolysaccharide and ATP before and after interleukin-1 blockade in patients with CIAS1 mutations, Arthritis Rheum., Vol. 56(9), 3138-3148., 2007
    Organism:Second Division of Pediatrics, IRCCS, Istituto G Gaslini, Genoa, Italy marcogattorno@ospedale-gaslinigeitFAU - Gattorno, Marco
    Abstract:OBJECTIVE: To examine the synthesis, processing, and secretion of interleukin-1beta (IL-1beta), as well as the clinical and biologic effects of IL-1 blockade, in patients with chronic infantile neurologic, cutaneous, articular (CINCA) syndrome and Muckle-Wells syndrome (MWS), in an effort to understand the molecular mechanisms linking mutations of the CIAS1 gene and IL-1beta hypersecretion, and the underlying response to IL-1 receptor antagonist (IL-1Ra). METHODS: Six patients with CINCA syndrome or MWS were treated with IL-1Ra and followed up longitudinally. Monocytes obtained from the patients and from 24 healthy donors were activated with lipopolysaccharide (LPS) for 3 hours, and intracellular and secreted IL-1beta levels were determined by Western blotting and enzyme-linked immunosorbent assay before and after exposure to exogenous ATP. RESULTS: LPS-induced IL-1beta secretion was markedly increased in monocytes from patients with CIAS1 mutations. However, unlike in healthy subjects, secretion of IL-1beta was not induced by exogenous ATP. Treatment with IL-1Ra resulted in a dramatic clinical improvement, which was paralleled by an early and strong down-regulation of LPS-induced IL-1beta secretion by the patients' cells in vitro. CONCLUSION: Our results showed that the requirements of ATP stimulation for IL-1beta release observed in healthy individuals are bypassed in patients bearing CIAS1 mutations. This indicates that cryopyrin is the direct target of ATP and that the mutations release the protein from the requirement of ATP for activation. In addition, the dramatic amelioration induced by IL-1Ra treatment is at least partly due to the strong decrease in IL-1beta secretion that follows the first injections of the antagonist. These findings may have implications for other chronic inflammatory conditions characterized by increased IL-1beta

25. HEILIGENHAUS A, MINGELS A, HEINZ Cet GANSER G: Methotrexate for uveitis associated with juvenile idiopathic arthritis: value and requirement for additional anti-inflammatory medication, Eur.J.Ophthalmol., Vol. 17(5), 743-748., 2007
    Organism:Department of Ophthalmology, St Franziskus Hospital, Muenster, Germany arndheilingenhaus@uveitis-zentrumdeFAU - Heiligenhaus, A
    Abstract:PURPOSE: To study the value of methotrexate (MTX) and the requirement for additional anti-inflammatory drugs for the treatment of severe chronic iridocyclitis associated with juvenile idiopathic arthritis (JIA). METHODS: Institutional study of 35 consecutive patients with JIA started on MTX as the single systemic immunosuppressive drug for the treatment of associated iridocyclitis. The clinical epidemiologic data, course of visual acuity (VA), development of complications, and the need for additional anti-inflammatory drugs were analyzed. RESULTS: Mean follow-up with MTX treatment was 27.6 months. Uveitic complications were present in 31 patients before MTX treatment. With MTX, quiescence of uveitis was obtained with (n=21) or without (n=4) additional topical steroids. Additional systemic immunosuppressive drugs were required in another 7 patients: cyclosporine A (n=4), azathioprine (n=1), infliximab (n=1), or etanercept (n=1). Three patients had active uveitis at the end of the follow-up period. During MTX therapy, uveitis first developed in the unaffected fellow eyes in 2 patients, and secondary glaucoma or ocular hypertension occurred in 7 patients. The VA deteriorated in 6, improved in 13, and was stable in the remaining eyes. CONCLUSIONS: The data suggest that MTX is very effective in controlling inflammation of uveitis in patients with JIA. However, additional topical steroids or systemic immunosuppressive drugs are often required

26. 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., 2007
    Organism:CDC, Atlanta, Georgia
    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

27. HO HH, YU KH, CHEN JY, LIN JL, WU YJ, LUO SFet LIOU LB: Coexisting ankylosing spondylitis and gouty arthritis, Clin.Rheumatol., Vol. 26(10), 1655-1661., 2007
    Organism:Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Chang Gung Memorial Hospital and Chang Gung University, 5 Fu-shin Street, Kuei-Shan, Tao-Yuan, Taiwan ling0126@cgmhorgtwFAU - Ho, Huei-Huang
    Abstract:The aim of this study was to investigate the clinical characteristics of patients with coexisting ankylosing spondylitis (AS) and gout. Between July 1987, and October 2004, sixty-five patients with coexisting AS and gout were enrolled. The clinical manifestations of both AS and gout in these patients were studied. Of the 65 patients included in the study, 61 were men and four were women (men-to-women ratio, 15.3:1). Sixty-three subjects were Han Chinese, and two were Atayal Aborigines. Mean ages at onset of AS and gout were 29.3 +/- 15.6 years (range 7-63) and 42.2 +/- 13.2 years (range 20-74), respectively. Fifty-six patients developed gout after (15.5 +/- 11.2 years; range, 1-51 years) onset of AS; nine patients developed gout before (average, 3.4 +/- 2.2 years; range. 1-7 years) onset of AS. Forty-four (67.7%) patients had chronic peripheral arthritis and all 65 (100%) patients had acute peripheral arthritis. Thirty-three (50.8%) cases had heel pain (enthesopathy), including 22 (33.9%) with chronic heel pain, seven (10.8%) with acute heel pain, and four (6.2%) with concurrent acute and chronic heel pain. Sixty-one (93.9%) subjects were HLA-B27 antigen positive. Medical conditions potentially associated with hyperuricemia or gout were urolithiasis (n = 17), hypertension (n = 21), diabetes mellitus (n = 8), hyperlipidemia (n = 34), congestive heart failure (n = 6), coronary heart disease (n = 5), and stroke (n = 3). The following drugs were prescribed: diuretics (n = 7), low-dose aspirin (n = 4), antituberculous drugs (n = 1), and sulphasalazine (n = 34). Six (6.2%) patients had iatrogenic Cushing syndrome with adrenal insufficiency. Patients with coexisting AS and gout are not rare. Distinguishing between peripheral arthritis or enthesopathies of AS and gout is essential, especially when the course of AS arthritis becomes acute or the course of gout becomes chronic

28. HOEGH SV, LINDEGAARD HM, SORENSEN GL, HOJ A, BENDIXEN C, JUNKER Pet HOLMSKOV U: Circulating surfactant protein D is decreased in early rheumatoid arthritis: a 1-year prospective study, Scand.J.Immunol., Vol. 67(1), 71-76., 2008
    Organism:Medical Biotechnology Center, Institute of Medical Biology, University of Southern Denmark, Odense, DenmarkFAU - Hoegh, S V
    Abstract:Innate immune system abnormalities, e.g., mannan-binding lectin (MBL) genotype variants, have been demonstrated to modify the disease course of rheumatoid arthritis (RA). Surfactant protein D (SP-D) shares important structural and functional properties with MBL suggesting that SP-D may be an additional RA disease modifier. The Met11Thr polymorphism in the N-terminal part of SP-D is an important determinant for the SP-D serum level, but this polymorphism is also essential to the function and assembly into oligomers. We aimed to compare the serum levels of SP-D in a cohort of newly diagnosed untreated RA patients with healthy matched controls, and to investigate if there was an association to core measures of disease activity within the first year after disease onset. Secondly, we aimed to investigate whether the Met11Thr polymorphism was associated with RA. Serum SP-D was significantly lower in DMARD naive RA patients compared with healthy controls (P = 0.016). Median SP-D concentration at inclusion was 878 ng/ml (95% CI: 730-1033) and 1164 ng/ml (95% CI: 1093-1366) in RA patients and matched controls, respectively. SP-D increased during Methotrexate treatment (P < 0.0001), and at 1-year follow-up median SP-D was 1032 ng/ml (95% CI: 777-1255). SP-D levels did not correlate with traditional disease activity measures. The Thr11/Thr11 genotype and the Thr11 allele tended to be more frequent in RA patients. In conclusion, the low serum level of SP-D and the lack of correlation with traditional disease activity measures indicate that SP-D reflects a distinctive aspect in the RA pathogenesis

29. IKAVALKO M, SKYTTA ETet BELT EA: One-year results of use of poly-L/D-lactic acid joint scaffolds and bone packing in revision metacarpophalangeal arthroplasty, J.Hand Surg.Eur.Vol., Vol. 32(4), 427-433., 2007
    Organism:Rheumatism Foundation Hospital, Heinola, FinlandFAU - Ikavalko, M
    Abstract:Revision metacarpophalangeal arthroplasty after silicone implant arthroplasty is frequently complicated by severe bone loss, osteolysis and diaphyseal perforations. Impacted, morselised allografts are frequently used to treat bone loss in revision surgery. A new method of treatment using bioreconstructive poly-L/D-lactic acid (PLDLA) joint scaffold and allograft bone packing, after complete removal of the original silicone implants, allows recovery of bony deficiencies, correction of malalignment and improves function of the hand. This article presents the one-year results of a prospective, non-randomised clinical and radiographic follow-up study of 21 patients with 52 revision metacarpophalangeal arthroplasties using the PLDLA implants and allograft bone packing

30. IMMONEN K, SAVOLAINEN HAet HAKALA M: Why can we no longer find juvenile idiopathic arthritis-associated amyloidosis in childhood or in adolescence in Finland?, Scand.J.Rheumatol., Vol. 36(5), 402-403., 2007
    Organism:

31. IMRIE FRet DICK AD: Biologics in the treatment of uveitis, Curr.Opin.Ophthalmol., Vol. 18(6), 481-486., 2007
    Organism:Academic Unit of Ophthalmology, University of Bristol and Bristol Eye Hospital, Bristol, UKFAU - Imrie, Fraser R
    Abstract:PURPOSE OF REVIEW: This review summarizes the current evidence for biologic therapies in the treatment of uveitis. The review emphasizes published research in this field since 2005. RECENT FINDINGS: The anti-tumour necrosis factor-alpha infliximab and adalimumab have demonstrated significant efficacy in controlling uveitis associated with seronegative spondyloarthropathies and juvenile idiopathic arthritis; however, etanercept has failed to show a similar treatment effect in uveitis associated with these conditions. The majority of reports of biologic therapies in posterior uveitis have been uncontrolled trials, or retrospective studies, of uveitis resistant to immunosuppression. Encouragingly, successful control of such refractory intraocular inflammation has been consistently reported with infliximab and interferon alpha, particularly Behcet's disease-associated uveitis. A limited number of reports of anti-interleukin therapies, daclizumab and anakinra, have supported a role for these therapies in some types of uveitis. SUMMARY: Biologic therapies have increased the treatment options for sight-threatening uveitis. Despite experimental rationale, the lack of evidence from randomized controlled studies limits our understanding of when to commence therapy, which agent to choose and how long to continue treatment. Additionally, the high cost and potential side effects of all biologic agents have limited their current use to uveitis refractory to immunosuppression

32. KARMAZYN B, BOWYER SL, SCHMIDT KM, BALLINGER SH, BUCKWALTER K, BEAM TTet YING J: US findings of metacarpophalangeal joints in children with idiopathic juvenile arthritis, Pediatr.Radiol., Vol. 37(5), 475-482., 2007
    Organism:Radiology, Riley Hospital for Children, Indianapolis, IN 46202-5200, USA bkarmazy@iupuieduFAU - Karmazyn, Boaz
    Abstract:BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children, with frequent involvement of the metacarpophalangeal joints (MCPJ). OBJECTIVE: To compare US findings with those of radiography and clinical examination. MATERIALS AND METHODS: All MCPJs in 20 children with JIA (17 females, median age 9.7 years, range 3.6 to 16.8 years) were evaluated clinically and imaged with gray-scale and color Doppler US, and 90 MCPJs were also imaged radiographically. Each MCPJ was graded on physical examination from 0 (normal) to 4 (severe) by the patient's rheumatologist. RESULTS: US demonstrated abnormalities in 64 of 200 MCPJs (32.0%), including pannus vascularity and/or tenosynovitis in 55 joints (27.5%) (pannus vascularity in 43, tenosynovitis in 40) and bone destruction in 25 joints (12.5%). Overall, US abnormalities and physical examination scores were significantly associated (P < 0.001). However, interobserver agreement between US and clinical evaluation was poor (kappa 0.1) and between US and radiography was only fair (kappa 0.4). CONCLUSION: US of the MCPJ in children with JIA can demonstrate cartilage thinning, bone erosions, and pannus vascularity. Abnormal US findings are significantly correlated with severity of disease as evaluated clinically

33. 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:Division of Immunology and Allergy, Department of Pediatrics, Allergy Research Center, Division of Endocrinology, Shiraz University of Medical Sciences, Shiraz, IranFAU - 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

34. KELLY Aet RAMANAN AV: Recognition and management of macrophage activation syndrome in juvenile arthritis, Curr.Opin.Rheumatol., Vol. 19(5), 477-481., 2007
    Organism:Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol and Royal National Hospital for Rheumatic Diseases, Bath, UKFAU - Kelly, Alison
    Abstract:PURPOSE OF REVIEW: Macrophage activation syndrome is a life-threatening complication seen predominantly in children with systemic onset juvenile idiopathic arthritis. It accounts for a significant amount of the morbidity and mortality seen with systemic onset juvenile idiopathic arthritis. RECENT FINDINGS: In this article, we will look at the new developments in the diagnosis, classification, pathogenesis and management of macrophage activation syndrome in systemic onset juvenile idiopathic arthritis patients. SUMMARY: More work is needed to further elucidate the pathophysiology of macrophage activation syndrome in systemic onset juvenile idiopathic arthritis. This would be the key to early diagnosis using more sensitive criteria and better management

35. KOENIG MK, PEREZ M, ROTHENBERG Set BUTLER IJ: Juvenile Onset Central Nervous System Folate Deficiency and Rheumatoid Arthritis, J.Child Neurol., Vol. ., 2007
    Organism: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 theautoantibodies diminishing the uptake of this folate

36. KOHEM CL, BRENOL JC, XAVIER RM, BREDEMEIER M, BRENOL CV, DEDAVID E SILVA TL, DE CASTILHOS MA, CANEDO AD, NEVES AGet CHIES JA: The chemokine receptor CCR5 genetic polymorphism and expression in rheumatoid arthritis patients, Scand.J.Rheumatol., Vol. 36(5), 359-364., 2007
    Organism:Rheumatic Diseases Division, Hospital de Clinicas de Porto Alegre, Porto Alegre, Brazil charleskohem@brturbocombrFAU - Kohem, C L
    Abstract:OBJECTIVES: To identify the genetic polymorphism of the chemokine receptor CCR5 (the Delta32 allelic variant) in patients with rheumatoid arthritis (RA) and compare the findings with healthy controls. To compare the CCR5 phenotypic expression in T cells and monocytes isolated from the peripheral blood and synovial fluid in a subgroup of RA patients. METHODS: CCR5 genes of 92 RA patients and 160 healthy controls were genotyped using specific primers flanking the region of deletion. The ethnic distribution was similar between the groups. Flow cytometric analysis was used for immunophenotyping the T cells and monocytes isolated from the peripheral blood and synovial fluid of eight RA patients. The isolated cells were triple stained with CD4 or CD8, CD25 and CCR5 monoclonal antibodies. RESULTS: There was no difference in the CCR5Delta32 genotypic frequency between the RA patients and the control group (0.055 and 0.063, respectively, p = 0.989). No homozygote for the CCR5Delta32 allele was seen in either group. Five heterozygotes were identified in the RA patient group, whose disease was shown to be aggressive. A significant enrichment of activated CCR5+ monocytes was seen in the synovial fluid of the RA patients subjected to arthrocentesis, who were all homozygotes for the CCR5 wild-type genotype. CONCLUSION: A protective role for the CCR5 allelic variant in RA development was not observed. Disease severity in the heterozygotes suggests that other proinflammatory mechanisms might overcome this mutation in vivo. The activated CCR5+ monocyte enrichment in the rheumatoid synovial fluid might indicate that this cell population has an important role in the pathogenesis of the disease

37. KOKKONEN J, ARVONEN M, VAHASALO Pet KARTTUNEN TJ: Intestinal immune activation in juvenile idiopathic arthritis and connective tissue disease, Scand.J.Rheumatol., Vol. 36(5), 386-389., 2007
    Organism:Department of Paediatrics, University of Oulu, Oulu, FinlandFAU - Kokkonen, J
    Abstract:OBJECTIVES: To examine the prevalence of immune activation in gastrointestinal (GI) mucosa in children with juvenile idiopathic arthritis (JIA) or connective tissue disease (CTD). STUDY DESIGN: We studied 27 children (15 girls, mean age 9.8+/-4.8 years) with JIA/CTD and GI symptoms, including nine with oligoarthritis, nine with polyarthritis, two with systemic arthritis, three with enthesitis-related arthritis, and four with various CTDs. The control group consists of 54 children (31 girls, mean age 11.3+/-6.3 years) with GI symptoms but shown to have no significant GI or rheumatoid disorder. The subjects were examined by gastroduodenoscopy (22 patients, 50 controls) and colonoscopy (23 patients, 16 controls). Intraepithelial CD3+, alpha/beta+, and gamma/delta+ lymphocytes were counted from duodenal and ileal biopsies. RESULTS: Five patients with JIA/CTD (19%) had ulcerative colitis. Lymphoid nodular hyperplasia (LNH) was more common in the patients [74% (20/27)] than in the controls [16% (8/50), p = 0.001], as well in the duodenal bulb [29% (7/24) vs. 10% (5/50)], terminal ileum [74% (14/19) vs. 38% (5/13)], and the colon [50% (11/22) vs. 14% (2/14)]. In the duodenum, CD3, alpha/beta+, and gamma/delta+ lymphocytes counts were higher in JIA/CTD (p<0.05). In the ileum, gamma/delta+ cell numbers had increased in JIA/CTD (p<0.05). Either LNH, increased gamma/delta+ count, or both were more common in JIA/CTD [89% (24/27)] than in the controls [13% (7/54), p<0.0001]. CONCLUSIONS: The majority of children suffering from JIA or CTD with GI symptoms show abnormalities consistent with activation of the intestinal immune system. The aetiology of this reaction remains unknown, but similar features are seen in delayed-type food allergy

38. KOMATSU Het TATENO A: Failure to distinguish systemic-onset juvenile idiopathic arthritis from incomplete Kawasaki disease in an infant, J.Paediatr.Child Health., Vol. 43(10), 707-709., 2007
    Organism:Department of Paediatrics, Sakura Hospital, Toho University, 564-1 Shimoshizu Sakura, Chiba 285-8741, Japan haruki-komatsu@chiveocnnejpFAU - Komatsu, Haruki
    Abstract:In an infant, an initial diagnosis of incomplete Kawasaki disease was made according to the American Heart Association guidelines. However, the diagnosis of systemic-onset juvenile idiopathic arthritis was established later. Physicians need to recognize that systemic-onset juvenile idiopathic arthritis can be mistaken for incomplete Kawasaki disease, even when the guidelines are used

39. KUNITOMI Tet IKEDA M: [Rheumatoid arthritis complicated with pericarditis--focused mainly on juvenile idiopathic arthritis], Nippon Rinsho., Vol. Suppl 5 Pt 2, 458-460., 2007
    Organism:Department of Pediatrics, Okayama Red Cross General HospitalFAU - Kunitomi, Taiji
    Abstract:

40. LACAILLE D, WHITE MA, BACKMAN CLet GIGNAC MA: Problems faced at work due to inflammatory arthritis: new insights gained from understanding patients' perspective, Arthritis Rheum., Vol. 57(7), 1269-1279., 2007
    Organism:University of British Columbia, and The Arthritis Research Centre of Canada, Vancouver, British Columbia, Canada dlacaille@arthritisresearchcaFAU - Lacaille, Diane
    Abstract:OBJECTIVE: A qualitative study was conducted to better understand patients' perspective on their experience at work in relation to their inflammatory arthritis (IA). Objectives were to identify the problems and barriers to employment that persons with IA face at work because of arthritis, understand why these issues are problematic, and identify strategies helpful for maintaining employment. METHODS: Five focus groups were conducted with 36 employed adults with IA (75% rheumatoid arthritis) recruited from rheumatology practices and outpatient arthritis treatment programs. Script design used brainstorming techniques to identify problems and helpful strategies, and root cause analysis to capture in-depth information about underlying causes of problems. Descriptive qualitative analysis of transcripts was performed by 2 researchers independently to identify problems and organize them into topics and broad categories. RESULTS: Problems clustered around 4 categories: arthritis symptoms, working conditions, interpersonal difficulties at work, and emotional challenges. New insights gained included identifying fatigue as the aspect of IA most limiting employment; challenges posed by invisibility, fluctuation, and unpredictability of arthritis; complexity of interpersonal relationships at work; reluctance to disclose or draw attention to arthritis; numerous barriers to using available supports and requesting job accommodations, including fear of disclosure and concern it could be perceived by coworkers as favoritism; loss of self-efficacy at work; and many emotional challenges. CONCLUSION: This research identified new issues that are meaningful to individuals working with arthritis and that deserve greater attention by professionals counseling people on employment, in intervention efforts to help maintain employment, and in arthritis employment studies

41. LONG AC, KRISHNAMURTHY Vet PALERMO TM: Sleep Disturbances in School-age Children with Chronic Pain, J.Pediatr.Psychol., Vol. ., 2007
    Organism:Oregon Health & Science University
    Abstract:OBJECTIVES: To examine associations between pain, functional outcomes, and sleep disturbances in children with chronic pain, specifically juvenile idiopathic arthritis (JIA), sickle cell disease (SCD), and headache (HA). Sleep disturbances were tested as a risk factor for increased functional disability and decreased health-related quality of life (HRQOL). METHODS: One hundred children (JIA n = 30, SCD n = 26, HA n = 44; 8-12 years; 56% female) and their caregivers participated. Children completed questionnaires regarding pain, depression, and functional disability. Caregivers completed questionnaires regarding sociodemographics, child sleep habits, functional disability, and HRQOL. RESULTS: Levels of overall sleep disturbances were above the clinical cutoff for 53% of children with chronic pain. Sleep disturbances predicted lower physical HRQOL and higher functional disability, according to parent report. CONCLUSIONS: Sleep disturbances are common and associated with daytime functioning in school-age children with chronic pain, suggesting that assessment and treatment of sleep problems is clinically relevant

42. MAJORCZYK E, JASEK M, PLOSKI R, WAGNER M, KOSIOR A, PAWLIK A, OBOJSKI A, LUSZCZEK W, NOWAK I, WISNIEWSKI Aet KUSNIERCZYK P: Association of PTPN22 single nucleotide polymorphism with rheumatoid arthritis but not with allergic asthma, Eur.J.Hum.Genet., Vol. 15(10), 1043-1048., 2007
    Organism:Laboratory of Immunogenetics, Department of Clinical Immunology, Ludwik Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Wroclaw, PolandFAU - Majorczyk, Edyta
    Abstract:PTPN22 gene encodes a lymphoid tyrosine phosphatase (LYP), an important negative regulator of T-cell responses. The 1858C>T (Arg620Trp) single nucleotide polymorphism (rs2476601) was found associated with autoimmune diseases, including rheumatoid arthritis (RA). Allergic diseases are similar to autoimmune diseases, by an exaggerated immune response to an antigen (allergen in this case) normally not invoking such response in healthy individuals. We investigated whether polymorphism 1858C>T in PTPN22 gene is associated with susceptibility to allergic asthma and RA in a Polish population. PTPN22 was genotyped in 173 patients with RA, in 198 patients with allergic asthma, and in 543 controls using PCR-RFLP. The patients with RA differed from healthy controls in frequencies of PTPN22 1858C>T alleles (P=0.0004; odds ratio (OR), 1.8; 95% CI, 1.33-2.55) and genotypes (P=0.0009). Strong associations of 1858T allele with RA limited to joints (0.21 vs 0.12, P=0.0002; OR, 2.1; 95% CI, 1.44-3.00), with erosive disease (0.20 vs 0.12, P=0.0003; OR, 1.92; 95% CI, 1.34-2.71), with a lack of rheumatoid factor (RF; 0.23 vs 0.12, P=0.0008; OR, 2.29; 95% CI, 1.44-3.63), and weak association with the presence of RF (0.17 vs 0.12, P=0.02; OR, 1.6; 95% CI, 1.10-2.40) in comparison with healthy controls were observed. Very strong association of 1858T allele (P<0.0001; OR, 2.72; 95% CI, 1.9-3.9) and T phenotype (P<0001; OR, 3.2; 95% CI, 2.1-4.9) with antibodies to cyclic citrullinated peptide (CCP) was found. When patients with allergic asthma were typed for PTPN22 1858C>T polymorphism, no difference with control was found. Subdivision of patients into those with mild, moderate, or severe asthma did not reveal any associations. In conclusion, we confirmed associations between several clinical manifestations of RA and PTPN22 1858T allele. However, no association with 1858C>T polymorphism was found for susceptibility to allergic asthma or for severity of the disease

43. MATHES EFet GILLIAM AE: A four-year-old boy with Fever, rash, and arthritis, Semin.Cutan.Med.Surg., Vol. 26(3), 179-187., 2007
    Organism:Departments of Dermatology and Pediatrics, University of California, San Francisco, CAFAU - Mathes, Erin F D
    Abstract:The triad of fever, rash, and arthritis in a hospitalized child suggests an inflammatory, infectious, or postinfectious process in most cases; however, malignancy must be considered. The most common causes in this age group are inflammatory conditions, including Kawasaki disease, Henoch-Schonlein Purpura, serum sickness-like reaction, and juvenile idiopathic arthritis. Other rarer inflammatory processes can present with this triad of symptoms such as Cryopyrin-related diseases (autoinflammatory disorders), urticarial vasculitis, and systemic lupus erythematosus. We will discuss the differential diagnosis and inpatient management of fever, rash, and arthritis in a young child, focusing on inflammatory conditions. The important features which can help distinguish these conditions include the nature of the rash, associated signs or symptoms, time course of the eruption, and characteristic laboratory and/or histologic findings

44. MULLAN RH, MATTHEWS C, BRESNIHAN B, FITZGERALD O, KING L, POOLE AR, FEARON Uet VEALE DJ: Early changes in serum type II collagen biomarkers predict radiographic progression at one year in inflammatory arthritis patients after biologic therapy, Arthritis Rheum., Vol. 56(9), 2919-2928., 2007
    Organism:St Vincent's University Hospital, Dublin, IrelandFAU - Mullan, Ronan H
    Abstract:OBJECTIVE: To investigate whether short-term changes in serum biomarkers of type II collagen degradation (C2C) and types I and II collagen degradation (C1,2C), as well as the biomarker for the synthesis of type II procollagen (CPII) can predict radiographic progression at 1 year following initiation of biologic therapy in patients with inflammatory arthritis. METHODS: Serum levels of biomarkers were measured at baseline and at 1, 3, 6, 9, and 12 months after initiation of biologic therapy. A composite score reflecting changes from baseline in all 3 biomarkers (DeltaCOL) was calculated. Associations with clinical responses according to the 28-joint count Disease Activity Score and with radiographic progression according to the modified Sharp/van der Heijde score (SHS) were assessed. RESULTS: The 1-year increase in the SHS correlated with the 1-month change in C2C results (r = 0.311, P = 0.028) and the DeltaCOL score (r = 0.342, P = 0.015). Radiographic progression was predicted by increases in serum C2C at 1 month (P = 0.031). The DeltaCOL score was significantly associated with 1-year radiographic progression after 1 (P = 0.022), 3 (P = 0.015), 6 (P = 0.048), and 9 (P = 0.019) months of therapy. Clinical remission was predicted by 1-month decreases in serum levels of C2C (P = 0.008) and C1,2C (P = 0.036). By regression analysis, 1-month changes in C2C, C1,2C, and CPII levels were independently associated with, and correctly predicted radiographic outcome in, 88% of the patients. CONCLUSION: Short-term changes in serum levels of collagen biomarkers following initiation of biologic therapy may better predict long-term clinical and radiographic outcomes. These collagen biomarkers may therefore be valuable new early indicators of short-term biologic treatment efficacy in clinical trials and in individual patients with inflammatory erosive arthritis

45. MUNCE SE, STANSFELD SA, BLACKMORE ERet STEWART DE: The role of depression and chronic pain conditions in absenteeism: results from a national epidemiologic survey, J.Occup.Environ.Med., Vol. 49(11), 1206-1211., 2007
    Organism:Women's Health Program, University Health Network, Toronto, Ontario, Canada sarahmunce@utorontocaFAU - Munce, Sarah E P
    Abstract:OBJECTIVE: This study examined whether depression is associated with absenteeism in a sample of individuals with chronic pain. METHODS: Data were obtained from the Canadian Community Health Survey Cycle 1.2. Key variables were chronic pain, defined as fibromyalgia, arthritis/rheumatism, back problems, and migraine headaches, absenteeism, and depression. The sample comprised 9,238,154 individuals who reported at least one chronic pain condition and were absent from their job in the previous week because of illness or disability. RESULTS: Nineteen percent of absent individuals met criteria for major depression versus 7.9% of non-absent individuals. The presence of major depression represented a three-fold risk of absenteeism. Other risk factors for absenteeism included younger age, higher income, and more education. CONCLUSIONS: Comorbid depression and chronic pain represents a significant source of disability in the workforce

46. NEROME Y, IMANAKA H, NONAKA Yet TAKEI S: Switching the therapy from etanercept to infliximab in a child with rheumatoid factor positive polyarticular juvenile idiopathic arthritis, Mod.Rheumatol., Vol. 17(6), 526-528., 2007
    Organism:Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan, nerome@mkufmkagoshima-uacjpFAU - Nerome, Yasuhito
    Abstract:Tumor necrosis factor alpha (TNFalpha)-blocking agents have been used increasingly in the treatment of severe refractory juvenile idiopathic arthritis (JIA). However, some patients have been forced to discontinue these agents because of the lack of efficacy or adverse events. In these situations, cases of switching from one TNF-blocking agent to another are reported in rheumatoid arthritis, but there are few cases in JIA. This report documents the case of a patient with JIA who improved following a switch from etanercept to infliximab

47. NEUBAUER P, WEBER AK, MILLER NHet MCCARTHY EF: Pigmented villonodular synovitis in children: a report of six cases and review of the literature, Iowa Orthop.J., Vol. 27, 90-94., 2007
    Organism:Department of Pathology and Orthopaedic Surgery, The Johns Hopkins Hospital, The Harry & Jeanette Weinberg Building, 401 N Broadway/Room 2242, Baltimore, MD 21231-2410, USAFAU - Neubauer, Philip
    Abstract:We report six children with pigmented villonodular synovitis. They ranged in age from seven to fifteen years. In four patients, the knee was involved. One patient had involvement of the ankle, and one had diffuse involvement along a metacarpal. In five cases, the diagnosis was not suspected clinically or radiographically, and the delay in making the correct diagnosis was as long as two years. Clinical diagnosis in these five patients was usually bacterial synovitis or juvenile rheumatoid arthritis. We feel that the diagnoses of pigmented villonodular synovitis should be considered in any child with chronic joint effusion

48. NISHIMOTO Net KISHIMOTO T: Humanized antihuman IL-6 receptor antibody, tocilizumab, Handb.Exp.Pharmacol., Vol. (181), 151-160., 2008
    Organism:Laboratory of Immune Regulation, Graduate School of Frontier Biosciences, Genentech, Inc, 1-3 Yamadaoka, Suita City, Osaka, Japan norihiro@fbsosaka-uacjpFAU - Nishimoto, N
    Abstract:Interleukin-6 (IL-6) is a pleiotropic cytokine that regulates immune responses and inflammatory reactions. Overproduction of IL-6 has been shown to play a role in inflammatory autoimmune diseases such as rheumatoid arthritis (RA), and juvenile idiopathic arthritis (JIA) and, therefore, an agent blocking IL-6 actions can be a therapy of these diseases. IL-6 belongs to a cytokine family, which shares the cytokine receptor subunit glycoprotein (gp) 130. This family also includes IL-11, oncostatin-M, and leukemia inhibitory factor (LIF). In the IL-6 receptor (IL-6R) system, both a membrane-bound IL-6R and a soluble form of IL-6R are able to mediate IL-6 signals into the cells through the interaction of gp130. Tocilizumab is a humanized antihuman IL-6 receptor antibody designed using genetic engineering technology. Tocilizumab recognizes both the membrane-bound and the soluble form IL-6R and specifically blocks IL-6 actions. Tocilizumab is expected to ameliorate the autoimmune inflammatory diseases with IL-6 overproduction and has been clinically developed as a therapeutic agent for RA, systemic-onset and articular types of JIA, Crohn's disease, etc. Tocilizumab has been shown to be effective not only for improving signs and symptoms but also for preventing joint destruction of RA. Immunopharmacology and clinical benefit of tocilizumab in RA is addressed

49. OLGAR S, ERTUGRUL T, DEVECIOGLU O, NISLI Ket OMEROGLU RE: Does red-man reaction stimulate macrophage activation syndrome in children with systemic juvenile idiopathic arthritis?, J.Rheumatol., Vol. 34(12), 2491-2494., 2007
    Organism:From the Pediatric Cardiology Unit and the Pediatric Hematology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, TurkeyFAU - Olgar, Seref
    Abstract:Macrophage activation syndrome (MAS) is a major cause of death in patients with systemic juvenile idiopathic arthritis (JIA). We describe 4 patients who developed MAS during or after vancomycin treatment. Vancomycin should be used with great care in patients with systemic JIA

50. PALMISANI E, SOLARI N, PISTORIO A, RUPERTO N, MALATTIA C, VIOLA S, BUONCOMPAGNI A, LOY A, MARTINI Aet RAVELLI A: Agreement between physicians and parents in rating functional ability of children with juvenile idiopathic arthritis, Pediatr.Rheumatol.Online.J., Vol. 5(1), 23, 2007
    Organism:ABSTRACT: OBJECTIVE: To investigate concordance between physicians and parents in rating the degree of functional ability of children with juvenile idiopathic arthritis (JIA). METHODS: The attending physician and a parent were asked to rate independently the level of physical functioning of 155 patients with disease duration > or = to 5 years on a 6-point scale ranging from 1=no disability (i.e. the child can do without difficulty all activities that children of his/her age can do) to 6=severe disability (i.e. all activities are difficult for the child). At study visit, measures of JIA activity and damage were assessed. Agreement was evaluated with weighted kappa (<0.40=poor agreement; 0.41-0.60=moderate agreement; 0.61-0.80=substantial agreement; >0.80 excellent agreement). Physician/parent evaluations were divided in 3 groups: 1) concordance; 2) parent over-rating= parent assessment over-rated relative to physician assessment; 3) physician over-rating= physician assessment over-rated relative to parent assessment. Factors affecting concordance/discordance were evaluated by means of Kruskal-Wallis or Chi-square/Fisher exact test. RESULTS: Concordance, parent over-rating and physician over-rating were observed in 107 (69%), 29 (18.7%) and 19 (12.3%) evaluations, respectively. Kappa value was 0.69. Parent over-rating was associated with greater intensity of pain (p=0.01) and higher Childhood Health Assessment Questionnaire (C-HAQ) score (p=0.004), whereas physician over-rating was associated with more severe joint disease (p=0.04 to <0.001), higher C-reactive protein (p=0.03) higher frequency of Steinbrocker functional class > or = to II (p<0.001), and greater articular damage, as measured with the Juvenile Arthritis Damage Index (p<0.001). CONCLUSION: Physicians and parents revealed fair concordance in rating functional ability of children with JIA. Parent over-rating was associated with greater child's pain and worse C-HAQ score, whereas physician over-rating was associated with greater severity of joint inflammation and damage

51. PELOSCHEK P, LANGS G, WEBER M, SAILER J, REISEGGER M, IMHOF H, BISCHOF Het KAINBERGER F: An automatic model-based system for joint space measurements on hand radiographs: initial experience, Radiology., Vol. 245(3), 855-862., 2007
    Organism:Department of Diagnostic Radiology, Medical University of Vienna, Vienna, AustriaFAU - Peloschek, Philipp
    Abstract:This ethics committee-approved pilot study was performed with informed consent. A Web-based service that was developed for automated measurement of joint space and automatic analysis of radiographs was tested prospectively. A total of 160 metacarpophalangeal joint spaces were measured in 20 patients (average age, 48 years; age range, 18-89 years; 16 women) suspected of having rheumatoid arthritis or osteoarthritis. The technical success rate was 93%. The smallest detectable difference in repeated automatic joint space width measurements varied from 0.08 to 0.31 mm, and the coefficient of variation was 2%-7%. Compared with the reference standard (interactive segmentation of the joint space widths) measurements, results were within a mean error of 0.19-0.40 mm. The proposed Web-based service enables reproducible joint space measurements to be obtained in metacarpophalangeal joints with moderate erosive and osteophytic disease

52. PREDA M, DAMIAN C, MANESCU R, DAVIDESCU L, IRIMIA Aet SOLLOSY M: [Anterior uveitis in a juvenile cronic artritis, oligoarticular form], Oftalmologia., Vol. 51(3), 58-61., 2007
    Organism:Clinica de Oftalmologie, Spitalul Clinic de Urgenta CraiovaFAU - Preda, Mirela
    Abstract:We present the case of a 16-year-old male teen-ager admitted in the Ophthalmology Clinic Craiova, diagnosed with Anterior neglected acute uveitis in the left eye. Before and simultaneous with the ocular manifestations, the patient complained of pain on the hips joints. The pediatric examination, based on the clinical features and the laboratory findings, diagnosed him with Juvenile chronic arthritis, oligoarthritis type. The evolution was good under the corticosteroid therapy

53. RABINOVICH CE: Use of tumor necrosis factor inhibitors in uveitis, Curr.Opin.Rheumatol., Vol. 19(5), 482-486., 2007
    Organism:Division of Pediatric Rheumatology, Duke University Medical Center, Durham, North Carolina 27710, USA rabin001@mcdukeeduFAU - Rabinovich, C Egla
    Abstract:PURPOSE OF REVIEW: Use of tumor necrosis factor-alpha blocking agents to treat chronic pediatric uveitis is becoming recognized as an important therapeutic modality. This review summarizes the rationale for this use, highlighting new studies of these agents in pediatric uveitis. RECENT FINDINGS: The majority of patients with pediatric uveitis either have idiopathic uveitis or uveitis associated with juvenile idiopathic arthritis. Ophthalmologic morbidity among these children is common. Most studies evaluating tumor necrosis factor-alpha blockade in pediatric uveitis are retrospective case series, with attendant limitations that are inherent to any retrospective study. Study of uveitis has been hampered by lack of standardization of disease and outcome measures, which has been addressed by uveitis experts with publication of consensus measures. Data to date suggest that tumor necrosis factor-alpha blockade is efficacious in refractory uveitis. Agents with direct tumor necrosis factor-alpha membrane receptor binding activity may be the most efficacious. There remain many unanswered questions in the treatment of pediatric uveitis, including optimal dosing regimen and long-term efficacy. SUMMARY: Tumor necrosis factor-alpha blocking agents play an important role in the treatment of chronic pediatric uveitis. Prospective comparative studies are needed so that we may better understand this role

54. RAVELLI A, IOSELIANI M, NORAMBUENA X, SATO J, PISTORIO A, ROSSI F, RUPERTO N, MAGNI-MANZONI S, ULLMANN Net MARTINI A: Adapted versions of the Sharp/van der Heijde score are reliable and valid for assessment of radiographic progression in juvenile idiopathic arthritis, Arthritis Rheum., Vol. 56(9), 3087-3095., 2007
    Organism:Universita degli Studi di Genoa, and IRCCS, Istituto G Gaslini, Genoa, Italy angeloravelli@ospedale-gaslinigeitFAU - Ravelli, Angelo
    Abstract:OBJECTIVE: To develop adapted versions of the Sharp/van der Heijde radiographic scoring system for use in juvenile idiopathic arthritis (JIA), and to investigate their validity in JIA patients with polyarticular disease. METHODS: The study group comprised 177 patients with polyarticular JIA. Radiographs of the wrist/hand of each patient were obtained at baseline (first observation) and then at 1, 3, 5, 7/8, and 10 years and were assessed independently by 2 pediatric rheumatologists according to different adaptations of the Sharp/van der Heijde method. To facilitate score assignment, the radiograph for each patient was compared with a bone age-related standard. Validation procedures included analysis of reliability, construct validity, and score progression over time. RESULTS: Interobserver and intraobserver agreement on longitudinal score values and score changes was good for all of the adapted scoring versions (intraclass correlation coefficient >0.85). Score changes over time were moderately to strongly correlated with the clinical indicators of long-term joint damage and with the amount of long-term radiographic damage as measured with the carpo:metacarpal ratio, thereby demonstrating good construct validity. A steady increase in scores over time was observed, with joint space narrowing being the most common form of damage throughout the disease course. The inclusion of 5 new areas appeared to increase the overall construct validity of erosion scores. CONCLUSION: Our results show that the adapted versions of the Sharp/van der Heijde score are reliable and valid for the assessment of radiographic progression in patients with JIA

55. REINA SD, DEL BLANCO J, BONET M, CASTANO C, CLAVAGUERA T, MATEO L, ROIG VD, RUIZ JMet RODRIGUEZ MJ: [Functional impairment in psoriatic arthritis. Multicentric study of 343 patients], Med.Clin.(Barc.)., Vol. 129(6), 201-204., 2007
    Organism:Servicio de Reumatologia, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Espana deliareinasanz@yahoocomFAU - Reina Sanz, Delia
    Abstract:BACKGROUND AND OBJECTIVE: The study is aimed at improving our knowledge about the functional impairment of the psoriatic arthritis through a multicentral series. PATIENTS AND METHOD: We have designed a transversal and multicentral study (centers of the same geographical area), including 343 patients with psoriatic arthritis. Eight medical centers have participated. Patients have been divided depending on the assistential level where they are visited. We have collected the following data: sex, age, assistential level, duration of psoriasis and arthritis, age at onset of psoriasis and arthritis, clinical form, ARA functional impairment, number of tender and swollen joints, presence of dactylitis, distal interphalangeal affection, axial involvement, ostheolisis or nail lesions, erithrocyte sedimentation rate (ESR), C-reactive protein, hemoglobine, leucocites, platelets, HLA-B27 and rheumatoid factor. RESULTS: 7.14% of the patients were significatly disabled (ARA functional class III and IV). 30.32% were patients visited in a primary assistential level, 30.90% in a secondary assistential level and 38.78% a tertiary and universitary hospital. We found statistically significant correlation between III and IV functional classes and age, assistential level, ostheolisis, corticoid treatment, ESR, leucocites, platelets and number of tender joints. CONCLUSIONS: We find a better functional capacity in our patients than in other studies. The inclusion of patients from different assistential levels instead of just patients visited in a tertiary hospital might be the cause of this difference

56. RINGOLD Set WALLACE CA: Measuring clinical response and remission in juvenile idiopathic arthritis, Curr.Opin.Rheumatol., Vol. 19(5), 471-476., 2007
    Organism:Department of Rheumatology, Children's Hospital and Regional Medical Center/University of Washington, Seattle, Washington, USAFAU - Ringold, Sarah
    Abstract:PURPOSE OF REVIEW: The increasing availability of new medications for the treatment of juvenile idiopathic arthritis has made the accurate assessment of treatment outcomes critically important. The purpose of this review is to describe recent investigations focused on the development of new outcome measures in the domains of disease activity and joint damage, and to summarize recently published data within the area of health-related quality of life. RECENT FINDINGS: Since the development of the preliminary definition of disease improvement in 1997, the American College of Rheumatology pediatric response criteria have become the primary outcome measures in therapeutic trials in juvenile idiopathic arthritis. Additional definitions, including preliminary definitions of flare and remission have subsequently been added. Investigations have also sought to determine whether measures currently in use in adult rheumatoid arthritis might have utility in juvenile idiopathic arthritis. As the pathogenesis of juvenile idiopathic arthritis becomes better understood, biomarkers have significant potential as outcome measures. Lastly, recent reports regarding the health-related quality of life in large cohorts of children with juvenile idiopathic arthritis are important in guiding investigators towards areas most in need of improved treatment. SUMMARY: Significant progress has been made in the measurement of outcomes in juvenile idiopathic arthritis. Outcome measures will continue to be designed and tested to keep pace with the development of new therapies and the improved understanding of the disease pathogenesis

57. ROSSATO LM, ANGELO Met SILVA CA: Care delivery for the child to grow up despite the pain: the family's experience, Rev.Lat.Am.Enfermagem., Vol. 15(4), 556-562., 2007
    Organism:rossato@uspbrFAU - Rossato, Lisabelle Mariano
    Abstract:This study aimed to understand the meaning of the experience of families having a child experiencing pain due to Juvenile Rheumatoid Arthritis and to construct a theoretical model representing this experience. Grounded Theory and Symbolic Interactionism were used as methodological framework and theoretical framework, respectively. Data were collected by semistructured interviews with 12 families. Data analysis allowed for the construction of the theoretical model Caring for the child to grow despite the pain, which describes an experience based on motivational elements: wanting to see the child without pain and wanting to see the child live a normal life, reviewing how the family lives the transition in its development cycles, retaking and integrating them in the family dynamic with the appearance of the disease and pain in the child. This theoretical model provides a framework for teaching, research and care, permitting advances in terms of theoretical nursing knowledge

58. RUPERTO N, LOVELL DJ, CUTTICA R, WILKINSON N, WOO P, ESPADA G, WOUTERS C, SILVERMAN ED, BALOGH Z, HENRICKSON M, APAZ MT, BAILDAM E, FASTH A, GERLONI V, LAHDENNE P, PRIEUR AM, RAVELLI A, SAURENMANN RK, GAMIR ML, WULFFRAAT N, MARODI L, PETTY RE, JOOS R, ZULIAN F, MCCURDY D, MYONES BL, NAGY K, REUMAN P, SZER I, TRAVERS S, BEUTLER A, KEENAN G, CLARK J, VISVANATHAN S, FASANMADE A, RAYCHAUDHURI A, MENDELSOHN A, MARTINI Aet GIANNINI EH: A randomized, placebo-controlled trial of infliximab plus methotrexate for the treatment of polyarticular-course juvenile rheumatoid arthritis, Arthritis Rheum., Vol. 56(9), 3096-3106., 2007
    Organism:IRCCS, Istituto G Gaslini, Genoa, Italy nicolaruperto@ospedale-gaslinigeitFAU - Ruperto, Nicolino
    Abstract:OBJECTIVE: To evaluate the safety and efficacy of infliximab in the treatment of juvenile rheumatoid arthritis (JRA). METHODS: This was an international, multicenter, randomized, placebo-controlled, double-blind study. One hundred twenty-two children with persistent polyarticular JRA despite prior methotrexate (MTX) therapy were randomized to receive infliximab or placebo for 14 weeks, after which all children received infliximab through week 44. Patients received MTX plus infliximab 3 mg/kg through week 44, or MTX plus placebo for 14 weeks followed by MTX plus infliximab 6 mg/kg through week 44. RESULTS: Although a higher proportion of patients in the 3 mg/kg infliximab group than in the placebo group had achieved responses according to the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30) criteria for improvement at week 14 (63.8% and 49.2%, respectively), the between-group difference in this primary efficacy end point was not statistically significant (P = 0.12). By week 16, after the crossover from placebo to infliximab 6 mg/kg when all patients were receiving infliximab, an ACR Pedi 30 response was achieved in 73.2% of all patients. By week 52, ACR Pedi 50 and ACR Pedi 70 responses had been reached in 69.6% and 51.8%, respectively, of patients. Infliximab was generally well tolerated, but the safety profile of infliximab 3 mg/kg appeared less favorable than that of infliximab 6 mg/kg, with more frequent occurrences of serious adverse events, infusion reactions, antibodies to infliximab, and newly induced antinuclear antibodies and antibodies to double-stranded DNA observed with the 3 mg/kg dose. CONCLUSION: While infliximab at 3 mg/kg and 6 mg/kg showed durable efficacy at 1 year, achievement of the primary efficacy end point at 3 months did not differ significantly between infliximab-treated and placebo-treated patients. Safety data indicated that the 6-mg/kg dose may provide a more favorable risk/benefit profile. These results warrant further investigation in children with JRA

59. SANCHEZ-CANO D, CALLEJAS-RUBIO JL, RIOS-FERNANDEZ Ret ORTEGO-CENTENO N: [CINCA syndrome (chronic, children, neurologic, cutaneous and joints)], Med.Clin.(Barc.)., Vol. 129(5), 200, 2007
    Organism:Unidad de Enfermedades Autoinmunes Sistemicas, Hospital Clinico San Cecilio, Granada, EspanaFAU - Sanchez-Cano, Daniel
    Abstract:

60. SHAPIRO C, MAENZ L, HOSSAIN A, PAHWA Pet ROSENBERG A: Onset to first visit intervals in childhood rheumatic diseases, J.Rheumatol., Vol. 34(9), 1913-1917., 2007
    Organism:Department of Pediatric and Community Health and Epidemiology, University of Saskatchewan, Saskatoon, Saskatchewan, CanadaFAU - Shapiro, Cal
    Abstract:OBJECTIVE: To determine time intervals between onset of symptoms of a childhood rheumatic disease and first visit to a pediatric rheumatology clinic and to evaluate factors influencing onset to first visit intervals. METHODS: Onset to first visit intervals were analyzed in 836 children representing the 10 most common diseases in a pediatric rheumatology clinic population of 1093. RESULTS: Among 836 subjects, 469 (56.1%) could identify month of symptom onset. Among patients with juvenile rheumatoid arthritis (JRA) 125 of 195 (64.1%) with pauciarticular, 58 of 105 (55.2%) with polyarticular, and 28 of 36 (77.8%) with systemic subtypes were able to determine time interval between symptom onset and first visit. Month intervals were confidently established in 80 of 250 with a spondyloarthropathy (32.4%), 19 of 52 (36.5%) with psoriatic arthropathy, 65 of 72 (90.3%) with Henoch-Schonlein purpura (HSP), 50 of 56 (89.3%) with Kawasaki disease, 22 of 34 (64.7%) with systemic lupus erythematosus, 13 of 18 (72.2%) with dermatomyositis, and 9 of 18 (50%) with localized scleroderma. Determination of onset was significantly more likely in HSP than in other diagnostic categories except systemic JRA, and more likely in Kawasaki disease than other disease categories except systemic JRA and dermatomyositis. In the group of 469, 287 (61.2%) were seen within 2 months of symptom onset and 447 (95.3%) within 1 year of symptom onset. CONCLUSION: Diseases ordinarily typified by an abrupt and acute onset of symptoms were referred most promptly, suggesting that acuity of symptoms at disease onset is the factor that most influences promptness of referral. Prospective studies are required to establish how onset to first visit intervals might influence disease outcomes and to devise best practice referral guidelines

61. SHARMA H, RANA B, MAHENDRA A, JANE MJet REID R: Outcome of 17 pigmented villonodular synovitis (PVNS) of the knee at 6 years mean follow-up, Knee., Vol. 14(5), 390-394., 2007
    Organism:Bone and Soft Tissue Tumour Service, Western Infirmary, Glasgow, G11 6NT, UK hksharma1@aolcomFAU - Sharma, H
    Abstract:Between January 1950 and December 2000, 16 patients were identified from Scottish Bone Tumour Registry with 17 histologically proven pigmented villonodular synovitis (PVNS) of the knee. The mean follow-up was 6 years (range, 1-14 years). A knee swelling of chronic duration with dull ache was the common presenting symptom. The mean duration of symptoms prior to presentation was 25 months (1-108 months), however it was much less (mean, 7 months) in four patients with a history of trauma. The mean age was 33 years (range, 16-58 years) with a slight male predominance. The lesion was predominantly anterior in nine patients, posterior in four, anterolateral in two, and medial and lateral in one each. Three patients (four knees) had localised disease and 13 diffuse. Anteroposterior and lateral radiographs of the knee revealed normal findings in 11 patients, features of gonarthrosis in four and a large suprapatellar loose body in one patient (both knees). Open (incisional-eight, excisional-eight) biopsy was carried out in all and all were histologically confirmed as PVNS. Removal of a localised synovial mass or loose body with surrounding partial synovectomy (four) was carried out for the localised variety, whilst open partial (three) or total (radical) synovectomy (10) was performed in all cases of diffuse PVNS. Three of seventeen knees had a recurrence, noted at 4, 6 and 8 years postoperatively (0% - localised, 23% - diffuse variety). A total (radical) synovectomy should be considered in diffuse PVNS in order to obtain optimal outcome

62. SHAW KL, SOUTHWOOD TRet MCDONAGH JE: Young people's satisfaction of transitional care in adolescent rheumatology in the UK, Child Care Health Dev., Vol. 33(4), 368-379., 2007
    Organism:Institute of Child Health, University of Birmingham, Birmingham, UKFAU - Shaw, K L
    Abstract:BACKGROUND: To examine the quality of transitional health care from the perspectives of young people with juvenile idiopathic arthritis (JIA) and their parents. METHODS: Adolescents with JIA and their parents were recruited from 10 major UK rheumatology centres. Satisfaction with health-care delivery was measured prior to, and 12 months after, the implementation of a structured and co-ordinated programme of transitional care using self-completed questionnaires designed for this study. RESULTS: Of 359 families invited to participate, 308 (86%) adolescents with JIA and 303 (84%) parents/guardians accepted. A fifth of adolescents had persistent oligoarthritis. Median age was 14.2 (11-18) years with median disease duration of 5.7 (0-16) years. Young people and their parents rated provider characteristics more important than aspects of the physical environment or process issues. Staff honesty and knowledge were rated as the most essential aspects of best practice. Prior to implementing the programme of transitional care, parents rated service delivery for all items significantly worse than best practice. Overall satisfaction improved 12 months after entering the programme. However, while parent satisfaction improved for 70.4% of items, significant improvements were only observed for three (13.6%) items rated by adolescents. CONCLUSION: The perceived quality of health care for young people with JIA and their parents was significantly lower than what they would like. Satisfaction with many aspects of care during transition from paediatric to adult services can be improved through the implementation of a structured, co-ordinated programme of transitional care

63. SHAW KL, SOUTHWOOD TRet MCDONAGH JE: Development and preliminary validation of the 'Mind the Gap' scale to assess satisfaction with transitional health care among adolescents with juvenile idiopathic arthritis, Child Care Health Dev., Vol. 33(4), 380-388., 2007
    Organism:Institute of Child Health, University of Birmingham, Birmingham, UKFAU - Shaw, K L
    Abstract:BACKGROUND: To develop a scale to assess satisfaction with transitional health care among adolescents with a chronic illness and their parents. METHODS: The 'Mind the Gap' scale was developed using evidence from a previous needs assessment, in three stages: (1) definition of the construct; (2) design of the scale items, response options and instructions; (3) full administration of the scale, item analysis and dimensionality analysis. The scale was administered to 308 adolescents with juvenile idiopathic arthritis (JIA) and 303 parents/guardians, prior to and 12 months after the implementation of an evaluation of a structured and co-ordinated programme of transitional care. The patient population involved adolescents with JIA and their parents recruited from 10 major UK rheumatology centres. RESULTS: A total of 301 (97.7%) adolescents and 286 (95.0%) parents chose to complete the questionnaire, with median item completion rates of 100.0% (0-100%) for both adolescents and parents thus confirming feasibility. Face and content validity were confirmed. Factor analyses revealed a three-factor structure which explained 49.5% and 56.1% of the variation in adolescent and parent scores respectively. The internal consistency of each subscale ('management of environment', 'provider characteristics' and 'process issues') was indicated by Cronbach's alphas of 0.71, 0.89 and 0.89 for adolescents, respectively, and 0.83, 0.91 and 0.92 for parents respectively. Cronbach's alphas for the entire scales were 0.91 and 0.94 for the adolescent and parent forms respectively. CONCLUSION: These preliminary results report the potential of the 'Mind the Gap' scale in evaluating transitional care for adolescents with JIA. In view of the generic nature of transitional care reflected in the scale, this scale has wider potential for use with adolescents with other chronic illness in view of the generic nature of transition. This development is particularly timely in the context of transitional care developments in the UK and further validation of the scale is in progress

64. SINGH-GREWAL D, SCHNEIDERMAN-WALKER J, WRIGHT V, BAR-OR O, BEYENE J, SELVADURAI H, CAMERON B, LAXER RM, SCHNEIDER R, SILVERMAN ED, SPIEGEL L, TSE S, LEBLANC C, WONG J, STEPHENS Set FELDMAN BM: The effects of vigorous exercise training on physical function in children with arthritis: a randomized, controlled, single-blinded trial, Arthritis Rheum., Vol. 57(7), 1202-1210., 2007
    Organism:The Hospital for Sick Children, Toronto, Ontario, CanadaFAU - Singh-Grewal, Davinder
    Abstract:OBJECTIVE: To examine the effectiveness of high-intensity aerobic training compared with low-intensity training in terms of energy cost of locomotion, peak oxygen uptake, peak power, and self-reported physical function in children with juvenile idiopathic arthritis (JIA). METHODS: Eighty children with JIA, ages 8-16 years, were enrolled in a randomized, single-blind controlled trial. Both groups participated in a 12-week, 3-times-weekly training program consisting of high-intensity aerobics in the experimental group and qigong in the control group. Subjects underwent exercise testing measuring submaximal oxygen uptake at 3 km/hour (VO(2submax)) as the primary outcome, maximal oxygen uptake, and peak power at the beginning and end of the program. Physical function was measured using the Child Health Assessment Questionnaire (C-HAQ). RESULTS: The exercise program was well tolerated in both groups. There was no difference in VO(2submax) or any other exercise testing measures between the groups through the study period and no indication of improvement. Both groups showed significant improvements in C-HAQ with no difference between the groups. Adherence was higher in the control group than the experimental group. CONCLUSION: Our findings suggest that activity programs with or without an aerobic training component are safe and may result in an important improvement in physical function. The intensity of aerobic training did not seem to provide any additional benefits, but higher adherence in the qigong program may suggest that less intensive regimens are easier for children with JIA to comply with, and provide a degree of benefit equivalent to more intensive programs

65. STICHERLING M, MINDEN K, KUSTER RM, KRAUSE Aet BORTE M: [Psoriasis und Psoriasis arthritis in childhood and adolescence. Overview and consensus statement of the 9th Worlitz Expert Round Table Discussion 2006 for the Society for Child and Adolescent Rheumatology], Z.Rheumatol., Vol. 66(4), 349-354., 2007
    Organism:Hautklinik, Universitatsklinikum Erlangen, Hartmannstrasse 14, 91052 Erlangen michaelsticherling@dermaimeduni-erlangendeFAU - Sticherling, M
    Abstract:There are about 1.2-1.6 million psoriasis sufferers in Germany. In about a third of these, the disease manifests before the age of 20. A classic complication of psoriasis is psoriasis arthritis (PsA), which, from the latest figures, effects about 20% of all psoriasis patients. PsA also starts in childhood and is included under the term juvenile idiopathic arthritis (JIA). The expert round table discussion which took place in 2006 in Worlitz elaborated the recommendations for the classification, comprehensive diagnostics and therapy of effected children and adolescents. As controlled studies are lacking, the treatment of PsA has been empirically based and carried out in analogy with the treatment of other forms of JIA. The use of methotrexate (MTX) shows a good success rate. In 2004, about a third of the patients found in the core documentation, including over 80% of children and adolescents undergoing a primary therapy, were treated with MTX, a quarter in combination with other medication. A total of 7% of all and 16% of those undergoing primary therapy were treated with etanercept, most (>80%) in combination with basis medication, usually MTX. Consensus opinion indicated that an early, and intensive local skin therapy should be applied in order to reduce inflammatory activity. If PsA is present, the early use of non-steroid anti-inflammatories as well as local therapy of the joints with the intra-articular application of glucocorticosteroids is recommended. The primary medication should preferentially be MTX, if necessary combined with other therapies. In cases of a severe, episodic progression as well as high inflammatory activity, systemic glucocorticosteroids should be considered. Further studies addressing both the clinical course of jPsA compared to the adult manifestation as well as optimal therapeutic procedures should be initiated in the near future

66. 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., 2007
    Organism:University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada
    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

67. 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., 2007
    Organism:Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
    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

68. TURKEL Set PAO M: Late consequences of chronic pediatric illness, Psychiatr.Clin.North Am., Vol. 30(4), 819-835., 2007
    Organism:Childrens Hospital Los Angeles, University of Southern California Keck School of Medicine, 4650 Sunset Blvd #82, Los Angeles, CA 90027, USAFAU - Turkel, Susan
    Abstract:There are many challenges in coping with and adapting to life with a chronic disease, and increased survival cannot be assumed to be associated with increased quality of life. A recent systematic review shows there is wide variation in outcomes depending on the definitions and measurements used to estimate the prevalence of chronic health conditions, making the impact of disability on children's health and social functioning difficult to assess; various authors have called for an international consensus about the conceptual definition of chronic health conditions in childhood. It frequently is difficult to determine if problems in psychosocial functioning are caused by the underlying illness, by treatment, or by the resultant effects of either illness or treatment on physical growth or cognitive development. Assessment and treatment of mental health should be an integral component of the comprehensive care of chronically ill children and adolescents. Transition of care is an important process that addresses significant changes from child-oriented to adult-oriented care. Adults who have chronic health conditions should continue to be evaluated periodically for late consequences of the childhood illness and early medical care, and attention should be paid to their ongoing psychosocial, psychiatric, educational, and vocational needs

69. TYLER LN, HARVILLE TOet BLACKALL DP: Multiple alloantibodies after transfusion in an infant treated with infliximab, N.Engl.J.Med., Vol. 357(20), 2092-2093., 2007
    Organism:

70. UEECK BA, MAHMUD NAet MYALL RW: Dealing with the effects of juvenile rheumatoid arthritis in growing children, Oral Maxillofac.Surg.Clin.North Am., Vol. 17(4), 467-473., 2005
    Organism:Oral and Maxillofacial Surgery, Oregon Health and Science University, 611 SW Campus Drive, Portland, OR 97239, USAFAU - Ueeck, Brett A
    Abstract:Juvenile rheumatoid arthritis is a chronic childhood disease that has a multiplicity of effects on the growth and development of the facial skeleton. An understanding of the disease in general and its therapy is necessary for successful surgical-orthodontic care

71. VANG T, MILETIC AV, BOTTINI Net MUSTELIN T: Protein tyrosine phosphatase PTPN22 in human autoimmunity, Autoimmunity., Vol. 40(6), 453-461., 2007
    Organism:The Burnham Institute for Medical Research, La Jolla, CA 92037, USAFAU - Vang, Torkel
    Abstract:The discovery that a single-nucleotide polymorphism (SNP) in lymphoid tyrosine phosphatase (LYP), encoded by the PTPN22 gene, is associated with type 1 diabetes (T1D) has now been verified by numerous studies and has been expanded to rheumatoid arthritis, juvenile rheumatoid arthritis (JRA), systemic lupus erythematosus, Graves' disease, generalized vitiligo and other human autoimmune diseases. In this paper, we discuss the association of PTPN22 with autoimmunity, the biochemistry of the PTPN22-encoded phosphatase, and the molecular mechanism(s) by which the disease-predisposing allele contributes to the development of human disease

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

73. WARD TM, BRANDT P, ARCHBOLD K, LENTZ M, RINGOLD S, WALLACE CAet LANDIS CA: Polysomnography and Self-reported Sleep, Pain, Fatigue, and Anxiety in Children with Active and Inactive Juvenile Rheumatoid Arthritis, J.Pediatr.Psychol., Vol. ., 2007
    Organism:Biobehavioral Nursing and Health Systems, Department of Family and Child Nursing, School of Nursing, and Department of Pediatrics, Immunology, Rheumatology & Infect Disease, School of Medicine, University of Washington
    Abstract:OBJECTIVE: To compare polysomnography (PSG) and self-reported sleep, symptoms (pain and fatigue), and anxiety between children with active and inactive juvenile rheumatoid arthritis (JRA) and examine relations among sleep, symptoms, and anxiety. METHODS: Two consecutive nights of PSG, self-reported sleep, and symptoms were obtained in 70 children 6-11 years of age with active (n = 35) or inactive (n = 35) JRA. RESULTS: On the second (study) night, PSG and self-reported sleep variables were not different, but pain and fatigue were significantly higher (both p <.02) in children with active compared to inactive disease. In a stepwise regression, age, medications, disease status, anxiety, evening pain, total sleep time, and arousals explained 36% of the variance in fatigue and age, disease status, and evening pain were significant (all p <.04) predictors of fatigue. All children showed longer sleep latency and reduced sleep efficiency on the first night in the laboratory. CONCLUSIONS: Sleep was not altered in children with active JRA, however, the "first night effect" suggests that valid laboratory sleep assessments require an adaptation night

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

75. YOUM JY, WOO JH, KIM TH, BAE SCet YOO DH: Interleukin-1beta and interleukin-1 receptor antagonist gene polymorphisms in Korean patients with adult-onset Still's disease, Scand.J.Rheumatol., Vol. 36(5), 390-393., 2007
    Organism:Division of Rheumatology, Department of Medicine, College of Medicine, The Hospital for Rheumatic Disease, Hanyang University, Seoul, South KoreaFAU - Youm, J-Y
    Abstract:OBJECTIVE: Interleukin-1 (IL-1) has been implicated in the pathogenesis of several rheumatic inflammatory diseases, including adult-onset Still's disease (AOSD) and systemic-onset juvenile idiopathic arthritis (SoJIA). Several clinical trials also suggest that anakinra, a human recombinant interleukin-1 receptor antagonist (IL-1Ra), is effective in patients with AOSD and SoJIA. We have therefore investigated whether IL-1beta and IL-1Ra gene polymorphisms are associated with the development and clinical features of AOSD. METHODS: Genomic DNA was isolated from 83 AOSD patients and 144 healthy controls. Genotyping of the two IL-1beta gene (IL-1B+3954 and IL-1B-511) polymorphisms was performed using polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP). Genotyping of the IL-1Ra gene (intron 2, VNTR) polymorphism was performed using PCR-based analysis. To compare genotype and allele frequencies, the chi2-test or Fisher's exact test was used. Haplotype frequencies and pairwise linkage disequilibrium were also estimated. A p-value <0.05 was considered significant. RESULTS: There were no significant differences in the genotype and allele frequencies of the IL-1beta and IL-1Ra gene polymorphisms. No differences were also found in the IL-1 gene cluster haplotypes between both groups. IL-1 gene cluster polymorphisms had no effect on the clinical course and joint involvement pattern. Nevertheless, the IL-1B-511 and IL-1RN (VNTR) polymorphic sites were in linkage disequilibrium. CONCLUSION: These results suggest that IL-1beta and IL-1Ra gene polymorphisms are not associated with the development and clinical features of AOSD in Korean patients

76. ZEBRACKI K, HOLZMAN K, BITTER KJ, FEEHAN Ket MILLER ML: Brief report: use of complementary and alternative medicine and psychological functioning in Latino children with juvenile idiopathic arthritis or arthralgia, J.Pediatr.Psychol., Vol. 32(8), 1006-1010., 2007
    Organism:Loyola University Chicago, Department of Psychology, IL 60626, USA kzebrac@luceduFAU - Zebracki, Kathy
    Abstract:OBJECTIVE: To describe the use of complementary and alternative medicine (CAM) and its relationship to symptoms of anxiety, depression, and dysthymia in Latino children with juvenile idiopathic arthritis (JIA) or arthralgia. METHODS: Parents of 36 children between the ages of 6 and 16 years with either JIA (n = 17) or arthralgia (n = 19) completed questionnaires during routine pediatric rheumatology clinic visits assessing use of CAM and psychological functioning. RESULTS: CAM was used by the majority of children primarily to treat pain episodes. The most common modalities were prayer and massage therapy. CAM use was associated with decreased symptoms of anxiety and dysthymia in children with arthralgia, but not in children with JIA. CONCLUSION: Preliminary findings suggest that CAM use is associated with improved psychological functioning in children with arthralgia. Healthcare providers are encouraged to routinely screen for CAM usage and to educate families about the potential benefits and limitations of CAM

77. 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 200001, ChinaFAU - Zhao, Yu
    Abstract: