Bibliography December 2006

1. ALVARADO-SANCHEZ B, HERNANDEZ-CASTRO B, PORTALES-PEREZ D, BARANDA L, LAYSECA-ESPINOSA E, ABUD-MENDOZA C, CUBILLAS-TEJEDA ACet GONZALEZ-AMARO R: Regulatory T cells in patients with systemic lupus erythematosus, J.Autoimmun., Vol. 27(2), 110-118., 2006
   Organism:Departamento de Inmunologia, Facultad de Medicina, UASLP, Ave V Carranza 2405, 78210 San Luis Potosi, SLP, MexicoFAU - Alvarado-Sanchez, Brenda
   Abstract:Regulatory T cells have an important role in the control of self-reactivity, and in the pathogenesis of autoimmune inflammatory conditions. The aim of this work was to perform a quantitative and functional analysis of regulatory T cells in patients with systemic lupus erythematosus (SLE). We studied twenty-three patients with SLE (19 active, 4 inactive), and twenty-seven healthy subjects as well as fifteen patients with rheumatoid arthritis (RA). The following cell subsets were analyzed in peripheral blood mononuclear cells by flow cytometry: CD4+CD25+, CD4+CD25(bright), CD4+Foxp3+ (Treg cells), CD8+CD28- (Ts cells), CD4+IL-10+ (Tr1 cells), and CD4+TGF-beta+ (Th3 cells). In addition, the in vitro suppressive activity of CD4+CD25+ lymphocytes was tested. We found no significant differences in the levels of all regulatory cell subsets studied in SLE patients compared to controls and RA patients. However, a defective regulatory function of CD4+CD25+T cells was observed in a significant fraction (31%) of patients with SLE. Our data indicate that although approximately one third of patients with SLE show an abnormal immunosuppressive function of Treg lymphocytes, their levels of the different regulatory T cell subsets in peripheral blood are not significantly different from those found in controls

2. ARABSHAHI Bet CRON RQ: Temporomandibular joint arthritis in juvenile idiopathic arthritis: the forgotten joint, Current Opinion in Rheumatology, Vol. 18, 490-495., 2006
   Organism:Childrens Hosp Philadelphia, Ctr Childhood Arthritis and Rheumat Dis, Div Rheumatol, 3615 Civic Ctr Blvd,ARC 1102B, Philadelphia, PA 19104 USA USA
   Abstract:Purpose of review This review explores the prevalence, clinical and radiographic signs, and treatment of temporomandibular joint arthritis in children with juvenile idiopathic arthritis.Recent findings Temporomandibular joint arthritis seems to be a more frequent manifestation in patients with juvenile idiopathic arthritis than previously believed, in part due to the paucity of clinical symptoms and poor sensitivity of conventional radiographs used for diagnosis. Antinuclear antibody positivity, early onset of disease, and presence of systemic or polyarticular disease are all risk factors for temporomandibular joint arthritis but may underpredict temporomandibular joint involvement in juvenile idiopathic arthritis. Magnetic resonance imaging enhanced with gadolinium is currently the gold standard in detection of temporomandibular joint arthritis, and treatment with intra-articular corticosteroids has been shown to be effective and safe, with minimal side effects.Summary Given the paucity of clinical symptoms in temporomandibular joint arthritis, detection of temporomandibular joint inflammation using contras t-enhanced magnetic resonance imaging is essential for instituting appropriate therapy in a timely fashion. The use of intra-articular corticosteroids holds promise for control of temporomandibular joint inflammation and prevention of associated morbidities

3. BERDELI A, OZYUREK AR, ULGER Z, GURSES D, LEVENT E, SALAR Ket GURPINAR AR: Association of macrophage migration inhibitory factor gene -173 G/C polymorphism with prognosis in Turkish children with juvenile rheumatoid arthritis, Rheumatol.Int., Vol. 26(8), 726-731., 2006
   Organism:Department of Pediatrics, Laboratory of Molecular Medicine, Ege University School of Medicine, Bornova, 35100, SSK Tepecik Hospital, Izmir, Turkey afig@medegeedutrFAU - Berdeli, Afig
   Abstract:The objectives of this study were to determine genotypic and allelic frequencies of macrophage migration inhibitory factor (MIF) gene -173 G/C polymorphism in patients with juvenile rheumatoid arthritis (JRA) and to evaluate the association of the MIF -173 C allele with the outcome of JRA. Genomic DNA was collected from 67 JRA patients and 153 healthy individuals. To evaluate the association of the MIF -173 polymorphism with the outcome, we analyzed the data concerning the treatment regimen, duration of glucocorticoid treatment, score on the childhood health assessment questionnaire (C-HAQ) and the number of joints with active arthritis. Nonsignificant differences were observed between the study and control groups in the distribution of genotype and allele frequencies of the MIF gene -173 G/C polymorphism. In JRA patients, carrying a MIF -173 C allele, the number of disease modifying antirheumatic drugs required for the treatment was more, the duration of glucocorticoid treatment was significantly longer, and at the last visits the C-HAQ scores and the number of joints with active arthritis were significantly higher. MIF gene -173 C allele frequency did not differ between the controls and JRA patients. MIF -173 C allele did not confer increased susceptibility to JRA in our study group. Carriage of the MIF -173 C allele was found to be a strong predictor of poor outcome in all types of JRA

4. BLOOM BJ, TOYODA M, PETROSIAN Aet JORDAN S: Anti-endothelial cell antibodies are prevalent in juvenile idiopathic arthritis: implications for clinical disease course and pathogenesis, Rheumatol.Int., Vol. ., 2006
   Organism:Pediatric Rheumatology Program of the Division of Pediatric Ambulatory Medicine, Department of Pediatric, Hasbro Children's Hospital, Providence, RI, USA
   Abstract:To determine the prevalence of anti-endothelial cell antibodies (AECA) in children with juvenile idiopathic arthritis (JIA) versus healthy control children. Twenty-eight children with active JIA were studied (ten each with polyarticular and oligoarticular disease, and eight with systemic onset disease). AECA were determined by a cell-based ELISA from samples obtained every 3 months over a 2 year period in each subject. These levels were compared against previously determined levels of von Willebrand factor antigen, fibrin d-dimer, and soluble forms of ICAM-1 and E-selectin, as well as clinical measures of disease activity. AECA were detected in 5/10 oligoarticular, 6/10 polyarticular, and 7/8 systemic JIA subjects and 0/14 controls. Mean levels of AECA were significantly higher in subjects with oligoarticular, and especially systemic disease as compared to polyarticular and control groups when analyzed by ANOVA. AECA are prevalent in JIA and are present more often and at higher levels in systemic disease

5. BOYKINOV Iet KATSAROV D: 1. Longtermperiodic genmutation determined febrility syndromes (LPGFS). 2. Autoinflammatory diseases (AID). 3. Multisysem inflammatory diseases (MID). 4. Whole body inflammation (WBI). 5. Periodic febrility syndromes (PFS)|, yoderma-ustokosis, 2006
   Organism:As autoinflammatory diseases (AIDs) are defined recurrent inflammatory events without any recognizable pathogens. They do not produce high titer autoantibodies or antigenspecific T-cells. About fourteen hereditary autoinflammatory diseases are established as product of the innate immune system, in which a lot of genmutations and a great intracellular protein complex (GIPC) are involved. The responsible gens of all AIDs have been discovered and it was found that they encode certain protein domens of the GIPC. The letter have the property to interact between each other with the outcome in domens CARD15, NF-kB or AP, which are involved in the development of inflammation or apoptosis. The inflammation is presented by elevation of CRP, ESR, hypergammaglobulinemy and proinflammatory cytocins -IL-1, TNF-alpha, IL-18. AIDs: TRAPS (Tumor necrosing factor receptor associated periodic fever syndrome; TRAPS; HIDS (=Dutch) - Hyperimmunoglobulin D periodic fever syndrome; MWS - Mucle-Wells syndrome; FC

6. BREDELLA MA, STEINBACH LS, MORGAN S, WARD Met DAVIS JC: MRI of the sacroiliac joints in patients with moderate to severe ankylosing spondylitis, AJR Am.J.Roentgenol., Vol. 187(6), 1420-1426., 2006
   Organism:Department of Radiology, University of California, San Francisco, San Francisco, CA, USA mbredella@partnersorgFAU - Bredella, Miriam A
   Abstract:OBJECTIVE: The objectives of our study were to evaluate whether MRI findings of the sacroiliac joints are able to distinguish between active and inactive disease in patients with established ankylosing spondylitis and to determine whether these findings correlate with markers of clinical activity, disease duration, severity, and degree of radiographic damage. MATERIALS AND METHODS: Eighteen patients with symptomatic moderate to severe ankylosing spondylitis were evaluated. MRI of the sacroiliac joint (1.5 T) was performed using fat-saturated T2-weighted, T1-weighted, STIR, and fat-saturated contrast-enhanced T1-weighted sequences. The sacroiliac joints were evaluated by two radiologists for enhancement, subchondral bone marrow edema, erosions, and subchondral fatty marrow infiltration. Findings on MRI were analyzed for correlation with multiple clinical characteristics and measures of disease activity, including radiographic scoring. RESULTS: In 17 patients, MRI showed abnormal findings of the sacroiliac joint. Ten patients showed active disease on MRI as measured by abnormal enhancement and subchondral bone marrow edema. Disease activity detected using MRI correlated in a positive fashion with only C-reactive protein (CRP) level. There was no correlation with the other measures of disease activity or with disease duration. In 14 patients, fatty subchondral bone marrow was detected on MRI. These changes were seen in patients with active and chronic disease and correlated with higher radiographic scores but not with disease duration or markers of disease activity. CONCLUSION: Contrast-enhanced MRI of the sacroiliac joint is sensitive in depicting sacroiliitis in patients with established ankylosing spondylitis. Subchondral edema and enhancement correlate with high CRP levels. Subchondral fatty bone marrow changes were seen in both active and chronic sacroiliitis and are correlated with higher radiographic scores; these changes may be a marker of more advanced disease

7. BRIK R, LIVNAT G, POLLACK S, CATZ Ret NAGLER R: Salivary Gland Involvement and Oxidative Stress in Juvenile Idiopathic Arthritis: Novel Observation in Oligoarticular-type Patients, J.Rheumatol., Vol. 33(12), 2532-2537., 2006
   Organism:From the Department of Pediatrics and Pediatric Rheumatology Service, Department of Immunology, and Oral Biochemistry Laboratory and Salivary Clinic, Meyer Children's Hospital of Haifa, Rambam Medical Center, and the Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel
   Abstract:OBJECTIVE: The salivary glands may become affected in various collagen diseases, but their involvement in juvenile idiopathic arthritis (JIA) has received little attention. We studied the salivary composition and the antioxidant profile in patients with JIA, as well as their serum antioxidant status. METHODS: Twenty-two children with JIA according to the American College of Rheumatology criteria (10 oligoarticular, 7 polyarticular, and 5 systemic-type) and 15 healthy controls were studied. Serum and saliva samples were obtained simultaneously and analyzed. RESULTS: Significantly raised levels of antioxidant enzyme activity were observed in the patients with JIA, in both saliva and serum. The salivary peroxidase activity was significantly higher in the total group of patients with JIA by 8.5% (p < 0.01) as compared to controls (0.76 vs 0.70 mU/ml). Salivary superoxide dismutase was found to be significantly increased mainly in the patients with systemic JIA (by 74%; p < 0.02). Significantly higher levels of peroxidase activity were observed in serum of patients with JIA, particularly of the polyarticular group, by 17% (p < 0.05). Major changes in saliva composition were observed in patients with oligoarticular disease compared to controls: the patients had a lower salivary flow by 33%, less acidic saliva, and significantly lower salivary levels of magnesium by 44% (p < 0.01), total protein by 44% (p < 0.02), amylase by 34% (p < 0.02), and lactate dehydrogenase by 62% (p < 0.02). CONCLUSION: Children with JIA exhibited a major increase in antioxidant enzyme activity, both in serum and in saliva. Patients with oligoarticular JIA displayed indications of significant and specific damage to the salivary glands, a novel observation

8. BRUNNER JKet SITZMANN FC: Anticyclic citrullinated peptide antibodies in juvenile idiopathic arthritis, Mod.Rheumatol., Vol. 16(6), 372-375., 2006
   Organism:Department of Pediatrics, Innsbruck Medical School, Anichstrasse 35, A-6020, Innsbruck, Austria, juergenbrunner@uklibkacatFAU - Brunner, Jurgen K H
   Abstract:Anticyclic citrullinated peptide (anti-CCP) antibodies have been detected in patients with juvenile idiopathic arthritis (JRA), particularly in those with polyarticular JIA. We analyzed the presence of anti-CCP antibodies of the IgG class in sera of patients with defined juvenile idiopathic arthritis (JIA) of various subgroups. One hundred and fifty-nine serum samples were investigated. Forty-five patients were diagnosed with JIA (15 male and 30 female) aged 1.9-17.3 years (median 12.9, mean 11.0). Thirty-eight samples were taken from patients suffering from other autoimmunopathies and 34 patients with other underlying diseases were taken at different time points in their disease course. Under 42 samples were taken from patients with noninflammatory diseases. Enzyme-linked immunosorbent assay (ELISA) was used for the detection of anti-CCP antibodies. Anti-CCP antibodies were found in 6.9% of all samples and in 4.4% patients with JIA. Disease duration and medication did not differ significantly between anti-CCP positive and negative patients. A review of the literature and our own results shows that anti-CCP antibodies can be detected in the sera of only some patients with JIA. Routine determination of anti-CCP cannot be recommended

9. BURNHAM JM, SHULTS J, SEMBHI H, ZEMEL BSet LEONARD MB: The dysfunctional muscle-bone unit in juvenile idiopathic arthritis, J.Musculoskelet.Neuronal.Interact., Vol. 6(4), 351-352., 2006
   Organism:The Children's Hospital of Philadelphia, Philadelphia, PA, USAFAU - Burnham, J M
   Abstract:

10. CAPPA M, UBERTINI G, COLABIANCHI D, FIORI Ret CAMBIASO P: Non-conventional use of growth hormone therapy, Acta Paediatr.Suppl., Vol. 95(452), 9-13., 2006
    Organism:Department of Paediatric Medicine, Endocrinology Unit, Ospedale Pediatrico Bambino Gesu, Scientific Institute (IRCCS), Rome, Italy cappa@opbgnetFAU - Cappa, Marco
    Abstract:Human growth hormone therapy is allowed in certain clinical conditions according to national healthcare criteria. Growth hormone, however, produces a wide spectrum of effects. Linear growth is only one of the many expected results, and there are interesting possibilities to explore which could provide additional means of improving the quality of life for the ever-increasing numbers of chronic paediatric patients. CONCLUSION: In this review, we discuss the rationale for and possibility of using growth hormone therapy in some conditions not strictly related to growth hormone deficiency

11. CHA SI, FESSLER MB, COOL CD, SCHWARZ MIet BROWN KK: Lymphoid interstitial pneumonia: clinical features, associations and prognosis, Eur.Respir.J., Vol. 28(2), 364-369., 2006
    Organism:Dept of Internal Medicine, Kyungpook National University Hospital, Daegu, South KoreaFAU - Cha, S-I
    Abstract:Lymphoid interstitial pneumonia (LIP) is rare and its clinical course incompletely described. The aim of this study was to examine the clinical features, associations and prognosis of surgical lung biopsy-proven LIP. The study group consisted of 15 subjects encountered over a 14-yr period. The majority of subjects were females (n = 11) and the mean age was 47 yrs (range 17-78 yrs). Underlying systemic immune disorders were frequent, including Sjogren's syndrome (n = 8), rheumatoid arthritis, systemic lupus erythematosus, polymyositis, common variable immunodeficiency and dysproteinaemia. Only three patients were classified as "idiopathic". Presenting symptoms were dominated by dyspnoea and cough. Restrictive physiology, reduced diffusion capacity (62.5+/-18.4% predicted) and bronchoalveolar lavage lymphocytosis (30.5+/-29.1% pred) were noted. Thirteen patients received corticosteroid therapy. Of the nine whose response could be assessed, four showed clinical improvement and four were stable. Overall, median survival was 11.5 yrs. Of the seven patients who died, respiratory problems were the primary cause of death in three. Conversion to lymphoma was not identified. In conclusion, histopathological lymphoid interstitial pneumonia is commonly associated with immune system dysregulation, with idiopathic lymphoid interstitial pneumonia being extremely rare. Clinical stability or improvement with corticosteroids can be expected; however, survival remains impaired

12. DE JAGER W, HOPPENREIJS EP, WULFFRAAT NM, WEDDERBURN LR, KUIS Wet PRAKKEN BJ: Blood and synovial fluid cytokine signatures in patients with juvenile idiopathic arthritis; a cross-sectional study, Ann.Rheum.Dis., Vol. ., 2006
    Organism:University Medical Center Utrecht, Netherlands
    Abstract:Juvenile idiopathic arthritis (JIA) consists of a heterogeneous group of disorders with, for the most part, an unknown immunopathogenesis. Although onset and disease course differ, the subtypes of JIA share the occurrence of chronic inflammation of the joints, with infiltrations of immune competent cells that secrete inflammatory mediators. We undertook the present study to identify a panel of cytokines that is specifically related to the inflammatory process in JIA. Using a novel technology, the multiplex immunoassay we measured 30 cytokines in plasma from 65 JIA patients, of which 34 were paired with synovial fluid. We compared these data with plasma of 20 healthy controls and 9 patients with type I diabetes, a chronic inflammatory disease. JIA patients have, irrespective of their subclassification, significant higher levels of TNFa, MIF, CCL2, CCL3, CCL11, CCL22 and CXCL9 in plasma compared with healthy controls. In paired plasma and synovial fluid samples of JIA patients significant higher levels IL6, IL15, CCL2, CCL3, CXCL8, CXCL9 and CXCL10 are present in synovial fluid. Cluster analysis within all JIA patients reveals a pre dominant pro inflammatory cytokine cluster during active disease whereas a regulatory / anti-inflammatory related cytokine cluster is observed during remission. Furthermore we tested if a discrimination profile of various cytokines could be helpful in determination of disease classification. We suggest several cytokines (IL18, MIF, CCL2, CCL3, CCL11, CXCL9 and CXCL10) may correspond to the activation status during inflammation in JIA and could be instrumental to monitor disease activity, and therapy outcome of (new) immuno-therapies

13. DEMIREL M, TURHAN E, DEREBOY F, AKGUN Ret OZTURK A: Surgical treatment of skier's thumb injuries: case report and review of the literature, Mt.Sinai J.Med., Vol. 73(5), 818-821., 2006
    Organism:Orthopaedics and Traumatology Department, Ankara Bayindir Hospital, Ankara,Turkey perthes2000@yahoocomFAU - Demirel, Murat
    Abstract:Inappropriate treatment of skier's thumb injuries has been reported to result in chronic painful instability, weakness of pinch, and arthritis. Therefore, surgical treatment is recommended for those fractures with 2 mm or more of displacement, or significant articular involvement with incongruency or rotation. The goal of surgery is restoration of anatomy with stable fixation. In this study we present some cases managed with internal fixation of the injury. This technique has the advantage of anatomic stable fixation with good outcome

14. FOSTER H, DAVIDSON J, BAILDAM E, ABINUN M, WEDDERBURN LRet BRIT SOC PA: Autologous haematopoeitic stem cell rescue (AHSCR) for severe rheumatic disease in children - Guidance for BSPAR members- executive summary, Rheumatology (Oxford), Vol. 45, 1570-1571., 2006
    Organism:Sch Med, Med Sch,Framlington Pl, Newcastle Upon Tyne NE2 4HH, Tyne and Wear, UK UK
    Abstract:Autologous haematopoeitic stem cell rescue (AHSCR) is a treatment option for children with severe rheumatic disease and to date most experience is with juvenile idiopathic arthritis (JIA) [1-6]. Currently two transplant centres in the UK have NSCAG ( National Specialist Commissioning Advisory Group) funding to perform AHSCR for children with severe rheumatic disease; these are Newcastle (Newcastle General Hospital) and London (Great Ormond Street), both of which follow latest protocols set with the Joint European Bone Marrow Transplant (EBMT) and European Society for Immunodeficiency (ESID) Wo rking Party Sub-Committee (www.esid.org), and submit patients to the EBMT Registry. In the absence of an evidence base to support the referral and selection process, this guideline is a consensus statement, commissioned by the BSPAR Executive, and targeted at BSPAR members. This states criteria for considering AHSCR and provides an outline of the process ( selection, independent assessment and referral to the transplant centre) and defines the roles of the stakeholders (i.e. referral team, independent assessor and transplant team). The guideline refers specifically to children with JIA, but the principles are likely to be similar for other severe rheumatic diseases of childhood. This is a short summary of the whole guideline. The full guideline is available on the journal website (see supplementary material for full guideline)

15. FOSTER Het WYLLIE R: Chronic arthritis in children and adolescents|, 2006
    Organism:Juvenile idiopathic arthritis is the commonest cause of chronic arthritis in children and a major cause of disability. This article focuses on the clinical presentations of JIA, the emphasis on clinical skills in making a diagnosis and the multidisciplinary approach to management including transitional care into the adult world. (c) 2006 Elsevier Ltd. All rights reserved

16. GHOSH JB, GUPTA Det CHATTOPADHYAY N: Juvenile idiopathic arthritis with peripheral gangrene, Indian Journal of Pediatrics, Vol. 73, 739-741., 2006
    Organism:N Pratapgarh, Dt Bankura, W Bengal, India India
    Abstract:Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disorder of the childhood and is manifested by synovitis with or without systemic features. Secondary vasculitis occurring in response to JIA is reflected clinically on. different structures or systems of the body. Here is reported a rare case of systemic onset JIA (SOJIA) with vasculitis leading to peripheral gangrene

17. GOUMRI S, EL KABLI H, ALAOUI FZ, BETTAL Set BENAMOUR S: [Adult-onset Still disease. 11 cases], Tunis Med., Vol. 84(7), 443-449., 2006
    Organism:Service de Medecine Interne, CHU Ibn Rochd, CasablancaFAU - Goumri, Siham
    Abstract:Still's Disease of the adult is a systemic disease that the cause is unknown. If the pediatric forms are frequent, the affection of the adult is rare and its diagnosis is difficult. The purpose of this study is to analyse the features of the clinical evolution of adult Still's disease and to compare our results with the literature. It is a retrospective study about 21 years that permitted to collect 11 cases according the criteria of Yamaguchi. It is a matter of 6 men and 5 women that the mean age is 31 years old (age range: 16 to 48 years old). The fever was constant, the skin rash was noticed in 8 patients (72.7%); a polyarthritis was noticed in all the patients, chronic in 10 cases (90.9%) which 2 erosive forms. Adenopathies were present in 5 patients, a splenomegaly and a hepatomegaly were noticed respectively in 4 and 2 cases. Inflammatory syndrome with hyperleukocytosis was constant, and a hepatic cytolysis was noticed in 80%. The total ferritinemia titrated in 8 patients was constantly high. The hemocultures realized in all the patients were sterile and the complete immunologic examination was negative. The strong dose of corticoid was prescribed with success in all the patients. The immediate evolution was favourable in 10 patients. We deplore one death after a state of deep denutrition. Still's disease of the adult is rare, its diagnosis is difficult, sensitive to corticotherapy and its clinical evolution in our country is comparable to the literature

18. GUOMUNDSSON MT: 31st Scandinavian Congress of Rheumatology, Reykjavic, ICELAND, August 16 -19, 2006, 9-56., 16-08-2001

19. HAMZA S, MRABET D, CHEOUR E, MEDDEB Net SELLAMI S: Juvenile idiopathic arthritis and lyme disease: A case report|, 2006
    Organism:Lyme disease is a systemic infection due to Borrelia burgdorferi. Joint involvement in children, when primary phase is unknown, can be confounded with juvenile idiopathic arthritis. We report a case of a 16 years old girl, who developed at the age of 14 a bilateral and symetrical polyarthritis of big and small joints with fever and cutaneous eruption of trunk. No clinical improvement was seen under disease modified treatment. More biological investigations were performed, leading to the diagnosis of Lyme disease. Clinical recovery was obtained under adapted antibiotherapy, Hence Lyme serology must be performed when atypical polyarthritis appears in a child especially in an endemic region of borrelia burdogferi

20. HANOVA P, PAVELKA K, DOSTAL C, HOLCATOVA Iet PIKHART H: Epidemiology of rheumatoid arthritis, juvenile idiopathic arthritis and gout in two regions of the Czech Republic in a descriptive population-based survey in 2002-2003, Clin.Exp.Rheumatol., Vol. 24(5), 499-507., 2006
    Organism:Institute of Rheumatology, 1st Medical Faculty, Charles University, Prague, Czech Republic p-hanova@seznamczFAU - Hanova, P
    Abstract:OBJECTIVE: To estimate the annual incidence and prevalence of rheumatoid arthritis (RA), juvenile arthritis (JIA) and gout in a population based study in two regions of the Czech Republic with total population of 186,000 inhabitants. METHODS: The study was conducted in the Town of Ceske Budejovice and district of Cheb in the Czech Republic (with a total population of 186,000 inhabitants) in the years 2002 and 2003. Incident cases were registered on condition that the definite diagnosis was confirmed according to existing classification criteria during the study period. Prevalence was studied on the basis of identification of established diagnosis from registers of patients of participating rheumatologists and other specialists. They were asked to report all living patients who had been diagnosed before 1st March 2002. Patients were only included in the study if their permanent address was in the selected study area. RESULTS: Overall, we found 48 incident and 947 prevalent cases of RA among adults (16+ years), 4 incident and 43 prevalent cases of JIA among children (less than 16 years old), and 64 incident and 425 prevalent cases of gout among adults (16+ years). The total annual incidence of RA was 31/100,000 in the adult population aged 16 years and more (95% CI 20 to 42/100,000). The prevalence of RA was 610/100,000 (95% CI 561 to 658/100,000) in the adult population. An annual incidence of gout in adults was 41/100,000 (95% CI 28 to 53/100,000). The prevalence of gout was 300/100,000 (95% CI 266 to 334/100,000). The annual incidence of JIA was 13/100,000 in children less than 16 years old (95%CI 1 to 20/100,000). The prevalence of JIA in children was 140/100,000 (95% CI 117 to 280/100,000). CONCLUSION: This study estimates the annual incidence and prevalence rates of RA, gout and JIA in the first population-based survey in the Czech Republic. The rates of RA and JIA compare well with figures reported from other countries; figures in gout seem to be lower than reported elsewhere

21. HASAN U: Tumour necrosis factor inhibitors--what we need to know, N.Z.Med.J., Vol. 119(1246), U2336, 2006
    Organism:Capital and Coast District Health Board umbzaf@hotmailcomFAU - Hasan, Umbreen
    Abstract:Biologics have revolutionised the treatment of various autoimmune diseases. Earlier in 2006, New Zealand's Pharmaceutical Management Agency (PHARMAC) approved adalimumab (anti-tumour necrosis factor [TNF] agent) for treatment of severe, unresponsive rheumatoid arthritis in adult patients. Etanercept has been available for chronic juvenile arthritis in selected, younger patients in New Zealand. This review article describes some of the common adverse events related to anti-TNF inhibitors and the importance of establishing guidelines for surveillance in the New Zealand health system

22. HAYASHI A, HANADA T, KOHDA T, OKADA S, KANZAKI S, KASAGI Tet UTSUNOMIYA Y: Mizoribine oral pulse therapy for a patient with polyarticular juvenile idiopathic arthritis, Pediatr.Int., Vol. 48(6), 638-640., 2006
    Organism:Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University, Yonago, Japan atsushih@grapemedtottori-uacjpFAU - Hayashi, Atsushi
    Abstract:

23. HEILIGENHAUS A: When should Intraocular lenses be implanted in patients with juvenile idiopathic arthritis-associated iridocyclitis?, Ophthalmic Research, Vol. 38, 316-317., 2006
    Organism:

24. INUI K, MAENO T, TADA M, TAKAOKA Ket KOIKE T: Open reduction of the dislocated hip in juvenile idiopathic arthritis: a case report, Mod.Rheumatol., Vol. 14(5), 399-401., 2004
    Organism:Department of Orthopaedic Surgery, Osaka City University Medical School, 1-4-3 Asahi-machi, Abeno-ku, Osaka, 545-8585, Japan, tatsuya@medosaka-cuacjpFAU - Inui, Kentaro
    Abstract:An 8-year-old girl with systemic-onset juvenile idiopathic arthritis (JIA) required surgical reduction for a dislocated left hip joint following failure of skin traction for 1 week. Unaided walking was achieved by 3 months postoperatively. Incongruence and irregularity of the hip joint remained but may resolve with maturation. Joint laxity caused by synovitis, flexion/adduction contracture with pain, and acetabular dysplasia by growth disturbance apparently caused hip dislocation

25. KABASAKAL Y, KITAPCIOGLU G, TURK T, ODER G, DURUSOY R, METE N, EGRILMEZ Set AKALIN T: The prevalence of Sjogren's syndrome in adult women, Scand.J.Rheumatol., Vol. 35(5), 379-383., 2006
    Organism:Department of Rheumatology, Ege University, Turkey yaseminkabasakal@yahoocomFAU - Kabasakal, Y
    Abstract:OBJECTIVES: The aim of this study was to determine the prevalence of primary Sjogren's syndrome (pSS) according to European criteria (1993) and to the US-European Consensus Group (US-EU) criteria (2002) in adult women in Bornova, Izmir, Turkey. MATERIALS AND METHOD: The study was designed as a two-phase cross-sectional survey consisting of a baseline questionnaire and collection of blood samples and clinical examination. In the initial phase, positivity for autoantibodies Ro(SS-A), La(SS-B), rheumatoid factor (RF), and anti-nuclear antibodies (ANA) was determined, and in the clinical phase, clinical examination, salivary and ocular tests were performed. Minor salivary gland biopsy was performed for those who had at least three of these five criteria positive. RESULTS: In our sample the prevalence of SS was 1.56% [95% confidence interval (CI) 0.92-2.66] according to the European criteria and 0.72% (95% CI 0.33-1.57) according to the US-EU criteria. CONCLUSION: To prevent the loss in diagnosis of pSS, the addition of ANA, RF, and tear break-up time (BUT) tests to US-EU criteria would be appropriate

26. KANATLI U, OZTURK AM, ERCAN NG, OZALAY M, DAGLAR Bet YETKIN H: Absence of the medial sesamoid bone associated with metatarsophalangeal pain, Clin.Anat., Vol. 19(7), 634-639., 2006
    Organism:Department of Orthopedics and Traumatology, Gazi University, Ankara, Turkey ukanatli@gaziedutrFAU - Kanatli, Ulunay
    Abstract:Pain at the first metatarsophalangeal (MTP) joint can result from inflammation, chondromalacia, flexor hallucis brevis tendinitis, osteochondritis dessecans, fracture of a sesamoid bone, avascular necrosis of sesamoids, inflamed bursae, intractable keratoses, infection, sesamoiditis, gout arthropathy, and rheumatoid arthritis. Congenital absence of a sesamoid bone is extremely rare. We present a 17-year-old male patient with pain at the plantar aspect of the right MTP joint associated with congenital absence of the medial sesamoid. There was tenderness and the range of motion was minimally restricted. He described the pain as necessitating changes in his social life. On radiographs, the medial hallucial sesamoid was absent on the right side. The MTP joint was also evaluated using magnetic resonance imaging (MRI). A metatarsal pad was prescribed and the patient was satisfied with the treatment at the 2 months follow-up period. MRI revealed no pathological tissue at the medial sesamoid site. Hallucial sesamoids absorb pressure, reduce friction, protect the tendons, act like a fulcrum to increase the mechanical force of the tendons, and provide a dynamic function to the great toe by elevating first metatarsal head. Congenital absence of these bones is very rare but we must consider it in a patient with MTP joint pain

27. KLIPSTEIN A, SCHENK P, HODLER J et LAEUBLI T: Joint Annual Meeting of the Swiss-Society-of-Rheumatology/Swiss-Society-of- Physical-Medicine-and-Rehabilitation, Geneva, SWITZERLAND, September 28 -29, 2006, 3S-19S., 28-09-2001

28. KONTTINEN L, HONKANEN V, UOTILA T, POLLANEN J, WAAHTERA M, ROMU M, PUOLAKKA K, VASALA M, KARJALAINEN A, LUUKKAINEN Ret NORDSTROM DC: Biological treatment in rheumatic diseases: results from a longitudinal surveillance: adverse events, Rheumatol.Int., Vol. 26(10), 916-922., 2006
    Organism:Department of Medicine, University of Helsinki, Helsinki, FinlandFAU - Konttinen, L
    Abstract:The objective of this study was to assess the long-term safety and tolerability of biologicals in a clinical setting. Data on adverse events (AEs) have been collected over a 5-year period by means of detailed reports sent in to the National Register of Biological Treatment in Finland (ROB-FIN) and validated by information collected by the National Agency for Medicines. Three hundred and eight reports on AEs were filed, concerning a total of 248 patients; this corresponds to 17% of all patients in the ROB-FIN register who started biological treatments. Skin reactions and infections comprised 35 and 28% of the AEs, respectively. Some cases of tuberculosis and other infections, heart failure and demyelinating conditions were seen. Our work demonstrates no unexpected AEs in a Finnish patient cohort consisting of rheumatoid arthritis and spondylarthropathy patients, although many of them were treated with combination treatments in common use in Finland. Biological treatment appears safe in the hands of the Finnish rheumatologists

29. KOTANIEMI Ket PENTTILA H: Intraocular lens implantation in patients with juvenile idiopathic arthritis-associated uveitis, Ophthalmic Research, Vol. 38, 318-323., 2006
    Organism:Rheumatism Fdn Hosp, Pikijarventie 1, FI-18120 Heinola, Finland Finland
    Abstract:Objective: To evaluate the development of cataract and the results of cataract surgery with intraocular lens (IOL) implantation in patients with chronic uveitis associated with juvenile idiopathic arthritis (JIA). Patients and Methods: A hospital-based retrospective case series consisted of 25 patients with JIA-associated uveitis. The mean age of the patients was 5.8 years at the onset of arthritis and 6.8 years at the onset of uveitis. During the 15-year study period cataract surgery with implantation of an IOL was performed in 36 eyes. In 17 eyes phacoemulsification and initial posterior capsulectomy with anterior core vitrectomy were performed. The treatment of JIA and uveitis was carefully adjusted with systemic immunosuppressive drugs and topical corticosteroids perioperatively. The mean postoperative follow-up period was 3.3 years. Results: The first signs of cataract were observed 2.3 years (mean) after the diagnosis of uveitis and the cataract operation of the first eye was performed 4.5 years (mean) after the diagnosis of uveitis. After IOL surgery the visual result was good (>= 0.5) in 64%, moderate (0.3 to < 0.5) in 11% and impaired (< 0.3) in 25% of eyes. Secondary cataract developed in 16 eyes but in none of the eyes with initial posterior capsulectomy and core vitrectomy. Secondary glaucoma developed in 18 eyes, retinal detachment in 2, cystoid macular edema in 16 and band keratopathy in 12 eyes. Conclusion: Cataract is an early complication of JIA-associated uveitis. Under strict control of uveitis, IOL implantation is an important alternative in visual rehabilitation for this type of patient. Copyright (c) 2006 S. Karger AG, Basel

30. KULKARNI ML, BASKAR Ket KULKARNI PM: A syndrome of immunodeficiency, autoimmunity, and spondylometaphyseal dysplasia, Am.J.Med.Genet.A., Vol. 143(1), 69-75., 2007
    Organism:Department of Pediatrics, JJMMC, Davangere, Karnataka, IndiaFAU - Kulkarni, M L
    Abstract:We treated a 5-year-old boy, in our hospital in south India, who had a history of recurrent respiratory infections, tuberculosis, and severe varicella infection. He was short in build and a radiological examination revealed evidence of spondylometaphyseal dysplasia. Investigation of the immune system was suggestive of compromised cellular immunity. Immunofluorescence and immunoblot assay for antibodies detected underlying multiple disorders such as systemic lupus erythematosus (SLE), autoimmune thrombocytopenia, and juvenile rheumatoid arthritis (JRA). Roifman et al. described a similar syndrome in 2000 and 2003, which was characterized by spondylometaphyseal dyplasia, combined immunodeficiency, and autoimmunity and called it Roifman-Costa syndrome (OMIM 607944). Hence a diagnosis of Roifman-Costa syndrome was made. Ours shall be the first report of such a condition from the Indian subcontinent and hence the communication. (c) 2006 Wiley-Liss, Inc

31. LEE YH, RHO YH, CHOI SJ, JI JD, SONG GG, NATH SKet HARLEY JB: The PTPN22 C1858T functional polymorphism and autoimmune diseases - A meta-analysis|, 2007
    Organism:Objective. To assess whether combined evidence shows the association between the protein tyrosine phosphatase non-receptor 22 (PTPN22) C1858T polymorphism and autoimmune diseases, and to summarize the effect size of the polymorphism associated with susceptibility of autoimmune diseases. Methods. We surveyed studies on the PTPN22 C1858T polymorphism and autoimmune diseases using comprehensive Medline search and review of the references. Meta-analysis was performed for genotypes T/T (recessive effect), T/T + C/T (dominant effect) and T-allele in random effects models. Results. Twenty-nine studies with 43 comparisons including 13 rheumatoid arthritis (RA), six systemic lupus erythematosus (SLE), six type-1DM (T1D), three Grave's disease (GD), four inflammatory bowel diseases (IBD), three juvenile idiopathic arthritis (JIA), two psoriasis, two multiple sclerosis, two Addison's disease and two Celiac disease were available for the meta-analysis. The overall odds ratios (ORS) for T-allele, T/T and T/T + C/T genotypes were significantly increased in RA, SLE, GD and T1D (OR for T-allele = 1.58, 1.49, 1.85, 1.61, respectively, P < 0.00001). This meta-analysis showed the association between the T-allele and the T/T genotype and JIA (OR = 1.34, P = 0.03; OR = 1.97, P = 0.02) but did not reveal the association between the PTPN22 C1858T poly morphism and IBD, psoriasis, multiple sclerosis, Addison's disease and Celiac disease. Conclusion. This meta-analysis demonstrates that the PTPN22 1858T allele confers susceptibility to RA, SLE, GD, T1D and JIA, supporting evidence of association of the PTPN22 gene with subgroup of autoimmune diseases. (c) 2007 Oxford University Press

32. LENG XM, ZHAO Y, ZHOU DB, SITU H, LI TS, SHEN T, ZHAO YQ, ZENG XF, ZHANG FC, DONG Yet TANG FL: A pilot trial for severe, refractory systemic autoimmune disease with stem cell transplantation, Chin Med.Sci.J., Vol. 20(3), 159-165., 2005
    Organism:Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730FAU - Leng, Xiao-Mei
    Abstract:OBJECTIVE: To evaluate the feasibility, efficacy, and safety of high dose immunosuppressive therapy (HDIT) and autologous hemopoietic stem cell transplantation (HSCT) with CD34+ cell selection in patients with severe, refractory autoimmune diseases. METHODS: Twenty-six patients with persistent systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), primary Sjogren's syndrome (pSS), or systemic sclerosis (SSc) who had been treated unsuccessfully with conventional treatment were enrolled in the trial in Peking Union Medical College Hospital from September 1999 to June 2004. The patients received HDIT with 200 mg/kg cyclophosphamide followed by an infusion of autologous stem cells that were CD34 selected. Disease activity, adverse effect, hemopoietic and immune reconstitution, and time to recurrence of disease were monitored. RESULTS: Overall treatment related mortality was 7.7% (2/26) with 1 patient died of cytomegalovirus infection and another of severe pneumonia. Relapse occurred in 3 SLE patients (17.6%) in 37, 26, and 19 months posttransplantation respectively, and 1 RA patient in 15 months posttransplantation. SLE Disease Activity Index (SLEDAI) scores of SLE survivors decreased significantly (P < 0.01). RA patients recorded a drop of Disease Activity Score 28 (DAS 28). The pSS patient remained symptoms free up to now, more than 50 months after the transplantation. CONCLUSION: HSCT can be performed relative safely in patients with severe autoimmune disease. Short-term effect of HSCT is promising. However treatment related mortality and relapse were observed in a subset of patients

33. LI J, SHEN W, KONG Ket LIU Z: Interleukin-21 induces T-cell activation and proinflammatory cytokine secretion in rheumatoid arthritis, Scand.J.Immunol., Vol. 64(5), 515-522., 2006
    Organism:Laboratory of Immunopathology, The Institute of Digestive Diseases, Department of Medicine, The Second Affiliated Hospital, Zhengzhou University, Zhengzhou, ChinaFAU - Li, J
    Abstract:Interleukin (IL)-21 is a CD4+ T-cell-derived cytokine, which is involved in innate and adaptive immune response. In this study, we analysed IL-21 receptor (IL-21R) expression in peripheral blood and synovial fluid mononuclear cells, and investigated the role of IL-21 in the induction of proinflammatory cytokine production by peripheral blood T cells (PB-T) and synovial fluid T cells (SF-T) from patients with rheumatoid arthritis (RA). Immunohistochemical staining demonstrated that IL-21R-positive cells were significantly increased in inflamed synovial tissues of RA patients compared with osteoarthritis (OA) and healthy controls. Flow cytometric analysis confirmed that IL-21R was mainly expressed in freshly isolated CD4, CD8, B and NK cells from peripheral blood and synovial fluid, but decreased gradually in T cells 24 h after anti-CD3 stimulation. PB- and SF-T cells from RA patients were more responsive to IL-21 when compared with controls. Importantly, isolated PB- or SF-T cells from RA patients, when stimulated with IL-21 and anti-CD3 MoAb, secreted markedly higher levels of TNF-alpha and IFN-gamma than controls. These data indicate that IL-21R is overexpressed in the inflamed synovial membrane and in peripheral blood or synovial fluid leukocytes of RA patients, and that IL-21 enhances local T-cell activation, proliferation and proinflammatory cytokine secretion. Thus, blockade of IL-21R signalling pathway may have a therapeutic potential in acute RA patients

34. LIN CJ, LIU JT, CHANG CHet NOWALK MP: Association of obesity and chronic diseases in Taiwan, Asia Pac.J.Public Health., Vol. 18(3), 8-14., 2006
    Organism:Department of Radiation Oncology, University of Pittsburgh, PA 15232, USA cjlin@pitteduFAU - Lin, C J
    Abstract:Two systems were used to classify weight status based on body mass index (BMI) of 3,178 Taiwanese adults who participated in the 1993-1996 Nutrition and Health Survey and to explore associations of BMI categories and disease. In the system proposed by the International Association for the Study of Obesity and the International Obesity Taskforce for Asian adults, overweight was associated with one disease (hypertension) and obesity was associated with four diseases: diabetes (OR = 2.66; 95% CI = 1.39-5.09; p < 0.01); gout (OR = 4.33; 95% CI = 1.92-9.75; p < 0.01); hypertension (OR = 4.92; 95% CI = 2.87-8.42; p < 0.01); thyroid disease (OR = 2.29; 95% CI = 1.12-4.67; p < 0.05). In the system devised by Taiwan Health Department for Taiwanese adults, overweight was associated with four diseases (arthritis, diabetes, gout, hypertension), and obesity was associated with three diseases: diabetes (OR = 2.11; 95% CI = 1.07-4.19; p < 0.05); gout (OR = 4.06; 95% CI = 1.77-9.28; p < 0.01); hypertension (OR = 5.28; 95% CI = 3.23-8.63; p < 0.01). The Obesity Taskforce may underestimate the association of excess weight and disease in Taiwan

35. LIPHAUS BL, KISS MH, CARRASCO Set GOLDENSTEIN-SCHAINBERG C: Increased Fas and Bcl-2 expression on peripheral blood T and B lymphocytes from juvenile-onset systemic lupus erythematosus, but not from juvenile rheumatoid arthritis and juvenile dermatomyositisdagger, Clin.Dev.Immunol., Vol. 13(2), 283-287., 2006
    Organism:Rheumatology Unit, Department of Pediatrics, School of Medicine, Children's Institute, University of Sao Paulo, BrazilFAU - Liphaus, Bernadete L
    Abstract:Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE), juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDM). Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC) were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal-Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation

36. MCDONAGH JE, SOUTHWOOD TRet SHAW KL: The impact of a coordinated transitional care programme on adolescents with juvenile idiopathic arthritis|, 2007
    Organism:Objective. There is an extensive evidence base for the need of transitional care, but a paucity of robust outcome data. The aim of the study was to determine whether the quality of life of adolescents with juvenile idiopathic arthritis (JIA) could be improved by a co-ordinated, evidence-based programme of transitional care. Methods. Adolescents with JIA aged 11, 14 and 17 yrs and their parents were recruited from 10 rheumatology centres in the UK. Data were collected at baseline, 6 and 12 months including core outcome variables. The primary outcome measure was health-related quality of life (HRQL): Juvenile Arthritis Quality of Life Questionnaire (JAQQ). Secondary ou tcome measures included: knowledge, satisfaction, independent health behaviours and pre-vocational experience. Results. Of the 359 families invited to participate, 308 (86%) adolescents and 303 (84%) parents accepted. A fifth of them had persistent oligoarthritis. Median disease duration was 5.7 (0-16) yrs. Compared with baseline values, significant improvements in JAQQ scores were reported for adolescent and parent ratings at 6 and 12 months and for most secondary outcome measures with no significant deteriorations between 6 and 12 months. Continuous improvement was observed for both adolescent and parent knowledge with significantly greater improvement in the younger age groups at 12 months (P = 0.002). Conclusions. This study represents the first objective evaluation of an evidence-based transitional care programme and demonstrates that such care can potentially improve adolescents' HRQL. (c) 2007 Oxford University Press

37. MOZOLOVA D, BIRC cet GROMOVA M: Juvenile idiopathic arthritis from the very cruel side|, 2006
    Organism:The case histories of 6 girls with juvenile idiopathic arthritis (JIA) describe the disease processes, which also represent very severe or even cruel condition for extraordinary serious defect of body height, damage to the eyes and marked deformity or even destruction of joints. In one case a total endoprosthesis of three major joints had to be applied. Even most recent mode of therapy did not stop the progress of the pathological process

38. NAKAHARA Het NISHIMOTO N: Anti-interleukin-6 receptor antibody therapy in rheumatic diseases, Endocrine Metabolic & Immune Disorders-Drug Targets, Vol. 6, 373-381., 2006
    Organism:Osaka Univ, Grad Sch Frontier Biosci, Lab Immune Regulat, 1-3 Yamadaoka, Suita, Osaka 5650871, Japan Japan
    Abstract:In the treatment of rheumatic diseases such as rheumatoid arthritis (RA) or systemic onset juvenile idiopathic arthritis (soJIA), new therapies targeting pro-inflammatory cytokines have been developed. IL-6 is a pleiotropic cytokine with a wide range of biological activities including a pro-inflammatory mediator activity. Overproduction of IL-6 has been reported to be pathologically involved in the rheumatic diseases and, therefore, blockade of IL-6 actions may improve the disease. Tocilizumab, a humanized monoclonal antibody against human interleukin-6 receptor (IL-6R), inhibits IL-6 binding to IL-6R and specifically interferes with IL-6 actions. Castleman's disease is an atypical lymphoproliferative disorder caused by the overproduction of IL-6. Tocilizumab therapy improves immunological and hematological abnormalities as well as systemic inflammatory symptoms including wasting. This translational study also confirmed the pathological significance of IL-6 in the disease. RA is a representative autoimmune inflammatory disease characterized by bone and cartilage destruction in multiple joints. Since IL-6 also plays pathological roles in RA, tocilizumab therapy has been introduced to the patients with refractory disease and has shown a strong therapeutic effect. Besides Castleman's disease and RA, tocilizumab has been shown to be effective for patients with soJIA and Crohn's disease. Tocilizumab treatment is generally well tolerated and safe. Therefore, tocilizumab can be a promising therapeutic agent for the rheumatic diseases in which IL-6 overproduction is pathologically involved

39. NIEHUES Tet LANKISCH P: Recommendations for the use of methotrexate in juvenile idiopathic arthritis, Paediatr.Drugs., Vol. 8(6), 347-356., 2006
    Organism:Department of Pediatric Oncology, Hematology and Immunology, Pediatric Immunology and Rheumatology, Centre for Child Health, Heinrich-Heine-University, Dusseldorf, GermanyFAU - Niehues, Tim
    Abstract:Juvenile idiopathic arthritis (JIA) is one of the most common rheumatic diseases in childhood. In a significant number of JIA cases the disease is resistant to therapy with NSAIDs, intra-articular corticosteroid injections, and physiotherapy, and methotrexate is used as a second-line agent. The efficacy of methotrexate therapy in children with JIA has been demonstrated in prospective controlled trials and this agent appears to have slightly superior efficacy compared with leflunomide. Data from randomized studies indicate a starting dose of 10-15 mg/m(2)/week orally. The dose of parenteral methotrexate can be increased to 15-20 mg/m(2)/week. Combination therapy with methotrexate and an NSAID is recommended. However, there are still no data on when to initiate methotrexate in JIA and how long children should be treated.The most common adverse effects are aversion to the drug and nausea. In the case of minor adverse effects the use of folic acid at a dosage of 1 mg/day is feasible. In JIA, daily folate supplementation has only been studied in one small heterogeneous cohort with a very short observation period and, at present, a general recommendation on daily folate supplementation cannot be made.In summary, methotrexate is seen by many pediatric rheumatologists as the first-choice, second-line drug; there is good evidence of its efficacy in JIA. However, in light of the recent introduction of biologic agents, the place of methotrexate in the treatment of JIA may have to be redefined in the coming years

40. NISTALA K, BABAR J, JOHNSON K, CAMPBELL-STOKES P, FOSTER K, RYDER Cet MCDONAGH JE: Clinical assessment and core outcome variables are poor predictors of hip arthritis diagnosed by MRI in juvenile idiopathic arthritis, Rheumatology (Oxford)., Vol. ., 2006
    Organism:Birmingham Children's Hospital and Institute of Child Health, Diana, Princess of Wales Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK
    Abstract:OBJECTIVES: To compare the diagnostic performance of clinical assessment against magnetic resonance imaging (MRI) diagnosed hip arthritis in a juvenile idiopathic arthritis (JIA) population. To determine the clinical and serological predictors of MRI diagnosed hip arthritis. METHODS: A total of 34 JIA patients with established disease (mean disease duration 6.3 yrs) had their hip MRIs scored for features of active hip arthritis and hip damage. Results were compared with clinical variables (disease subtype, history of hip pain, core outcome variables (COV)) and the clinician's assessment of active hip arthritis. RESULTS: MRI features of active hip arthritis were found in 45 hips (70%) and hip damage in 36 hips (56%). Clinical assessment had fair agreement with MRI scoring of active arthritis in patients with disease duration <4 yrs (kappa score 0.38, P = 0.045). Clinical assessment had a sensitivity of 25.7% and specificity of 91% for detecting MRI diagnosed arthritis. Of the core outcome variables only erythrocyte sedimentation rate predicted inflammation detected on MRI (r = 0.44, P = 0.014). CONCLUSIONS: The association between the clinician's assessment, core outcome variables and MRI findings in this study was limited. This indicates that clinical and laboratory findings are inadequate diagnostic tools for the assessment of hip arthritis when compared with MRI as the gold standard

41. NOONAN CW, PFAU JC, LARSON TCet SPENCE MR: Nested case-control study of autoimmune disease in an asbestos-exposed population, Environ.Health Perspect., Vol. 114(8), 1243-1247., 2006
    Organism:Center for Environmental Health Sciences, University of Montana, Missoula, Montana 59812, USA curtisnoonan@umontanaeduFAU - Noonan, Curtis W
    Abstract:OBJECTIVE: To explore the potential association between asbestos exposure and risk of autoimmune disease, we conducted a case-control study among a cohort of 7,307 current and former residents of Libby, Montana, a community with historical occupational and environmental exposure to asbestos-contaminated vermiculite. METHODS: Cases were defined as those who reported having one of three systemic autoimmune diseases (SAIDs): systemic lupus erythematosus, scleroderma, or rheumatoid arthritis (RA). Controls were randomly selected at a 3:1 ratio from among the remaining 6,813 screening participants using frequency-matched age and sex groupings. RESULTS: The odds ratios (ORs) and 95% confidence intervals (CIs) for SAIDs among those >or=65 years of age who had worked for the vermiculite mining company were 2.14 (95% CI, 0.90-5.10) for all SAIDs and 3.23 (95% CI, 1.31-7.96) for RA. In this age group, exposure to asbestos while in the military was also an independent risk factor, resulting in a tripling in risk. Other measures of occupational exposure to vermiculite indicated 54% and 65% increased risk for SAIDs and RA, respectively. Those who had reported frequent contact with vermiculite through various exposure pathways also demonstrated elevated risk for SAIDs and RA. We found increasing risk estimates for SAIDs with increasing numbers of reported vermiculite exposure pathways (p<0.001). CONCLUSION: These preliminary findings support the hypothesis that asbestos exposure is associated with autoimmune disease. Refined measurements of asbestos exposure and SAID status among this cohort will help to further clarify the relationship between these variables

42. NORDSTROM DC, KONTTINEN L, KORPELA M, TIIPPANA-KINNUNEN T, EKLUND K, FORSBERG S, ILVA K, KAIPIAINEN-SEPPANEN O, MALMI T, YLA-KERTTULA Tet HONKANEN V: Classic disease modifying anti-rheumatic drugs (DMARDs) in combination with infliximab. The Finnish experience, Rheumatol.Int., Vol. 26(8), 741-748., 2006
    Organism:Department of Internal Medicine, Helsinki University Central Hospital, Haartmanink 4, 00290, Helsinki, Finland dannordstrom@husfiFAU - Nordstrom, D C
    Abstract:To assess the performance of infliximab in a clinical setting, 364 rheumatoid arthritis (RA) patients from the National Register of Biological Treatment in Finland (ROB-FIN) were analysed. Corticosteroid usage and dose diminished (p<0.05 and 0.001, respectively) in patients on infliximab, of whom 51% also used one, 28% two and 16% three other concomitant DMARDs. A 34% of the RA patients used methotrexate+/-corticosteroids without any other DMARD. Methotrexate was most frequently used with sulphasalazine and/or hydroxychloroquine. Non-methotrexate patients most frequently used leflunomide or azathioprine combined with corticosteroids. The clinical effect of these combinations was similar to that of infliximab with methotrexate alone. The results indicate that infliximab can be used together with other DMARDs than methotrexate alone, quite according to the philosophy of the combination drug therapy, as the effectiveness is as good as or even slightly better than that of methotrexate and infliximab

43. NORMAN SB, MEANS-CHRISTENSEN AJ, CRASKE MG, SHERBOURNE CD, ROY-BYRNE PPet STEIN MB: Associations between psychological trauma and physical illness in primary care, J.Trauma Stress., Vol. 19(4), 461-470., 2006
    Organism:Department of Psychiatry, University of California, San Diego, CA, USAFAU - Norman, Sonya B
    Abstract:Psychological trauma is associated with poor physical health. We examined whether specific trauma types (assaultive, sexual, any) are associated with specific medical illnesses and whether posttraumatic stress disorder (PTSD) mediated these relationships in 680 primary care patients. For men, trauma history was associated with arthritis and diabetes; PTSD mediated the association between trauma and arthritis but not diabetes. Among women, trauma was associated with digestive diseases and cancer; PTSD did not mediate these relationships. Awareness of the presence of the physical illnesses examined here may help with the identification and treatment of primary care patients with trauma histories

44. NOZAKI Y, TAMAKI C, YAMAGATA T, SUGIYAMA M, IKOMA S, KINOSHITA Ket FUNAUCHI M: All-trans-retinoic acid suppresses interferon-gamma and tumor necrosis factor-alpha; a possible therapeutic agent for rheumatoid arthritis, Rheumatol.Int., Vol. 26(9), 810-817., 2006
    Organism:Department of Nephrology and Rheumatology, Kinki University School of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, 589-8511 Osaka, JapanFAU - Nozaki, Yuji
    Abstract:OBJECTIVES: To study the effects of all-trans-retinoic acid (ATRA), we determined the proliferation and cytokine production by peripheral blood mononuclear cells (PBMCs) and CD4+ T cells in healthy volunteers and patients with rheumatoid arthritis (RA), and explored the possibility of using ATRA as a therapeutic agent for autoimmune diseases. METHODS: Proliferation of these cells was determined by modified MTT assay, and expression of CC chemokine receptors 4 (CCR4) and CCR5 was determined by flow cytometry. Production and expression of interferon (IFN)-gamma, interleukin (IL)-2, IL-4, and tumor necrosis factor (TNF)-alpha was determined by Enzyme-Linked Immunosorbent Assay (ELISA) and reverse transcription-polymerase chain reaction (RT-PCR). The presence of STAT6 protein was determined by Western blot analysis. RESULTS: ATRA did not affect the proliferation or production of IL-2 and IL-4. We did not detect STAT6 protein, and saw no evidence of the differentiation of PBMCs to Th1 or Th2 cells. In contrast, ATRA suppressed the production of IFN-gamma and TNF-alpha significantly. There were no significant differences between the healthy volunteers and RA patients. CONCLUSIONS: ATRA was demonstrated to affect the cytokine production of IFN-gamma and TNF-alpha. ATRA might be useful in the treatment of autoimmune diseases such as RA

45. OELZNER P, LEHMANN G, EIDNER T, FRANKE S, MULLER A, WOLF Get HEIN G: Hypercalcemia in rheumatoid arthritis: relationship with disease activity and bone metabolism, Rheumatol.Int., Vol. 26(10), 908-915., 2006
    Organism:Department of Internal Medicine III, Division of Rheumatology/Osteology, Friedrich-Schiller University, Erlanger Allee 101, 07740 Jena, Germany peteroelzner@meduni-jenadeFAU - Oelzner, Peter
    Abstract:To investigate the relationship between ionized calcium and disease activity, parameters of bone metabolism and bone mineral density (BMD) at the lumbar spine (BMD-LS) and the femoral neck (BMD-FN) measured by dual X-ray absorptiometry in rheumatoid arthritis (RA). In 146 patients with RA, the following parameters were investigated: serum levels of ionized calcium, total calcium, vitamin D metabolites 25-hydroxyvitamin D3 (25D3) and 1,25-dihydroxyvitamin D3 (1,25D3), intact parathyroid hormone (iPTH), interleukin-6, osteocalcin, erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP); renal excretion of pyridinolin (PYD)- and desoxypyridinolin (DPD)-crosslinks. A total of 30.1% of the patients were hypercalcemic (ionized calcium >1.30 mmol/l). In comparison with normocalcemic patients, those with hypercalcemia had significantly higher ESR (P<0.01) and CRP values (P<0.05) and significantly lower serum levels of both iPTH (P<0.01) and 1,25D3 (P<0.05) and a significantly lower BMD-LS (P<0.05). The results indicate that a substantial part of RA patients is hypercalcemic. Hypercalcemia is associated with high disease activity and may contribute to suppression of PTH secretion and vitamin D hormone synthesis. High levels of ionized calcium may be a reflection of disease-activity-related systemic bone loss, and could be a predictor of BMD at the lumbar spine in RA

46. OKUDA Yet TAKASUGI K: Successful use of a humanized anti-interleukin-6 receptor antibody, tocilizumab, to treat amyloid A amyloidosis complicating juvenile idiopathic arthritis, Arthritis & Rheumatism, Vol. 54, 2997-3000., 2006
    Organism:Dohgo Spa Hosp, Ctr Rheumat Dis, Dept Internal Med, 21-21 Otsu Dohgo Himezuka, Matsuyama, Ehime 7900858, Japan Japan
    Abstract:We report an excellent clinical response to treatment with a humanized anti-interleukin-6 receptor antibody, tocilizumab, in a patient with progressive amyloid A (AA) amyloidosis complicating very active juvenile idiopathic arthritis. Treatment with tocilizumab immediately normalized the serum AA (SAA) level, and subsequently all of the clinical symptoms of AA amyloidosis disappeared. Serial gastrointestinal biopsy specimens showed marked lasting regression of AA protein deposits. The patient's functional ability score improved dramatically, she maintains her mobility, and she has regained her previous quality of life. Tocili-zumab appears to have an excellent ability to suppress SAA levels and could therefore be an important therapeutic strategy in AA amyloidosis secondary to rheumatic diseases

47. PALMISANI E, SOLARI N, MAGNI-MANZONI S, PISTORIO A, LABO E, PANIGADA S, MARTINI Aet RAVELLI A: Correlation between juvenile idiopathic arthritis activity and damage measures in early, advanced, and longstanding disease, Arthritis Rheum., Vol. 55(6), 843-849., 2006
    Organism:Istituto di Ricovero e Cura a Carattere Scientifico G Gaslini, Genoa, Italy
    Abstract:OBJECTIVE: To compare the correlation between juvenile idiopathic arthritis (JIA) measures of disease activity and damage in patients with early and late disease. METHODS: Three cohorts of patients with JIA disease duration </=1 year (early disease, n = 70), 5-9.9 years (advanced disease, n = 114), and >/=10 years (longstanding disease, n = 39) were studied. Measures included physician's global assessment of overall disease activity (MD global), parent's global assessment of the child's well-being (parent global) and pain (parent pain), joint counts, Childhood Health Assessment Questionnaire (CHAQ), erythrocyte sedimentation rate, C-reactive protein level, and Poznanski score of radiographic damage. RESULTS: In all cohorts, the MD global assessment was generally well correlated with the other variables, except the Poznanski score. The parent global assessment was correlated strongly with the parent pain assessment and moderately with the CHAQ irrespective of disease duration. Correlations between the CHAQ and the joint counts were low in early disease, moderate in advanced disease, and high to moderate in longstanding disease. Correlation between the CHAQ and the Poznanski score was low in early and advanced disease and moderate in longstanding disease. The Poznanski score was highly correlated with the number of joints with restricted motion in longstanding disease. CONCLUSION: We found important differences in the level of correlation between JIA measures of activity and damage in patients with different lengths of disease duration. These findings have important implications for clinical trials because they indicate that the responsiveness of some variables and their correlation with other variables change as disease duration changes

48. PASSACANTANDO A, PARZANESE I, RASCENTE M, PETRUCCI C, MINISOLA Get TONIETTI G: Synovial fluid OX40T lymphocytes of patients with rheumatoid arthritis display a Th2/Th0 polarization, Int.J.Immunopathol.Pharmacol., Vol. 19(3), 499-505., 2006
    Organism:ASL RMD, Rome, Italy passacant@virgilioitFAU - Passacantando, A
    Abstract:Rheumatoid arthritis (RA) is an autoimmune disease in which T-cell activation plays a pivotal role in the induction of articular damage. CD4+/OX40+ T cells accumulate in the synovial fluid (SF) of RA patients, which suggests that they are involved in the pathogenesis of the disease. In this study, we assessed the intracellular cytokine production of peripheral blood and SF CD4+ and CD4+/OX40+ T cells from RA patients in order to evaluate their role in this disorder. Our results show that SF CD4+ cells are predominantly interferon gamma (IFN-gamma)-positive and express a Th1-like cytokine pattern. In SF, significantly more CD4+/OX40+ T cells expressed interleukin-4 (IL-4) and IL4/IFN-gamma than IFN-gamma alone. Our data demonstrate that SF CD4+/OX40+ T cells express a Th2/Th0 cytokine profile, which suggests that they are involved in inflammatory responses in RA joints

49. PHELAN JDet THOMPSON SD: Genomic progress in pediatric arthritis: recent work and future goals, Current Opinion in Rheumatology, Vol. 18, 482-489., 2006
    Organism:Childrens Hosp, Med Ctr, Div Rheumatol, MLC 4010,3333 Burnett Ave, Cincinnati, OH 45229 USA USA
    Abstract:Purpose of review Pediatric arthritis is a heterogeneous group of chronic arthropathies that are influenced by complex genetic and perhaps environmental factors. Interacting genetic traits may one day be identified that provide the basis for predicting disease risk and other characteristics such as course, age of onset, and disease severity. The purpose of this review is to describe the recent progress towards identifying the multiple genes related to pediatric arthritis and understand how they relate to each other and to disease pathology.Recent findings Candidate gene studies are by far the most widely reported type of genetic studies to date for juvenile arthritis with only one genome-wide screen for juvenile rheumatoid/idiopathic arthritis published. Particular attention is paid to studies of candidate genes with potential immunological roles and those associated with other forms of autoimmunity.Summary Genomic studies may perhaps one day provide information to allow future classification systems of childhood arthritis to include molecular biomarkers as a complement to clinical observations, as well as understand how these genes or proteins relate to each other and to disease pathogenesis

50. POKORNA-KAL s, MUSZYNSKA A, REKSA D, SAPILAK BJ, DROBNIK Jet STECIWKO A: Diagnostics and treatment in diseases of the bone and joint system in general practice|, 2006
    Organism:The paper presents anatomy and physiology of joints and the most common causes of their ailment. We described the principles of management, diagnostic process and treatment of the bone and joints system impairments. Diseases of the bone and joints system affect mostly adults, but there are also several different types of juvenile chronic arthritis, for example Still disease, which begins in patients under 16 years of age and has a very acute course. Patients affected by rheumatic diseases, in most cases women, emphasize that the joint pain, called rheumatic pain, is the one of the severest problem resulted from the rheumatic disease. The pain have a different intensi ty - from weak, described as a spinning in joints - to intense, acute pain, which immobilizes patient in a bed. There have been classified over 200 different rheumatic diseases so far. (c) Copyright by Wydawnictwo Continuo

51. RIGANTE D, DE ROSA G, BERTONI B, ANSUINI V, PARDEO M, LA T, I, GASPARI Set STABILE A: Large pericardial effusion requiring pericardiocentesis as cardinal sign of macrophage activation syndrome in systemic onset-juvenile idiopathic arthritis, Rheumatol.Int., Vol. ., 2006
    Organism:Department of Pediatric Sciences, Universita Cattolica Sacro Cuore, Largo A Gemelli no 8, 00168, Rome, Italy, drigante@gmailcom
    Abstract:We report a case of large pericardial effusion which has been managed with pericardiocentesis as the main presentation feature of a dramatic clinical picture, only retrospectively framed as referred to macrophage activation syndrome in a child with juvenile idiopathic arthritis at its onset. The risk of developing this rare and severe complication should be recognized in various pathological settings of childhood, above all in children displaying systemic signs of juvenile idiopathic arthritis

52. ROSSI F, DI DIA F, GALIPO O, PISTORIO A, VALLE M, MAGNI-MANZONI S, RUPERTO N, TOMA P, MARTINI Aet RAVELLI A: Use of the Sharp and Larsen scoring methods in the assessment of radiographic progression in juvenile idiopathic arthritis, Arthritis & Rheumatism, Vol. 55, 717-723., 2006
    Organism:Ist Ricovero and Cira Carraterre Sci G Gaslini, Largo G Gaslini 5, I-16147 Genoa, Italy Italy
    Abstract:Objective. To investigate the applicability of the Sharp and Larsen scoring methods for radiographic damage in juvenile idiopathic arthritis (JIA).Methods. Wrist/hand radiographs of 25 patients with polyarthritis obtained at first observation and then yearly for 4-5 years were assessed independently by 2 pediatric rheurnatologists according to the Sharp and Larsen methods. To facilitate score assignment, each patient radiograph was compared with a bone age-related standard. A third pediatric rheurnatolo-ist measured the Poznanski score, and a pediatric radiologist provided a serniquantitative assessment of radiographic damage severity.Results. Interobserver and intraobserver agreement on longitudinal scores were good for both Sharp and Larsen methods, with intraclass correlation coefficient > 0.9. Agreement on change assessment was good for the Sharp method and moderate for the Larsen method. Both methods yielded a steady increase in scores during the study, with score chan-e being more marked in the first year. Sharp and Larsen scores were highly correlated (rs = 0.96). Correlations of both scores with the Poznanski score were moderate to high (rs from -0.62 to -0.72). Radiologist score was correlated at borderline-high level with both Sharp (rs = 0.70) and Larsen (rs = 0.71) scores. Sharp and Larsen score change from baseline to final visit was moderately to highly correlated with the number of joints with active arthritis and restricted motion and the Childhood Health Assessment Questionnaire score at final visit.Conclusion. Our results demonstrate that the Sharp and Larsen scoring systems are potentially reliable and valid for assessment of radiographic progression in patients with polyarticular JIA

53. RUPERTO N, RAVELLI A, CASTELL E, GERLONI V, HAEFNER R, MALATTIA C, KANAKOUDI-TSAKALIDOU F, NIELSEN S, BOHNSACK J, GIBBAS D, RENNEBOHM R, VOYGIOYKA O, BALOGH Z, LEPORE L, MACEJKOVA E, WULFFRAAT N, OLIVEIRA S, RUSSO R, BUONCOMPAGNI A, HILARIO MO, ALPIGIANI MG, PASSO M, LOVELL DJ, MERINO R, MARTINI Aet GIANNINI EH: Cyclosporine A in juvenile idiopathic arthritis. Results of the PRCSG/PRINTO phase IV post marketing surveillance study, Clin.Exp.Rheumatol., Vol. 24(5), 599-605., 2006
    Organism:IRCCS G Gaslini, Pediatria II-Reumatologia, PRINTO, Genova, Italy nicolaruperto@ospedale-gaslinigeitFAU - Ruperto, N
    Abstract:OBJECTIVE: To investigate the clinical use patterns, clinical effect and safety of cyclosporine A (CSA) in juvenile idiopathic arthritis (JIA) in the setting of routine clinical care. METHODS: An open-ended, phase IV post marketing surveillance study was conducted among members of the Pediatric Rheumatology Collaborative Study Group (PRCSG) and of the Paediatric Rheumatology International Trials Organisation (PRINTO) to identify patients with polyarticular course JIA who had received CSA during the course of their disease. RESULTS: A total of 329 patients, half of whom had systemic JIA, were collected in 21 countries. Data were collected during 1240 routine clinic visits. CSA was started at a mean of 5.8 years after disease onset and was given at a mean dose of 3.4 mg/kg/day. The drug was administered in combination with MTX in 61% and along with prednisone in 65% of the patients who were still receiving CSA. Among patients who were still receiving CSA therapy at the last reported visit, remission was documented in 9% of the patients, whereas in 61% of the patients the disease activity was rated as moderate or severe. The most frequent reason for discontinuation of CSA was insufficient therapeutic effect (61% of the patients); only 10% of the patients stopped CSA because of remission. In 17% of the patients, side effects of therapy was given as the primary reason for discontinuation. CONCLUSION: This survey suggests that CSA may have a less favourable efficacy profile than MTX and etanercept, whereas the frequency of side effects may be similar. The exact place of CSA in the treatment of JIA can only be established via controlled clinical trial

54. RUPP I, BOSHUIZEN HC, ROORDA LD, DINANT HJ, JACOBI CEet VAN DEN BG: Poor and good health outcomes in rheumatoid arthritis: the role of comorbidity, J.Rheumatol., Vol. 33(8), 1488-1495., 2006
    Organism:Department of Social Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands irupp@amcuvanlFAU - Rupp, Ines
    Abstract:OBJECTIVE: To assess the predictive value of selected sociodemographic characteristics, rheumatoid arthritis (RA)-specific clinical factors, and comorbidity with respect to patient-reported health outcomes, i.e., pain, disability, and health-related quality of life, among patients with RA. METHODS: Data were collected between 1997 and 2002 among 882 patients with RA of varying disease duration using questionnaires and clinical examinations. Health outcomes were evaluated over 5 years as a function of disease duration by means of random intercept linear regression. Then we selected the 10% of patients with the poorest and best health outcomes during the 5 years of followup compared to others with equal disease duration. Separate multivariate logistic regression analyses were conducted to identify factors associated with poor and good outcomes. RESULTS: Sociodemographic characteristics seemed to be less important in the prediction of health outcomes. After RA-specific clinical factors, comorbidity appeared to be a major predictive factor for health outcomes. In particular, psychological comorbidity, i.e., depressive symptomatology, was a consistent predictive factor with respect to all health outcomes. CONCLUSION: Assessment of comorbidity needs to be incorporated into the management of RA in order to prevent poor outcomes and to adapt therapies to the specific situation of individual patients. Periodic routine screening for and monitoring of somatic and psychological comorbidity should be included in clinical practice

55. SANTOS M, MARCOS R, ASSUNCAO Met MATOS AJ: Polyarthritis associated with visceral leishmaniasis in a juvenile dog, Vet.Parasitol., Vol. 141(3-4), 340-344., 2006
    Organism:Cytology Diagnostic Services, Laboratory of Histology and Embryology, ICBAS - Institute of Biomedical Sciences Abel Salazar, Porto, Portugal mssantos@icbasupptFAU - Santos, Marta
    Abstract:Canine visceral leishmaniasis (CL) is a zoonosis and a chronic systemic disease characterized by a wide spectrum of clinical signs, including, in rare occasions, polyarthritis. This report describes a case of CL in an 8-month old male boxer dog with a history of lameness, fever and lymphadenopathy. A definitive diagnosis of CL was based on the observation of the Leishmania amastigotes seen concomitantly, and for the first time, in the lymph nodes aspiration smears (in macrophages), synovial fluid (in macrophages and neutrophils) and blood (in neutrophils). Despite this extensive dissemination of the parasite, the animal was successfully treated with a multi-step combination of meglumine antimoniate, aminosidine and allopurinol

56. SANTOS M, MARCOS R, ASSUNCAO Met MATOS AUGUSTO JF: Polyarthritis associated with visceral leishmaniasis in a juvenile dog, Veterinary Parasitology, Vol. 141, 340-344., 2006
    Organism:Inst Biomed Sci Abel Salazar, Lab Histol and Embryol, Cytol Diagnost Serv, Lg Prof Abel Salazar 2, P-4099003 Oporto, Portugal Portugal
    Abstract:Canine visceral leishmaniasis (CL) is a zoonosis and a chronic systemic disease characterized by a wide spectrum of clinical signs, including, in rare occasions, polyarthritis. This report describes a case of CL in an 8-month old male boxer dog with a history of lameness, fever and lymphadenopathy. A definitive diagnosis of CL was based on the observation of the Leishmania amastigotes seen concomitantly, and for the first time, in the lymph nodes aspiration smears (in macrophages), synovial fluid (in macrophages and neu trophils) and blood (in neutrophils). Despite this extensive dissemination of the parasite, the animal was successfully treated with a multi-step combination of meglumine antimoniate, aminosidine and allopurinol. (c) 2006 Elsevier B.V. All rights reserved

57. SAURENMANN RK, LEVIN AV, FELDMAN BM, LAXER RM, SCHNEIDER Ret SILVERMAN ED: Risk of new-onset uveitis in patients with juvenile idiopathic arthritis treated with anti-TNFalpha agents, J.Pediatr., Vol. 149(6), 833-836., 2006
    Organism:Division of Rheumatology, the Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada traudelsaurenmann@kispiunizhchFAU - Saurenmann, R K
    Abstract:OBJECTIVE: To determine whether treatment with tumor necrosis factor alpha (TNFalpha)-blocking agents alters the incidence of new-onset uveitis in patients with juvenile idiopathic arthritis (JIA). STUDY DESIGN: Cohort study based on retrospective chart review. The charts of all 1109 patients with a diagnosis of JIA seen between January 1, 1996, and June 30, 2003, at our clinic were reviewed for diagnosis of uveitis and treatment with TNFalpha inhibitors. Cox regression analysis was performed with anti-TNFalpha treatment as a time-dependent covariate for risk of development of uveitis. RESULTS: We identified 70 patients treated with anti-TNFalpha without a prior diagnosis of uveitis. Two of these 70 patients (2.9%), both treated with etanercept, had development of new-onset uveitis during anti-TNFalpha therapy. One had juvenile psoriatic arthritis diagnosed 4.1 years before onset of uveitis. The other had extended oligoarticular JIA diagnosed 6.4 years before onset of uveitis. We found no statistically significant difference in the risk for development of uveitis between patients with or without anti-TNFalpha treatment. CONCLUSIONS: In our patients with JIA, anti-TNFalpha treatment did not alter the risk for development of new-onset uveitis. However, anti-TNFalpha therapy with etanercept did not prevent the development of uveitis in 2 patients

58. SHAW KL, HACKETT JL, SOUTHWOOD TRet MCDONAGH JE: The prevocational and early employment needs of adolescents with juvenile idiopathic arthritis: The occupational therapy perspective|, 2006
    Organism:The purpose of the study was to explore the prevocational needs of adolescents with juvenile idiopathic arthritis (JIA) from the perspective of occupational therapists and to examine the role of occupational therapy in addressing these. A questionnaire was distributed to members of occupational therapy organisations (n = 494) to assess the perceived importance of addressing prevocational issues for adolescents with JIA and the respondents' knowledge, confidence and perceived role. The questionnaire was completed by 175 (35.4%) individuals. The results showed that although the occupational ther apists felt that they were an appropriate profession to address the vocational needs of adolescents, they reported limited knowledge and confidence to do so. Significant unmet training needs were highlighted. This study echoes previous calls for vocational issues to be addressed within adolescent rheumatology and provides evidence that occupational therapy is well placed to coordinate this area of need

59. SIM KT, VENNING HE, BARRETT S, GREGSON RMet AMOAKU WM: Extended Oligoarthritis and Other Risk Factors for Developing JIA-Associated Uveitis Under ILAR Classification and Its Implication for Current Screening Guideline, Ocul.Immunol.Inflamm., Vol. 14(6), 353-357., 2006
    Organism:Queens Medical Centre, Department of Ophthalmology and Visual Science, Nottingham, UKFAU - Sim, Kuan T
    Abstract:Purpose: To investigate the risk factors for developing uveitis in a regional cohort of patients with juvenile idiopathic arthritis (JIA) as classified under ILAR criteria. Patients and Methods: The clinical factors for developing uveitis and its visual outcome were studied retrospectively for all children diagnosed with JIA at Nottingham University Hospital, England from 1974 to 2001. Results: A total of 202 patients with juvenile idiopathic arthritis were identified. Twenty-three patients (11.4%) were found to have uveitis. The mean age of arthritis onset in those with uveitis was 4.9 (95% CI 3.4-6.4) and in those without uveitis was 7.6 (95% CI 7.0-8.3), p = 0.002. Both the persistent and extended oligoarthritis groups are at significant risk of developing uveitis on Kaplan-Meier analysis with p = 0.001 and 0.013, respectively, compared to other ILAR subtypes. Extended oligoarthritis (1 to 4 joints affected in first 6 months of disease but 5 or more cumulative joints after first 6 months) had the highest prevalence of uveitis (25%) among the ILAR subtypes. Patients with extended oligoarthritis also developed uveitis earlier than persistent group, p = 0.017. Gender, race, and antinuclear antibody (ANA) status were not significant risk factors. The visual outcome was favorable, with 90% achieving acuity of 6/12 or better. Conclusion: Patients with extended oligoarthritis are at higher risk and have a shorter interval from diagnosis of arthritis to development of uveitis and need to be monitored more closely. Screening guideline for JIA-associated uveitis based on ILAR classification is called for

60. SOLOMON DH, GOODSON NJ, KATZ JN, WEINBLATT ME, AVORN J, SETOGUCHI S, CANNING Cet SCHNEEWEISS S: Patterns of cardiovascular risk in rheumatoid arthritis, Ann.Rheum.Dis., Vol. 65(12), 1608-1612., 2006
    Organism:Division of Pharmacoepidemiology, Brigham and Women's Hospital, 1620 Tremont Street, Suite 3030, Boston, MA 02120, USA dhsolomon@partnersorgFAU - Solomon, D H
    Abstract:BACKGROUND: Although it is known that rheumatoid arthritis is associated with an increased risk of cardiovascular disease (CVD), the pattern of this risk is not clear. This study investigated the relative risk of myocardial infarction, stroke and CVD mortality in adults with rheumatoid arthritis compared with adults without rheumatoid arthritis across age groups, sex and prior CVD event status. METHODS: We conducted a cohort study among all residents aged >or=18 years residing in British Columbia between 1999 and 2003. Residents who had visited the doctor at least thrice for rheumatoid arthritis (International Classification of Disease = 714) were considered to have rheumatoid arthritis. A non-rheumatoid arthritis cohort was matched to the rheumatoid arthritis cohort by age, sex and start of follow-up. The primary composite end point was a hospital admission for myocardial infarction, stroke or CVD mortality. RESULTS: 25 385 adults who had at least three diagnoses for rheumatoid arthritis during the study period were identified. During the 5-year study period, 375 patients with rheumatoid arthritis had a hospital admission for myocardial infarction, 363 had a hospitalisation for stroke, 437 died from cardiovascular causes and 1042 had one of these outcomes. The rate ratio for a CVD event in patients with rheumatoid arthritis was 1.6 (95% confidence interval (CI) 1.5 to 1.7), and the rate difference was 5.7 (95% CI 4.9 to 6.4) per 1000 person-years. The rate ratio decreased with age, from 3.3 in patients aged 18-39 years to 1.6 in those aged >or=75 years. However, the rate difference was 1.2 per 1000 person-years in the youngest age group and increased to 19.7 per 1000 person-years in those aged >or=75 years. Among patients with a prior CVD event, the rate ratios and rate differences were not increased in rheumatoid arthritis. CONCLUSIONS: This study confirms that rheumatoid arthritis is a risk factor for CVD events and shows that the rate ratio for CVD events among subjects with rheumatoid arthritis is highest in young adults and those without known prior CVD events. However, in absolute terms, the difference in event rates is highest in older adults

61. STOLL ML, ZURAKOWSKI D, NIGROVIC LE, NICHOLS DP, SUNDEL RPet NIGROVIC PA: Patients with juvenile psoriatic arthritis comprise two distinct populations, Arthritis Rheum., Vol. 54(11), 3564-3572., 2006
    Organism:Children's Hospital Boston, Harvard Medical School, Massachusetts 02115, USAFAU - Stoll, Matthew L
    Abstract:OBJECTIVE: Psoriatic arthritis (PsA) in children is clinically heterogeneous. We examined a large population of children with juvenile PsA for evidence of phenotypic clustering that could suggest the presence of distinct clinical entities. METHODS: We reviewed the medical records of 139 patients meeting the Vancouver criteria for juvenile PsA. To identify segregation into phenotypic groups, we compared younger patients with their older counterparts and subjected the whole population to 2-step cluster analysis. RESULTS: Among patients with juvenile PsA, the age at onset is biphasic, with peaks occurring at approximately 2 years of age and again in late childhood. Compared with children ages 5 years and older, younger patients are more likely to be female, exhibit dactylitis and small joint involvement, and express antinuclear antibodies. Progression to polyarticular disease (>or=5 joints) is more common in younger children, although joint involvement remains oligoarticular in the majority of children. In contrast, older patents tend to manifest enthesitis, axial joint disease, and persistent oligoarthritis. Uveitis is equally represented in both age groups. Despite a higher utilization of methotrexate therapy, younger patients required, on average, more than twice as long to achieve clinical remission (23 months versus 9.2 months; P = 0.044). Cluster analysis identified largely overlapping subgroups but suggested that the presence of dactylitis, rather than age, has the greatest capacity to predict essential features of the clinical phenotype. CONCLUSION: Juvenile PsA comprises 2 distinct populations of patients. Although the pathophysiologic correlate of this finding remains undefined, future studies should avoid the assumption that PsA in childhood constitutes a single etiologic entity

62. STOLL MLet NIGROVIC PA: Subpopulations within juvenile psoriatic arthritis: A review of the literature, Clin.Dev.Immunol., Vol. 13(2), 377-380., 2006
    Organism:Division of Immunology, Program in Rheumatology, Children's Hospital Boston, Boston, MA, USAFAU - Stoll, Matthew L
    Abstract:The presentation of juvenile psoriatic arthritis (JPsA) has long been recognized to be clinically heterogeneous. As the definition of JPsA expanded to accommodate atypical manifestations of psoriasis in young children, studies began to reflect an increasingly clear biphasic distribution of age of onset, with peaks in the first few years of life and again in early adolescence. These two subpopulations differ in gender ratio, pattern of joint involvement, laboratory findings and potentially response to therapy. Intriguingly, a similar distribution of age of onset has been observed in juvenile rheumatoid arthritis (JRA), and correlates with patterns of HLA association. While a secure classification of subpopulations within JPsA awaits improved pathophysiologic understanding, future research must consider the possibility that different disease mechanisms may be operative in distinct subsets of patients with this disorder

63. SUZUKI M, MIYAGI J, KURIBAYASHI M, NEGISHI E, UENO Ket MORIYA H: Evaluation of allele frequencies in the PADI4 gene and anti-cyclic citrullinated peptide antibodies of patients with rheumatoid arthritis in a Japanese population, Ann.Rheum.Dis., Vol. 65(10), 1399-1400., 2006
    Organism:

64. SZTAJNBOK F, CORONEL-MARTINEZ DL, DIAZ-MALDONADO A, NOVARINI C, PISTORIO A, VIOLA S, RUPERTO N, BUONCOMPAGNI A, MARTINI Aet RAVELLI A: Discordance between physician's and parent's global assessments in juvenile idiopathic arthritis|, 2007
    Organism:Objective. To investigate the discrepancy between physician's and parent's global assessments of disease status and the factors explaining discordance in patients with juvenile idiopathic arthritis (JIA). Methods. The mothers of 197 patients with JIA rated the child's overall well-being on a 10 cm visual analogue scale (VAS) and the attending physician rated the child's overall disease activity on a 10 cm VAS. A discordance score was calculated by subtracting the physician's global assessment from that of the parent's, leading to the definition of three patient groups: (1) no discordance, when physician's and parent's assessments were within 1 cm of each other; (2) negative discordance, when parent's assessment was underrated relative to the physician; and (3) positive discordance, when parent's assessment was over-rated relative to the physician. Negative and positive discordance was defined as 'marked' when the difference between the two assessments was greater than 3 cm. Results. No discordance was found in 40.6% of the patients. Negative discordance was found in 51.3% of the patients, with 34% showing marked discordance. Positive discordance was found in 8.1% of the patients, w ith 2% showing marked discordance. Significant differences between groups included a shorter disease duration among patients with a markedly positive discordance (P = 0.02) and a greater frequency of ongoing second-line drug therapy among patients with no discordance or with positive discordance (P = 0.008). Patients with no discordance or with marked positive discordance had a significantly lower joint counts (P = 0.02-0.004). Conclusion. Parents and physicians often perceive the health status of children with JIA differently, with parents providing most frequently lower rating. (c) 2007 Oxford University Press

65. TANAKA H, TSUGAWA K, SUZUKI K, OKI ES, NONAKA K, KIMURA Set ITO E: Treatment of difficult cases of systemic-onset juvenile idiopathic arthritis with tacrolimus, Eur.J.Pediatr., Vol. ., 2006
    Organism:Department of Pediatrics, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562, Japan, hirotana@cchirosaki-uacjp
    Abstract:Since a proportion of systemic-onset juvenile idiopathic arthritis (SOJIA) patients continue to require long-term corticosteroid therapy for disease control, an effective and safe therapeutic strategy for controlling the activity of refractory SOJIA remains to be established. We report the efficacy of tacrolimus for the treatment of SOJIA in two patients with refractory SOJIA, one of them showing poor response to cyclosporine A. Tacrolimus might be the treatment of choice in selected patients with refractory systemic-onset juvenile idiopathic arthritis. Further studies to confirm the long-term efficacy and safety of tacrolimus in larger numbers of patients are, however, needed

66. TOLEDO MARIA MM, MARTINI G, GIGANTE C, DA DALT L, TREGNAGHI Aet ZULIAN F: Is there a role for arthroscopic synovectomy in oligoarticular juvenile idiopathic arthritis?, Rinsho Ganka, Vol. 33, 1868-1872., 2006
    Organism:Univ Padua, Dipartimento Pediat, Via Giustiniani 3, I-35128 Padua, Italy Italy
    Abstract:Objective. To evaluate the longterm efficacy and safety of arthroscopic synovectomy (AS) in children with oligoarticular juvenile idiopathic arthritis (JIA).Methods. Patients with oligoarticular JIA and persistent monoarticular involvement, refractory to nonsteroidal anti inflammatory drugs (NSAID) and/or intraarticular corticosteroid (IAC) treatment underwent AS followed, one month later, by IAC. The efficacy of AS was prospectively evaluated, and a good response was defined as absence of synovitis or >= 60% decrease in articular score from baseline. Clinical, laboratory, and radiological variables (radiographs, ultrasound, magnetic resonance imaging) were noted to examine possible factors predictive of the result.Results. Twenty-two patients with JIA (15 female, 7 male) entered the study. Age at disease onset was 77 months (range 13-168). Mean disease duration at the time of AS was 50 months (3-324). Nineteen knees, 2 temporomandibular joints, and one shoulder were treated; the mean followup was 57 months (12-168). Thirty-six percent of patients relapsed within 12 months of the procedure, 14% within 24 months, and 14% thereafter. Eight patients (36%) remain in remission after a mean 65 months' followup. Variables found to be predictive of good response were persistent monoarticular course (p 0.004), short disease duration at the time of AS (p = 0.03), and normal erythrocyte sedimentation rate and C-reactive protein at baseline (p = 0.008 and 0.01, respectively).Conclusions. AS is a safe but only partially effective procedure in patients with oligoarticular JIA. Best results are achieved early in the disease course in children with persistent monoarticular involvement and no evidence of systemic inflammation

67. TOLEDO MM, MARTINI G, GIGANTE C, DA DALT L, TREGNAGHI Aet ZULIAN F: Is there a role for arthroscopic synovectomy in oligoarticular juvenile idiopathic arthritis?, J.Rheumatol., Vol. 33(9), 1868-1872., 2006
    Organism:Department of Pediatrics, University of Padua, Padua, ItalyFAU - Toledo, Maria M Martinez
    Abstract:OBJECTIVE: To evaluate the longterm efficacy and safety of arthroscopic synovectomy (AS) in children with oligoarticular juvenile idiopathic arthritis (JIA). METHODS: Patients with oligoarticular JIA and persistent monoarticular involvement, refractory to nonsteroidal antiinflammatory drugs (NSAID) and/or intraarticular corticosteroid (IAC) treatment underwent AS followed, one month later, by IAC. The efficacy of AS was prospectively evaluated, and a good response was defined as absence of synovitis or > or = 60% decrease in articular score from baseline. Clinical, laboratory, and radiological variables (radiographs, ultrasound, magnetic resonance imaging) were noted to examine possible factors predictive of the result. RESULTS: Twenty-two patients with JIA (15 female, 7 male) entered the study. Age at disease onset was 77 months (range 13-168). Mean disease duration at the time of AS was 50 months (3-324). Nineteen knees, 2 temporomandibular joints, and one shoulder were treated; the mean followup was 57 months (12-168). Thirty-six percent of patients relapsed within 12 months of the procedure, 14% within 24 months, and 14% thereafter. Eight patients (36%) remain in remission after a mean 65 months' followup. Variables found to be predictive of good response were persistent monoarticular course (p = 0.004), short disease duration at the time of AS (p = 0.03), and normal erythrocyte sedimentation rate and C-reactive protein at baseline (p = 0.008 and 0.01, respectively). CONCLUSION: AS is a safe but only partially effective procedure in patients with oligoarticular JIA. Best results are achieved early in the disease course in children with persistent monoarticular involvement and no evidence of systemic inflammation

68. TSAI WS, YANG YH, WANG LCet CHIANG BL: Abrupt temperature change triggers arthralgia in patients with juvenile rheumatoid arthritis, J.Microbiol.Immunol.Infect., Vol. 39(6), 465-470., 2006
    Organism:Department of Pediatrics, National Taiwan University Hospital, Taipei, TaiwanFAU - Tsai, Wei Shien
    Abstract:BACKGROUND AND PURPOSE: Juvenile rheumatoid arthritis (JRA) is the most common form of arthritis in children and affects both quality of life and school attendance. Weather and temperature conditions are believed to affect joint pains; however, very few studies have investigated this issue. This study examined the association between joint pain in JRA patients and weather conditions. METHODS: The daily pain ratings of 52 patients previously diagnosed with JRA were recorded on visual analog scales over 4 months beginning January 1, 2004. These ratings were then compared with weather data to evaluate possible correlation between these two factors. RESULTS: Twenty nine patients kept daily records during the first 2 months. There was no positive correlation between weather parameters (such as temperature, humidity, and barometric pressure) and pain ratings. Interestingly, the pain rating significantly increased the day after the advent of a cold wave (sign test, p<0.01; Wilcoxon signed ranks test, p=0.001). The number of patients who experienced joint swelling was not related to weather conditions. Twenty one participants continued maintaining the diaries during the next 2 months. The patients reported higher pain levels in the first 2 months during the cold wave period than in the next 2 months when the cold wave period had ended (p<0.001). CONCLUSION: A dramatic weather change such as a sudden cold wave might influence the experience of joint pain

69. VAN DER HM, BULL S, BRAMA PA, BARNEVELD AB, VAN WEEREN PRet VAN DE LC: Nitrite and nitrotyrosine concentrations in articular cartilage, subchondral bone, and trabecular bone of normal juvenile, normal adult, and osteoarthritic adult equine metacarpophalangeal joints, J.Rheumatol., Vol. 33(8), 1662-1667., 2006
    Organism:Department of Equine Sciences, Faculty of Veterinary Medicine, Utrecht University, Utrecht, The Netherlands markvanderharst@hetnetnlFAU - van der Harst, Mark
    Abstract:OBJECTIVE: Osteoarthritis (OA) is a chronic debilitating joint disorder in which the importance of inflammation is increasingly recognized. In advanced cases, both the articular cartilage and the underlying bony layers are affected, but the exact sequence of events and their localization in the initial phase of pathogenesis remain uncertain. We measured nitric oxide (NO) end products in tissue layers that constitute the bearing surface of the joint, as possible indicators of physiological and pathological processes. METHODS: Nitrite as a measure for NO and nitrotyrosine was measured in articular cartilage, subchondral bone, and the underlying trabecular bone of the proximal articular surface of the first phalanx of healthy mature horses (n = 15; age range 5-18 yrs), mature horses affected by OA (n = 15; age range 8-22 yrs), and unaffected juvenile horses (n = 13; age range 6 months-4 yrs). Data were correlated with cartilage damage, as quantified by the Cartilage Degeneration Index. RESULTS: In all 3 layers the nitrite concentration was higher in OA joints (cartilage, p < 0.001; subchondral and trabecular bone, p < 0.05). The concentration of nitrite was significantly higher in cartilage and subchondral bone of juvenile horses compared with mature horses (p < 0.001). Nitrotyrosine concentrations were significantly higher in subchondral bone of OA horses compared with healthy controls (p < 0.001), but significantly lower in trabecular bone of juvenile horses (p < 0.01). CONCLUSION: The similarities observed over the 3 tissue layers support the concept of the bearing surface of the joint as a functional entity. Nitrite concentration seems to be a good indicator of tissue metabolic activity, but cannot discriminate between physiological (juvenile animals) and pathological (OA cases) processes. The increased nitrotyrosine levels in subchondral bone of OA-affected animals suggest that this layer is important in early or moderate OA, and implies a role of oxidative stress in the development of the disease

70. VENKATESH P, MACRAE Met FLECK BW: Lothian combined paediatric ophthalmology and rheumatology service, Br.J.Ophthalmol., Vol. 90(12), 1549-1550., 2006
    Organism:

71. WELCH TR: Uveitis in juvenile idiopathic arthritis, J.Pediatr., Vol. 149(6), A3, 2006
    Organism:

72. YOU CR, KIM HR, YOON CH, LEE SH, PARK SHet KIM HY: Macrophage activation syndrome in juvenile rheumatoid arthritis successfully treated with cyclosporine a: a case report, J.Korean Med.Sci., Vol. 21(6), 1124-1127., 2006
    Organism:Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul, KoreaFAU - You, Chan Ran
    Abstract:Macrophage activation syndrome (MAS) is one of the serious complications of juvenile rheumatoid arthritis (JRA) and recently, cyclosporine A has been found to be effective in patients with corticosteroid-resistant MAS. A 29-yr-old male was admitted with high fever and jaundice for one month. He was diagnosed as juvenile arthritis 16 yr ago. Physical and laboratory results showed hepatosplenomegaly, high fever, pancytopenia and impaired liver and renal function tests, elevated triglyceride and serum ferritin levels. Bone marrow biopsy showed hyperplasia of histiocytes with active hemophagocytosis. He was diagnosed as MAS associated with juvenile rheumatoid arthritis and managed with high-dose corticosteroids initially, but clinical symptoms and laboratory findings did not improve immediately. Finally, he completely recovered after treatment with cyclosporine A (3 mg/kg/day)

73. YOUNG D: FDA advisers endorse Celebrex for juvenile rheumatoid arthritis: Lack of studies in children raises safety concerns, Am.J.Health Syst.Pharm., Vol. 64(1), 11-12., 2007
    Organism:

74. ZIMMERMANN-GORSKA I: Still's disease in adults|, 2006
    Organism:Still's disease is a variant of juvenile chronic arthritis, characterized by seronegative polyarthritis in association with systemic inflammatory illness. In adults onset of Still's disease is observed usually under the age of 35. The most common symptoms are: arthralgia, fever, myalgia, arthritis, sore throat, rash, lymphadenopathy, spleno- and hepatomegaly, pleuritis, pericarditis and abdominal pain. Frequent laboratory features are: elevated ESR, leukocytosis, anemia, thrombocytosis, elevated hepatic enzymes, elevated serum ferritin. Patients should be treated with NSAIDs, in some cases corticosteroid therapy. DMARDs as well as TNF-alpha inhibitors may be used. In some patients there is a need for joint arthroplasty, especially of the hip. Death in Still's disease may be attributed mainly to infections, liver failure, amyloidosis, respiratory distress syndrome and heart failure. (c) Copyright by Wydawnictwo Continuo

75. ZINK A, STRANGFELD A, SCHNEIDER M, HERZER P, HIERSE F, STOYANOVA-SCHOLZ M, WASSENBERG S, KAPELLE Aet LISTING J: Effectiveness of tumor necrosis factor inhibitors in rheumatoid arthritis in an observational cohort study: comparison of patients according to their eligibility for major randomized clinical trials, Arthritis Rheum., Vol. 54(11), 3399-3407., 2006
    Organism:German Rheumatism Research Centre and Charite University Medicine, Berlin, Germany Zink@DRFZdeFAU - Zink, Angela
    Abstract:OBJECTIVE: Randomized clinical trials (RCTs) evaluate the efficacy of treatments in selected groups of patients defined by strict inclusion criteria. The value of these trials in predicting therapeutic effectiveness in "real world" patients is limited. This observational cohort study was designed to complement the knowledge obtained in RCTs by evaluating the effectiveness of tumor necrosis factor (TNF) inhibitors in patients with rheumatoid arthritis (RA) according to their eligibility for the major trials. METHODS: Using the data from the German biologics register Rheumatoid Arthritis Observation of Biologic Therapy (RABBIT [in German]), we investigated how many of the RA patients who were treated with a TNF inhibitor (infliximab, etanercept, or adalimumab) would have been eligible for the major clinical trials that led to approval of the drugs. In addition, therapeutic effectiveness was compared in the eligible and ineligible patients using the American College of Rheumatology 20% (ACR20) and 50% (ACR50) improvement response criteria. RESULTS: Only 21-33% of the patients in the RABBIT register would have been eligible for the major trials. In these patients, the ACR20 and ACR50 improvement responses, indicating therapeutic effectiveness, were comparable with the response rates in the published trials. ACR response rates were lower in those patients considered ineligible for the trials; however, absolute improvement was similar to that in eligible patients. Ineligible patients had lower baseline disease activity, more comorbidity, and lower functional status. CONCLUSION: RCT cohorts reflect only a minor proportion of the patients treated with biologic agents in routine care. In the clinic setting, the indications for treatment with biologic agents are not identical to the inclusion criteria for trials. Despite the smaller relative improvement achieved in these patients with longstanding, severe RA who would not fulfill the inclusion criteria of a major trial, the majority of such patients would nevertheless benefit from biologic therapy

76. ZOEGER A, BLAU M, EGERER K, FEIST Eet DAHLMANN B: Circulating proteasomes are functional and have a subtype pattern distinct from 20S proteasomes in major blood cells, Clin.Chem., Vol. 52(11), 2079-2086., 2006
    Organism:Institut fur Biochemie, Charite-Universitatsmedizin-Berlin, Berlin, GermanyFAU - Zoeger, Annette
    Abstract:BACKGROUND: 20S proteasomes, the proteolytic core particles of the major intracellular protein degradative pathway, are potential disease markers because they are detectable in human plasma as circulating proteasomes and their concentrations are increased in patients suffering from various diseases. To investigate the origin of circulating proteasomes, we compared some of their features with those of proteasomes isolated from major blood cells. METHODS: We isolated circulating proteasomes from the plasma of 2 patients with rheumatoid arthritis and 2 with systemic lupus erythematosus and from human plasma from healthy donors. We purified the proteasomes to apparent homogeneity and then used electron microscopy for imaging and chromatography for subtype spectrum analysis. We compared subtype results with those from 20S proteasomes purified from 4 major blood cell populations. We also tested proteasomes for enzymatic activity and immunosubunit content. RESULTS: Circulating proteasomes from plasma of healthy donors and from patients with autoimmune disease were found to have the same size and shape as erythrocyte proteasomes, be proteolytically active, and contain standard- and immunosubunits. Chromatography revealed 6 circulating proteasome subtype peaks in healthy donor plasma and 7 in patient donor plasma. Proteasomes from erythrocytes had 3 subtype peaks and those of monocytes, T-lymphocytes, and thrombocytes each had 5 different subtype peaks. CONCLUSION: Circulating proteasomes were intact and enzymatically active in plasma from healthy donors and from patients with autoimmune disease. Because the subtype patterns of circulating proteasomes clearly differ from those of proteasomes from blood cells, these cells cannot be regarded as a major source of circulating proteasomes

77. ZUBER Z, PIEGZA A, SOBCZYK Met GARLICKA A: Clinical utility of the anti-cyclic citrullinated peptide assay in children with juvenile idiopathic arthritis|, 2006
    Organism:Antibodies against cyclic citrullinated peptide (anti-CCP) are considered to be specific for rheumatoid arthritis (RA). Aim: to correlate serum anti-cyclic citrullinated peptide antibody levels with juvenile idiopathic arthritis (JIA). The study group comprised 51 patients with JIA (16 boys, 35 girls). Diagnoses were made using ILAR criteria from 1997, patients mean age was 10.75 years (from 3 to 17 years old), mean disease duration was 2.5 years (from 3 months to 13.5 years). As a control group, anti-CCP were tested in sera of 21 children with neurological diseases. Anti-CCP were tested by en zyme-linked immunosorbent assay (ELISA) in serum, values above 5 relative units (>5 RU/ml) were regarded as positive. Positive anti-CCP values were found in sera of three girls with seropositive polyarticular JIA (5.88%). Anti-CCP positivity is identified rarely in JIA patients; it cannot be used as a diagnostic test with high specificity. Detection of anti-CCP positivity in a specific group of patients: girls with seropositive polyarticular JIA, allow to separate picture closest to the classical form of RA in adult patients