Bibliography January 2008

1. AGARWAL S, MISRA Ret AGGARWAL A: Interleukin 17 Levels Are Increased in Juvenile Idiopathic Arthritis Synovial Fluid and Induce Synovial Fibroblasts to Produce Proinflammatory Cytokines and Matrix Metalloproteinases, J.Rheumatol., Vol. ., 2008
   Organism:From the Department of Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
   Abstract:OBJECTIVE: Cytokines are the major mediators of joint damage in chronic arthritis. Data on synovial fluid (SF) concentration of Th17 cell-derived cytokine interleukin 17 (IL-17) in patients with juvenile idiopathic arthritis (JIA) are sparse. We measured levels of IL-17 in SF specimens from children with enthesitis-related arthritis (ERA) and polyarticular JIA (poly-JIA), and studied the ability of IL-17 to produce matrix metalloproteinases (MMP) and cytokines by fibroblast-like synoviocytes (FLS) from patients with ERA. METHODS: IL-17 levels were measured in SF of patients with ERA (n = 43), poly-JIA (n = 17), rheumatoid arthritis (RA; n = 35), and osteoarthritis (OA; n = 10) by ELISA. In patients with JIA, 10 paired serum samples were also assayed. FLS were cultured from SF of patients with ERA and subsequently stimulated for 48 h by IL-17 or tumor necrosis factor-alpha. Later the production of IL-6, IL-8, MMP-1, MMP-3, and tissue inhibitor of metalloproteinase (TIMP)-1 was measured in the culture supernatants by ELISA. RESULTS: Median IL-17 levels in SF were higher in patients with JIA [28 pg/ml (range 0-200)] compared to OA [0 pg/ml (range 0-84); p < 0.001] and RA (p < 0.05). The levels were comparable between poly-JIA patients and the ERA group. The median SF IL-17 levels were significantly higher compared to serum levels in children with JIA (p < 0.005). In ERA, SF IL-17 correlated with number of swollen joints (r = 0.35; p < 0.05), number of joints with limited mobility (r = 0.55; p < 0.001), and number of tender joints (r = 0.46; p < 0.01); however, no correlation was seen with erythrocyte sedimentation rate. IL-17 induced FLS to produce IL-6, IL-8, MMP-3, and MMP-1. However, there was no effect on the production of TIMP. CONCLUSION: Increased IL-17 levels in ERA SF correlate with disease activity and this may be due to increased production of MMP and cytokines by IL-17

2. ARMAGAN O, EKIM A, DINC Aet ONER C: Ankylosing spondylitis in a patient with Turner syndrome: a case report, Rheumatol.Int., Vol. 27(12), 1177-1180., 2007
   Organism:Department of Physical Therapy and Rehabilitation, Faculty of Medicine, Eskisehir Osmangazi University Medical School, Meselik Campus, 26480, Eskisehir, Turkey aoarmagan@superonlinecomFAU - Armagan, Onur
   Abstract:Turner's syndrome (TS) is a chromosomal disorder where phenotypic females have either a missing chromosome (45 X0) or a structural aberration of one of the chromosomes. It is possible for TS to accompany such autoimmune diseases as thyroid diseases, inflammatory intestinal diseases, diabetes mellitus, psoriatic arthritis and juvenile rheumatoid arthritis. Herein, we present an unusual case with Ankylosing spondylitis (AS) and autoimmune thyroiditis associated with TS. We suggest that the possibility that TS patients may also develop such other diseases as AS apart from the already known accompanying autoimmune diseases should not be ruled out when monitoring TS patients

3. ARTAC H, ALP H, KELES S, REISLI Iet TUNC R: Systemic onset juvenile rheumatoid arthritis presenting with absence of B lymphocytes, Rheumatol.Int., Vol. 27(10), 981-983., 2007
   Organism:Departments of Pediatrics, Pediatric Immunology and Allergy Division, Selcuk University Meram Medical Faculty, Beysehir Yolu, 42080 Konya, Turkey hasibeartac@yahoocomFAU - Artac, Hasibe
   Abstract:Juvenile rheumatoid arthritis (JRA) is a complex disease involving the interactions of several cell populations with different mediators. Herein, we report a five-year-old girl with systemic-onset JRA. At admission, peripheral blood flowcytometric analysis showed the percentages of CD19(+) and CD20(+) B cells were <1%. These values returned to normal on the tenth day of steroid treatment. This is the first report of JRA presented with absence of B lymphocytes in the literature and suggested that lymphocytes subset analysis could change with treatment in patients with JRA. Different clinical signs and symptoms reflecting aspects of JRA are critical for the etiology of the disease and to identify new strategies for treatment

4. ATZENI F, DORIA A, GHIRARDELLO A, TURIEL M, BATTICCIOTTO A, CARRABBA Met SARZI-PUTTINI P: Anti-thyroid antibodies and thyroid dysfunction in rheumatoid arthritis: prevalence and clinical value, Autoimmunity., Vol. 41(1), 111-115., 2008
   Organism:Rheumatology Unit, L Sacco University Hospital, Milan, ItalyFAU - Atzeni, Fabiola
   Abstract:OBJECTIVES: The aim of this study was to assess thyroid function as well as the prevalence and clinical value of anti-thyroid antibodies in patients with rheumatoid arthritis (RA). METHODS: Seventy patients with active RA (ACR criteria), 9 males and 61 females, mean age 47 years (range 15-77) were analyzed. Anti-thyroperoxidase (TPOAb) and anti-thyroglobulin antibodies (TgAb) were tested using radioimmunoassay. Free thyroxine (FT4) and free triiodothyronine (FT3) and thyroid-stimulating hormone (TSH) serum levels were measured using electro-immunochemiluminescence (ECLIA, Elecsys Roche). Clinical variables, including tender and swollen joint count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), anti-cyclic citrullinated peptide antibody (anti-CCP) and antinuclear antibodies (ANA) were also evaluated. Statistics were performed by the SPSS statistical software for Windows. RESULTS: Twenty-six patients (37%) with RA were positive for TPOAb and 16 (23%) for TgAb. In 5 (7.1%) patients TSH level was slightly elevated, ranging between 4.52 and 15.65 UI/ml. The increase of TSH levels was associated with normal FT4 in 3 cases (4.2%) and with reduced FT4 in 2 cases (2.8%). One patient (1.5%) had low TSH serum value along with normal FT4. No differences in clinical and serological data between anti-thyroid positive and negative patients were observed. CONCLUSION: Our study shows an increased prevalence of anti-thyroid antibodies in RA patients with a low prevalence of hormonal alterations. However, anti-thyroid antibodies do not seem to identify any peculiar RA phenotype

5. BEKKERING WP, TEN CATE R, VAN ROSSUM MAet VLIET VLIELAND TP: A comparison of the measurement properties of the Juvenile Arthritis Functional Assessment Scale with the childhood health assessment questionnaire in daily practice, Clin.Rheumatol., Vol. 26(11), 1903-1907., 2007
   Organism:Department of Physical Therapy (HO-Q), Leiden University Medical Centre, PO Box 9600, 2300 RC, Leiden, The Netherlands WPBekkering@LUMCNLFAU - Bekkering, W Peter
   Abstract:We compared the measurement properties of a performance test (Juvenile Arthritis Functional Assessment Scale; JAFAS) with a questionnaire-based instrument (Childhood Health Assessment Questionnaire; CHAQ) to measure functional ability in patients with juvenile idiopathic arthritis on the level of individual items. In 28 consecutive children visiting an outpatient paediatrics clinic, the JAFAS (range 0-20) and CHAQ (range 0-3) were applied, and measures of disease activity and joint range of motion (ROM) were determined. Twenty-eight children with a median age of 10 years and median disease duration of 3.2 years were included. The median JAFAS score was 0, and the median CHAQ score was 0.125. Cronbach's alpha was 0.92 for the JAFAS and 0.96 for the CHAQ. The Spearman correlation coefficient between the JAFAS and the CHAQ was 0.55 (P < 0.01). With six out of ten items, the JAFAS classified the child as less disabled than with corresponding CHAQ activities. Overall, associations with measures of disease activity and ROM were higher for the CHAQ than for the JAFAS. A performance test (JAFAS) does not appear to have an added benefit over the questionnaire-based assessment (CHAQ) of physical function in a cross-sectional study

6. CAMFIELD CSet CAMFIELD PR: Long-term social outcomes for children with epilepsy, Epilepsia., Vol. 48 Suppl 9, 3-5., 2007
   Organism:Department of Pediatrics, Dalhousie University and IWK Health Centre, Halifax, Nova Scotia, Canada Camfield@dalcaFAU - Camfield, Carol S
   Abstract:Children with epilepsy often grow into adults with significant social problems including decreased employment, marriage, social relationships, and independent living arrangements. These problems are noted in population-based longitudinal and cross-sectional studies from many countries. Learning disorder and mental handicap are the most consistent predictors of poor social outcome. Epilepsy variables, even remission, appear to have little effect. The influence of epilepsy on social outcome is greater than found in other childhood chronic disease control groups. More attention and research is needed to correct these unfortunate outcomes

7. CANHAO H, FONSECA JE, SANTOS MJet GOMES JA: [Protocol for clinical monitoring of juvenile idiopathic arthritis], Acta Reumatol.Port., Vol. 32(3), 277-281., 2007
   Organism:Servico de Reumatologia, Hospital de Santa Maria, Lisbon helenacanhao@netcaboptFAU - Canhao, Helena
   Abstract:The development of new and more efficacious therapeutic agents, though expensive and potentially toxic, helped to implement objective measures to quantify the improvement and to monitor the evolution of inflammatory rheumatic diseases. The aim of our protocol (PMAIJ) is to supply rheumatologists and paediatricians with a useful tool for follow-up of juvenile arthritis patients using validated instruments for the evaluation of activity, functional capacity and response to treatment. PMAIJ has 2 pages. The first page is filled only at the initial evaluation; the second page is filled at first and in all the appointments after that. The application of this protocol would contribute to the standardization of procedures in different Paediatric Rheumatology Centres and would help to obtain useful information on the clinical evolution of JIA patients followed in Portugal

8. CEPUCH Get WORDLICZEK J: [Pain versus activity and fatigue in adolescents hospitalized because of cancer and rheumatoid diseases], Folia Med.Cracov., Vol. 47(1-4), 3-20., 2006
   Organism:Zaklad Pielegniarstwa Klinicznego, Instytut Pielegniarstwa i Poloznictwa Wydzilu Ochrony Zdrowia, Collegium Medicum Uniwersytetu Jagiellonskiego gcepuch@pocztaonetplFAU - Cepuch, Grazyna
   Abstract:INTRODUCTION: Chronic disease in adolescence is followed by many negative effects of somatic and psychosocial nature. These effects can be observed especially in oncological and rheumatologic diseases. This is due not only to the character of the disease, its chronic course, but also aggressive treatment. The objective of this work was to evaluate relationship between pain experience and sleep, fatigue and physical, social and intellectual functioning of teenage patients. MATERIAL AND METHODS: 124 adolescents, 14 to 20 years old, hospitalized because of cancer and juvenile rheumatoid arthritis participated in the study. Level of experienced pain was measured with VAS--Visual Analog Scale and NRS--Numeric Rating Scale. Quality of sleep was assessed with Polish version of Melzacks Questionnaire. Fatigue and activity were assessed with a questionnaire of our own construction. RESULTS: Pain was a significant symptom accompanying rheumatologic and oncological disease, although the sources of pain experience were different. Significant percentage of participants suffered from sleep disruption and activity impairment. An important relationship between increase of pain intensity and sleep disruption in oncological patients was found. Significant relationships between pain intensity vs. fatigue and also pain intensity vs. functioning were identified. CONCLUSIONS: Pain, fatigue and sleep disruption account for important factors in rheumatologic and oncological diseases. They also cause decrease in physical, social and mental functioning of teenage patients. Results show that there is a significant relationship between outcomes of disease, its treatment and impact on functioning and developmental course of adolescents. Care delivered to those patients must be integrated and involve multidisciplinary factors

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

10. CHUNG L, KRISHNAN Eet CHAKRAVARTY EF: Hospitalizations and mortality in systemic sclerosis: results from the Nationwide Inpatient Sample, Rheumatology (Oxford)., Vol. 46(12), 1808-1813., 2007
    Organism:Division of Immunology and Rheumatology, Stanford University, Stanford, CA, USA shauwei@stanfordeduFAU - Chung, L
    Abstract:OBJECTIVE: To study the causes of hospitalizations and predictors of subsequent adverse outcomes for contemporary cohorts of patients with systemic sclerosis (SSc) in the USA. METHODS: The data source was the 2002 and 2003 Healthcare Cost and Utilization Project-Nationwide Inpatient Sample (HCUP-NIS) databases. We identified all discharges with an International Classification of Diseases-Clinical Modification (ICD9-CM) code of 710.1 (limited and diffuse SSc), then excluded those with concomitant diagnoses for lupus or rheumatoid arthritis. We calculated hospitalization rates, in-hospital mortality rates and mean length of stay (LOS). Multivariate logistic and linear regression models for in-hospital death and LOS were performed adjusting for sociodemographic and comorbidity covariates. RESULTS: The overall in-hospital mortality rate was 6.3% and the mean LOS was 6.6 days. Hospitalization rates were 4.5 times higher in women than in men, but in-hospital mortality was approximately 25% lower (P = 0.005). SSc was the most common principal diagnosis for all SSc hospitalizations, with the most common secondary diagnosis (24%) being pulmonary fibrosis. After SSc, respiratory failure was the second most common principal diagnosis in patients who died. Pulmonary fibrosis increased the odds of in-hospital death by 2.63 [95% confidence interval (CI) 1.98-3.49] fold and increased LOS by 7.25% (95% CI 0.90-13.60). CONCLUSIONS: Women with SSc had higher rates of hospitalization but lower in-hospital mortality than men. Pulmonary fibrosis was the major predictor of poor hospitalization outcomes in SSc patients in recent years, emphasizing the importance of continuing to develop more effective therapies for this fatal complication of the disease

11. CHURCH LD, COOK GPet MCDERMOTT MF: Primer: inflammasomes and interleukin 1beta in inflammatory disorders, Nat.Clin.Pract.Rheumatol., Vol. 4(1), 34-42., 2008
    Organism:Leeds Institute of Molecular Medicine, St James's University Hospital, Leeds, UKFAU - Church, Leigh D
    Abstract:Inflammasomes are large, multimeric protein complexes that link the sensing of microbial products and metabolic stress to the proteolytic processing of prointerleukin (pro-IL)-1beta to its active form. NALP1 and NALP2 are founding members of the Nod-like receptor family. Other Nod-like receptors, including NALP3 and NOD2, which are associated with inflammatory disorders, have also been described. The NALP1 and NALP3 inflammasomes are located in the cytoplasm and can, therefore, detect intracellular infection through recognition of microbial pathogen-associated molecular patterns. The inflammasome pathways cooperate with Toll-like receptor pathways to mediate a rapid and appropriate response to pathogens and genotoxic stress. Mutations in both pyrin and NALP3 components of inflammasomes are associated with innate-immune-mediated diseases (familial Mediterranean fever and the 'cryopyrinopathies'), and aberrant IL-1beta processing has been reported in several autoinflammatory conditions, including Muckle-Wells syndrome, chronic infantile neurologic, cutaneous and articular syndrome/neonatal onset multisystem inflammatory disease, and gout. The effectiveness of IL-1beta blockade in treating many of these conditions has transformed the understanding and management of these disorders and also highlighted the role of aberrant IL-1beta signaling in other conditions, such as adult-onset Still's disease and systemic juvenile idiopathic arthritis

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

13. DUCOS dL, TERRADA C, TRAN TH, CASSOUX N, LEHOANG P, BODAGHI Bet KODJIKIAN L: Maculopathy in uveitis of juvenile idiopathic arthritis: an optical coherence tomography study, Br.J.Ophthalmol., Vol. 92(1), 64-69., 2008
    Organism:Department of Ophthalmology, Pitie-Salpetriere Hospital, 47 bd de l'Hopital, Paris, FranceFAU - Ducos de Lahitte, G
    Abstract:AIM: The aim of this study was to examine the frequency and characteristics of macular lesions observed in juvenile idiopathic arthritis (JIA) uveitis, using optical coherence tomography (OCT). METHODS: In this cross-sectional study, 38 consecutive patients were recruited from a tertiary referral center in uveitis. All eyes with JIA uveitis underwent complete ophthalmic examination including OCT 3. Exclusion criterion was the inability to obtain OCT scans. Flare and visual acuity were also analysed by using linear regression. RESULTS: We analysed foveal thickness (FT) and central foveal thickness (CFT), using software mapping, to describe macular lesions in 61 eyes. Maculopathy was observed in 51 eyes (84%) compared with 12% in the literature (p<0.0001) and comprised four types: perifoveolar thickening in 45 eyes (74%), macular oedema in 29 eyes (48%), foveal detachment in 11 eyes (18%) and atrophic changes in six eyes (10%). Only four eyes did not demonstrate any lesion. CONCLUSIONS: Among children with JIA uveitis, macular involvement is frequent and characterised by perifoveolar thickening and serous retinal detachment. OCT is a non-invasive instrument. It can easily identify this maculopathy, which could impair visual function, and require therapeutic intensification

14. DUER A, OSTERGAARD M, HORSLEV-PETERSEN Ket VALLO J: Magnetic resonance imaging and bone scintigraphy in the differential diagnosis of unclassified arthritis, Ann.Rheum.Dis., Vol. 67(1), 48-51., 2008
    Organism:Department of Rheumatology, Copenhagen University Hospital at Hvidovre, Copenhagen, DenmarkFAU - Duer, A
    Abstract:OBJECTIVES: To investigate the value in clinical practice of hand magnetic resonance imaging (MRI) and whole body bone scintigraphy in the differential diagnosis of patients with unclassified arthritis. METHODS: 41 patients with arthritis (> or = 2 swollen joints, > 6 months' duration) which remained unclassified despite conventional clinical, biochemical and radiographic (hands and feet) examinations were studied. Patients who fulfilled the ACR criteria for rheumatoid arthritis (RA) or had radiographic bone erosions were excluded. Contrast enhanced MRI of the wrist and metacarpophalangeal joints of the most symptomatic hand and whole body bone scintigraphy were performed. Two rheumatologists agreed on the most likely diagnosis and the patients were treated accordingly. A final diagnosis was made by another specialist review 2 years later. RESULTS: Tentative diagnoses after MRI and bone scintigraphy were: RA (n = 13), osteoarthritis (n = 8), other inflammatory diseases (n = 11), arthralgias without inflammatory or degenerative origin (n = 9). Two years later 11 of 13 patients with an original tentative diagnosis of RA had fulfilled the ACR criteria while two were reclassified (one to psoriatic arthritis (RF negative + psoriasis); one to non-specific self-limiting arthritis). No patients classified as non-RA at baseline had fulfilled the ACR criteria after 2 years. The presence of MRI synovitis, MRI erosion and bone scintigraphic pattern compatible with RA showed 100% specificity for a diagnosis of RA at 2 year follow-up. CONCLUSIONS: In patients with arthritis unclassified despite conventional clinical, biochemical and radiographic examinations, MRI and scintigraphy allowed correct classification as RA or non-RA in 39 of 41 patients when fulfilment of ACR criteria 2 years later was considered the standard reference

15. ELLINGSEN T, MOLLER BK, HERLIN Tet STENGAARD-PEDERSEN K: Up-regulated CD26 density on synovial fluid monocytes from patients with juvenile idiopathic arthritis, Scand.J.Rheumatol., Vol. 37(1), 76-78., 2008
    Organism:RheumatologyFAU - Ellingsen, T
    Abstract:

16. GILLIAM BE, WOLFF AEet MOORE TL: Partial C4 deficiency in juvenile idiopathic arthritis patients, J.Clin.Rheumatol., Vol. 13(5), 256-260., 2007
    Organism:Division of Rheumatology, Saint Louis University School of Medicine, 1402 South Grand Boulevard, Saint Louis, MO 63104, USAFAU - Gilliam, Brooke E
    Abstract:OBJECTIVES: C4 is encoded by 2 distinct but closely linked loci within the major histocompatibility complex locus on human chromosome 6. C4A deficiencies have been associated with autoimmune disease and C4B with increased frequency of infection. C4 deficiencies have rarely been associated with juvenile idiopathic arthritis (JIA). Our aim was to investigate the prevalence of deficiencies in C4 allotypes in JIA patients. METHODS: We evaluated 61 patients [35 JIA patients, 15 systemic lupus erythematosus patients, 9 rheumatoid arthritis patients, and 2 mixed connective tissue disease (CTD) patients] for C4 deficiency. Genomic DNA was isolated from whole blood and subjected to polymerase chain reaction using sequence-specific primers for C4 allotypes. RESULTS: We found 5 JIA patients with C4 deficiencies. Two IgM rheumatoid factor-positive JIA polyarthritis patients had C4 deficiencies, one with complete C4A deficiency and another with partial C4A and complete C4B deficiency. Two oligoarthritis patients displayed partial C4B deficiencies, and complete C4B deficiency was revealed in 1 IgM rheumatoid factor-negative polyarthritis patient. Three patients had histories of recurrent infections and 2 demonstrated a more severe disease course. Disease controls showed 8 systemic lupus erythematosus patients had partial C4 deficiencies, whereas no deficiencies were revealed in the rheumatoid arthritis or mixed CTD patients. CONCLUSIONS: Defects in the complement system have been implicated in the pathogenesis of CTD. However, the specific role of C4 in JIA is not clear. We demonstrate partial C4 deficiencies in 5 JIA patients. Our findings suggest an association between C4 deficiency and another CTD, JIA, as well as with disease severity and recurrent infections

17. GRAINGER M, CASTLEDINE G, WOOD Net DILLEY C: Researching the management of constipation in long-term care. Part 2, Br.J.Nurs., Vol. 16(19), 1212-1217., 2007
    Organism:Institute of Ageing and Health, BirminghamFAU - Grainger, Michelle
    Abstract:The management of constipation is a problem in any healthcare setting. Constipation can affect all individuals; older people and those suffering from disabilities and long-term chronic conditions, such as Parkinson's disease and rheumatoid arthritis, are particularly vulnerable. This two-part article is based on a research study carried out in nine care homes, among patients of various ages with a variety of chronic conditions. The aim of the study was to investigate and improve bowel care in long-term care settings. The background to the study, including the definition, causes, risk assessment and management of constipation, were discussed in Part 1 (Castledine et al, 2007). Part 2 presents the main part of the study. Results show that appropriate education of staff improves their knowledge and practice in dealing with constipation. The importance of educating and training all members of the care team, especially healthcare assistants, in the management of bowel care is highlighted. An evidence-based approach using a constipation risk assessment, management of constipation flow chart and an interventions tool are identified as key factors in the ongoing care of patients in long-term settings

18. HAINES KA: Juvenile idiopathic arthritis: therapies in the 21st century, Bull.NYU.Hosp.Jt.Dis., Vol. 65(3), 205-211., 2007
    Organism:Department of Pediatrics, New York University, School of Medicine, New York, NY, USA khaines@humedcomFAU - Haines, Kathleen A
    Abstract:Juvenile idiopathic arthritis (JIA) is an umbrella term for seven or more clinical patterns of arthritis of unknown cause in children. Until the mid-1980s, therapy for children, with what was then called juvenile rheumatoid arthritis in the United States and juvenile chronic arthritis (JRA) elsewhere, consisted primarily of a small repertoire of antiinflammatory drugs and corticosteroids. However, only a small percentage of children respond to NSAIDs (nonsteroidal antiinflammatory drugs) alone; almost all will respond to corticosteroids, but with the cost of unacceptable toxicities. Juvenile arthritis was often a crippling disease. The controlled trial that demonstrated methotrexate therapy was safe and effective in children was the major advance of that decade. With the burgeoning understanding of the immune system and the advent of biologic agents in the 21st century, pediatric rheumatologists now have many more therapies to offer patients, with the expectation that their disease will be controlled. This review will discuss current therapy and the approach to treatment of JIA

19. HAMPTON T: Trials reveal promising options for treating juvenile rheumatoid arthritis, JAMA., Vol. 299(1), 27-28., 2008
    Organism:

20. HOLL-WIEDEN A, SUERBAUM Set GIRSCHICK HJ: Seronegative Lyme arthritis, Rheumatol.Int., Vol. 27(11), 1091-1093., 2007
    Organism:Children's hospital, Section of Pediatric Rheumatology, Immunology and Infectious diseases, University of Wuerzburg, Josef-Schneider-Str 2, 97090 Wuerzburg, GermanyFAU - Holl-Wieden, A
    Abstract:We present a 10-year-old girl who had been diagnosed with juvenile idiopathic arthritis 5 years before and who experienced a flare of arthritis affecting one knee while she was off medication for almost 3 years. Seronegative Lyme arthritis had to be diagnosed based on the detection of Borrelia burgdorferi DNA in synovial fluid. No humoral immune response to Borrelia burgdorferi was detectable before, at the time of diagnosis and up to 3 years later

21. HOLL-WIEDEN A, BEER M, MARX A, BONFIG R, TAPPE Det GIRSCHICK HJ: Infection of an urachal cyst during etanercept therapy in juvenile idiopathic arthritis, Rheumatol.Int., Vol. ., 2008
    Organism:Children's Hospital, Section of Pediatric Rheumatology, Immunology and Infectious Diseases, University of Wuerzburg, Josef-Schneider-Str 2, 97090, Wuerzburg, Germany, holl_a@kinderklinikuni-wuerzburgde
    Abstract:Etanercept, a tumor necrosis factor (TNF) receptor alpha antagonist is licensed for the treatment in patients affected by polyarticular juvenile idiopathic arthritis, who do not tolerate or had an inadequate response to methotrexate. Infections related to immunosuppression by etanercept are of major concern. We report on a 17-year-old boy with enthesitis-related arthritis who developed a major infection of an urachal cyst 18 months after initiation of etanercept therapy, which required surgery. The urachus had not been symptomatic before

22. HUFFMEIER U, BOIERS U, LASCORZ J, REIS Aet BURKHARDT H: Loss-of-function mutations in the filaggrin gene: no contribution to disease susceptibility, but to autoantibody formation against citrullinated peptides in early rheumatoid arthritis, Ann.Rheum.Dis., Vol. 67(1), 131-133., 2008
    Organism:Institute of Human Genetics, University Erlangen-Nuremberg, Erlangen, GermanyFAU - Huffmeier, U
    Abstract:OBJECTIVES: Autoantibody formation to citrullinated (pro)filaggrin has proven to be a highly specific serological marker for rheumatoid arthritis (RA). To test the potential relevance of mutations of the filaggrin (FLG) gene for disease susceptibility and elicitation of humoural autoimmunity in RA, a case-control association study of three loss-of-function FLG variants was performed. METHODS: DNA was obtained from 282 patients with early RA (mean disease duration: 6.5 months) and from 376 control individuals. Three loss-of-function variants of the FLG gene (*R501X, *2282del4 and *3702del1) were genotyped. RESULTS: No significant differences in genotype frequencies were observed between control probands and the population of RA patients. The FLG*3702del1 allele was not identified in any of the patients nor controls, and none of the probands was homozygous or compound heterozygous. In the RA cohort, heterozygous carriers of either of the FLG variants exhibited a significantly elevated prevalence of autoantibodies to citrullinated peptides (CCP-2) (80%) compared to non-carriers (51.9%) (p = 0.018, odds ratio: 3.71 (1.20-11.46)). CONCLUSIONS: The investigated FLG variants do not confer an overall risk for the development of RA. However, loss-of-function mutations in the FLG gene may contribute to the development of humoural autoimmunity, targeting citrullinated determinants in early RA

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

24. IWAMOTO V, DOS SANTOS SH, SKARE TLet SPELLING PF: Evaluation of psychological stress in primary caregivers of patients with juvenile idiopathic arthritis, J.Pediatr.(Rio J.)., Vol. 84(1)., 2008
    Organism:Hospital Universitario Evangelico de Curitiba, Curitiba, PR, Brazil
    Abstract:OBJECTIVE: To assess psychological stress in primary caregivers of juvenile idiopathic arthritis (JIA) pediatric patients. METHODS: Uncontrolled cross-sectional analytical study of 40 caregivers of JIA patients. Caregivers were evaluated using the Caregiver Burden Scale, which analyzes five domains of stress on a scale of 1 to 4: general strain, isolation, disappointment, emotional involvement and strained caused by environmental barriers. The data were subjected to statistical analysis. RESULTS: Caregivers of JIA patients were mainly female (87.5%), married (92.1%) and close relatives (90%). Stress levels were higher in caregivers of polyarticular JIA patients (p = 0.006), single caregivers (p = 0.019) and female caregivers (p = 0.017). Environment-related difficulties were reported as the most stressful category by caregivers. CONCLUSION: Caregivers of JIA patients are usually married female relatives. Caring for polyarticular JIA patients is more stressful than caring for oligoarticular JIA patients. Strain caused by environmental barriers accounts for the highest levels of stress among the caregivers included in this study

25. JOLLES BM, GROSSO Pet BOGOCH ER: Shoulder arthroplasty for patients with juvenile idiopathic arthritis, J.Arthroplasty., Vol. 22(6), 876-883., 2007
    Organism:Hopital Orthopedique de la Suisse Romande, Centre Hospitalier Universitaire Vaudois, University of Lausanne, Lausanne, SwitzerlandFAU - Jolles, Brigitte M
    Abstract:Between 1986 and 1997, 13 shoulders in adult patients who had severe polyarticular juvenile idiopathic arthritis were treated with primary arthroplasty. Eleven shoulders were evaluated retrospectively by an independent observer with a mean follow-up of 9 years. Patient evaluation included pain Visual Analogue Scale, range of motion, Disabilities of the Arm, Shoulder and Hand score, and Short-Form 36. Patients' pain decreased significantly after surgery (mean 6.7). Forward elevation improved on average by 41.1 degrees and external rotation by 39.1 degrees , without evidence of shoulder instability. Final Short-Form 36 scores and Disabilities of the Arm, Shoulder and Hand results (mean, 44.7) were poor, but all patients rated themselves satisfied with the procedure. Shoulder arthroplasty provided pain relief for end-stage shoulder involvement in adult juvenile idiopathic arthritis. Improvement in external rotation in this severely affected group appears to have a beneficial effect on functional outcome

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

27. KARANTANAS A, DAILIANA Zet MALIZOS K: The role of MR imaging in scaphoid disorders, Eur.Radiol., Vol. 17(11), 2860-2871., 2007
    Organism:Department of Radiology, Medical School, University of Crete, Heraklion, Greece apolsen@yahoocomFAU - Karantanas, Apostolos
    Abstract:The scaphoid bone of the wrist is one of the most commonly fractured bones in the body. Due to its importance in the biomechanics and functionality of the wrist, it is important to depict and characterize the type of injury. Plain radiographs and scintigraphy may fail to disclose the type and severity of the injury. In patients with normal initial plain radiographs, MR imaging can discriminate occult fractures from bone bruises and may also demonstrate ligamentous disruption. MR imaging can also discriminate the proximal pole viability versus avascular necrosis secondary to previous fracture, which is important for treatment planning. Treatment of non-united fractures with vascularized grafts can be evaluated with contrast-enhanced MR imaging. Idiopathic osteonecrosis or Preiser's disease was originally described after trauma. The non-traumatic disorders of the scaphoid include post-traumatic osteoarthritis, inflammatory bone marrow edema in patients with rheumatoid arthritis, and osteomyelitis. MR imaging is helpful in all the above disorders to demonstrate early bone marrow edema, cartilage degeneration and associated subchondral marrow changes. The most commonly found tumors in the scaphoid are usually benign and include enchondroma, osteoblastoma and osteoid osteoma. MR imaging is not mandatory for the initial diagnosis, which should be based on plain X-ray findings

28. KAUSHIK VV, AMBALAVANAN Set BINYMIN K: Comment on: Use of the QuantiFERON TB Gold test as part of a screening programme in patients with RA under consideration for treatment with anti-TNF-alpha agents: the Newcastle (UK) experience, Rheumatology (Oxford)., Vol. 46(12), 1863-1864., 2007
    Organism:

29. KIM HA, KIM SHet SEO YI: Ultrasonographic findings of the shoulder in patients with rheumatoid arthritis and comparison with physical examination, J.Korean Med.Sci., Vol. 22(4), 660-666., 2007
    Organism:Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Korea kimha@hallymackrFAU - Kim, Hyun Ah
    Abstract:The objectives of this study were: 1) to identify the ultrasonographic (US) abnormalities and 2) to compare the findings of physical examination with US findings in rheumatoid arthritis (RA) patients with shoulder pain. We studied 30 RA patients. Physical examination was performed systemically as follows: 1) area of tenderness; 2) range of passive and active shoulder motion; 3) impingement tests; 4) maneuvers for determining the location of the tendon lesions. US investigations included the biceps, the supraspinatus, infraspinatus, and subscapularis tendons; the subacromial-subdeltoid bursa; and the glenohumeral and acromioclavicular joints. Thirty RA patients with 35 painful and 25 non-painful shoulders were examined. The range of motion affected the most by shoulder pain was abduction. The most frequent US finding of shoulder joint was effusion in the long head of the biceps tendon. Among the rotator cuff tendons, subscapularis was the most frequently involved. Tendon tear was also common among non-painful shoulders. Physical examination used for the diagnosis of shoulder pain had low sensitivity and specificity for detecting abnormalities in the rheumatoid shoulder joint. In conclusion, US abnormalities showed frequent tendon tears in our RA patients. Physical examination had low sensitivity and specificity for detecting rotator cuff tear in the rheumatoid shoulder joint

30. KIMURA E, OGA Set PEREIRA RM: Comparative study of the pharmacokinetics of MTX in juvenile idiopathic arthritis patients receiving long-term MTX monotherapy or MTX plus chloroquine, J.Clin.Pharm.Ther., Vol. 32(6), 579-584., 2007
    Organism:Department of Pharmacy and Pharmacology, State University of Maringa, Parana, Brazil ekimura@uembrFAU - Kimura, E
    Abstract:OBJECTIVE: To compare the pharmacokinetics and report on the clinical effects of methotrexate (MTX) in patients with juvenile idiopathic arthritis (JIA), receiving long-term MTX or MTX plus chloroquine (CQ). METHODS: The pharmacokinetics of MTX, clinical characteristics (morning stiffness, joint tenderness and number of swollen joints) and biochemical markers (A-amyloid substance, C-reactive protein, erythrocyte sedimentation rate, fibrinogen and alpha-glycoprotein acid, alanine transaminase and aspartate transaminase) of the JIA patients were determined. Eight patients were treated with MTX (0.15 mg/kg) and another eight with MTX (0.15 mg/kg) plus CQ (4 mg/kg) for at least 6 months. RESULTS: All patients had polyarticular involvement and the clinical characteristics and biochemical markers were similar for the two groups. The pharmacokinetics of MTX were also similar with the Cmax and AUC values being 455.00 +/- 101.00 nm and 1469.92 +/- 299.77 nm/h for MTX group and 425.00 +/- 169.60 nm and 1560.73 +/- 615.49 nm/h for MTX plus CQ group, respectively. The respective creatinine clearance was 117.95 +/- 12.58 for MTX group and 99.17 +/- 22.65 mL/min for MTX plus CQ. CONCLUSION: The pharmacokinetics of MTX in JIA patients treated chronically with MTX are similar, with or without CQ co-treatment

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

32. MAKAY B, YILMAZ S, TURKYILMAZ Z, UNAL N, OREN Het UNSAL E: Etanercept for therapy-resistant macrophage activation syndrome, Pediatr.Blood Cancer., Vol. 50(2), 419-421., 2008
    Organism:Department of Pediatric Rheumatology, Dokuz Eylul University Faculty of Medicine, Balcova, Izmir, Turkey balahanbora@deuedutrFAU - Makay, Balahan
    Abstract:Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of childhood rheumatic diseases, especially systemic onset juvenile idiopathic arthritis (SoJIA). We report a 4-year-old girl with probable SoJIA who presented with MAS. She did not respond to pulse methyl prednisolone and Cyclosporine A (CsA). She also failed to respond to intravenous immunoglobulin (IVIG) therapy. Etanercept was started, based on the observation of increased serum levels of tumor necrosis factor-alpha (TNF-alpha) in patients with MAS. Her condition improved following etanercept, suggesting that etanercept might have a therapeutic role in resistant MAS

33. MANGER B, MICHELS H, NUSSLEIN HG, SCHNEIDER Met SIEPER J: [Revision of the recommendations of the Commission on Pharmacotherapy of the German society for Rheumatology. Therapy with tumour necrosis factor blockers for inflammatory rheumatic illnesses], Z.Rheumatol., Vol. 66(1), 72-75., 2007
    Organism:Medizinische Klinik Ill mit Poliklinik, Friedrich-Alexander-Universitat, Erlangen-Nurnberg bernhardmanger@med3imeduni-erlangendeFAU - Manger, B
    Abstract:Inflammatory rheumatic illnesses are associated with various types of pain as well as handicaps. The so called basic therapies are not sufficiently effective in many patients or are terminated due to side effects. This is where tumor necrosis factor blockers (TNF) can be used. In many cases, they lead to a substantial improvement of the symptoms, a reduction in disease related laboratory parameters, improvement in quality of life to stopping disease related damage when their effect is rapid. Common and severe side effects are few, although long-term data are still limited. The following contribution lists recommendations for the indications and symptomatology for the use of TNF blockers

34. MCDONALD K, TOMS AP, ARMON K, JOHNSON Ket MARSHALL TJ: Carpal-tarsal osteolysis with elbow involvement, Skeletal Radiol., Vol. 36(11), 1097-1101., 2007
    Organism:Department of Radiology, Norfolk and Norwich University Hospital, Colney Lane, Norwich, Norfolk NR4 7UY, UKFAU - McDonald, Kirsteen
    Abstract:Carpal-tarsal osteolysis is a rare condition that manifests as the progressive resorption of carpal and tarsal bones in young children. The diagnosis of this condition is often difficult and delayed as the initial clinical presentation is non-specific. Radiographic changes occur gradually, are often not seen at presentation and depend on recognising loss of bone in the ossification centres of the carpus and tarsus. MRI demonstrates morphological abnormalities in the cartilaginous, as well as the osseous components, of the developing carpal and tarsal bones and therefore may be helpful in predating the radiographic changes. Ultrasound appears to contribute little to the diagnosis and may be misleading. Exclusion of other conditions, particularly juvenile idiopathic arthritis, is important in making the diagnosis. MRI can be useful in excluding an inflammatory arthropathy, and suggesting the diagnosis of carpal-tarsal osteolysis

35. MERT A, KUMBASAR H, OZARAS R, ERTEN S, TASLI L, TABAK Fet OZTURK R: Erythema nodosum: an evaluation of 100 cases, Clin.Exp.Rheumatol., Vol. 25(4), 563-570., 2007
    Organism:Infectious Diseases and Clinical Microbiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey doktoralimert@yahoocomFAU - Mert, A
    Abstract:OBJECTIVES: In this study, we investigated the clinical features, etiology, and also predictive factors of secondary erythema nodosum (EN) in patients with EN. METHODS: A total of 100 patients (mean age: 37 years) diagnosed with EN between 1993 and 2004 in our clinic were included in the study prospectively. A skin biopsy was performed in 46 of the patients. Patients were considered to have secondary EN when an underlying condition was found, and to have primary EN when no such condition was found. For the diagnosis of the underlying diseases, the pertinent diagnostic criteria and/or diagnostic methods were used. Categorical and continuous variables were compared by using chi-square and Mann-Whitney U tests respectively. Multiple regression analysis was applied to the significantly different variables. RESULTS: The majority of the patients were female (female/male: 6/1) and nearly half (47%) of the cases had a determined etiology. The leading etiology was poststreptococcal (11%), followed in decreasing order by primary tuberculosis (10%), sarcoidosis (10%), Behcet's syndrome (BS) (6%), drugs (5%), inflammatory bowel diseases (IBD) (3%), and pregnancy (2%). Fifteen (15%) patients complained of cough; the diagnosis was primary tuberculosis in eight cases and sarcoidosis in seven. Four patients with arthritis were diagnosed as having BS (in 3) and Crohn's disease (in 1). All the patients were followed for a mean duration of 4.5 years. The nodosities relapsed annually in 62% (33/53) of idiopathic EN patients but in only one (BS) in the secondary EN group. The histology was consistent with EN in all biopsied patients. Our study revealed that fever, leukocytosis, elevated CRP level, accelerated ESR, presence of cough, sore throat, diarrhea, arthritis, and pulmonary pathology were predictors of secondary EN. Recurrence in EN significantly predicted primary EN. All of the patients had bed rest and the majority was given an anti-inflammatory agent (naproxen sodium). The outcomes were usually favorable within 7 days. The patients with an underlying disease were given the specific treatment. CONCLUSION: EN has been associated with numerous diseases. In order to reduce cost and duration of diagnosis, every centre should determine its own most frequent etiologic factors. Predictive variables for secondary EN should also be determined and an optimum management for such patients should be clarified. Our study revealed streptococcal pharyngitis, primary tuberculosis, sarcoidosis, IBD, and BS as the main etiologies of EN

36. MURNAGHAN LM, THURGUR CH, FORSTER BB, SAWATZKY BJ, HAWKINS Ret TREDWELL SJ: A clinicoradiologic study of the shoulder in Apert syndrome, J.Pediatr.Orthop., Vol. 27(7), 838-843., 2007
    Organism:Department of Orthopaedics, University of British Columbia, CanadaFAU - Murnaghan, Lucas M
    Abstract:To provide a comprehensive radiographic, clinical, and functional description of the shoulder in Apert syndrome. METHODS: A cohort of 9 Apert syndrome patients (ages, 9-27 years) followed at a tertiary care facility was included in this prospective study. Patients were clinically assessed with physical examination and completion of 2 validated functional assessment tools, the Shoulder Pain and Disability Index (SPADI) and American Academy of Orthopaedic Surgeons Pediatric Outcomes Data Collection Instrument (AAOS PODCI). Radiographs were obtained of both shoulders, and standardized-protocol magnetic resonance imaging was performed on the dominant shoulder of all participants. RESULTS: All patients had some degree of functional impairment attributable to their shoulder pathologic abnormality. Physical examination consistently revealed reduced forward flexion and abduction. Radiographic findings were similar to previous reports, with pervasive osseous dysplasia of the shoulder joint. Medial humeral head hypoplasia was seen in 8 of 9 patients and greater tuberosity overgrowth in 7 of 9 patients. Magnetic resonance imaging of the shoulder, not previously performed in a cohort of Apert patients, allowed better delineation of abnormalities seen radiographically such as a central glenoid cleft seen in 8 of 9 patients. It also revealed a new finding of inferior glenoid inclination (7/9 patients) that has not been described in the literature. Very few soft tissue or degenerative abnormalities were demonstrated. CONCLUSIONS: The findings of this study confirm that patients with Apert syndrome are functionally impaired by their shoulder pathologic abnormality, which may have a similar clinical impact as the more well-described hand and foot anomalies. The global functioning of patients with Apert syndrome is equivalent to patients with juvenile rheumatoid arthritis. The shoulder range of motion in Apert patients is decreased, most significantly in flexion and abduction. Radiographs confirmed previous imaging findings of glenohumeral dysplasia. The novel magnetic resonance imaging component demonstrated consistent inferior glenoid inclination, which may be a significant factor in their shoulder impairment. Magnetic resonance imaging revealed no significant soft tissue or degenerative abnormalities to account for their clinical disability. These findings have potential relevance in the surgical and clinical management of these patients. LEVEL OF EVIDENCE: Level IV

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

38. PESSLER F, PAESSLER ME, LAMBERT M, MORGAN DEet SHERRY DD: Polyarthritis in a child with Rosai-Dorfman disease, Clin.Exp.Rheumatol., Vol. 25(4), 645-648., 2007
    Organism:Division of Rheumatology, The Children's Hospital of Philadelphia, PA 19104, USA pessler@emailchopeduFAU - Pessler, F
    Abstract:A 5-year-old boy presented with fever, rash, lymphadenopathy and polyarthritis. Systemic onset juvenile idiopathic arthritis was initially considered in the differential diagnosis, but lymph node biopsy established the diagnosis of Rosai-Dorfman disease (RDD). The arthritis recurred twice. Both times it correlated with the severity of the other clinical and laboratory abnormalities of RDD and responded to treatment with dexamethasone and vinblastine. This report adds inflammatory arthritis to the extranodal manifestations of RDD in children and suggests that this disorder should be considered as a rare cause of fever with rash, lymphadenopathy and arthritis

39. PRUUNSILD C, UIBO K, LIIVAMAGI H, TARRASTE S, TALVIK Tet PELKONEN P: Prevalence and short-term outcome of juvenile idiopathic arthritis: a population-based study in Estonia, Clin.Exp.Rheumatol., Vol. 25(4), 649-653., 2007
    Organism:Department of Pediatrics, Tartu University Children's Clinic, Estonia ChrisPruunsild@kliikikumeeFAU - Pruunsild, C
    Abstract:OBJECTIVES: To study the point prevalence of juvenile idiopathic arthritis (JIA) in children in Estonia on December 31, 2000. To examine the short-term clinical outcome of the disease. METHOD: Identification of patients diagnosed with JIA between 1995-2000. Prospective follow-up of new cases diagnosed between 1998-2000 for two years. Retrospective analysis of the medical records of patients diagnosed between 1995-1997. The study was population-based. RESULT: One hundred and ninety-seven (197) patients fulfilled the study criteria. On December 31, 2000, the point prevalence of JIA was 83.7 (95% CI: 72.4; 95.8) per 100 000 children aged 0-15 years, 90.7 (95% CI: 74.1; 108.9) for girls and 77.1 (95% CI: 62.2; 93.5) for boys. Prevalence was the highest among 11-15 year-old girls (132; 95% CI: 100.7; 167.4) and the lowest in 0-3 year-old girls (9.6; 95% CI: 1.2; 26.7). For 44 patients (22.3%), the disease was inactive after 2 years since the onset of the disease. For 76 patients (38.6%). the disease was active or stable after 2 years.CONCLUSION: This is the first population-based study on the prevalence and outcome of JIA in Estonia in which the new ILAR criteria have been used. A longer follow-up of JIA patients is needed to have a better overview of the course of the disease. Good cooperation between family doctors and specialists is crucial for diagnosing JIA as early as possible

40. RAMSEIER LE, SCHOENIGER R, VIENNE Pet ESPINOSA N: Treatment of late recurring idiopathic clubfoot deformity in adults, Acta Orthop.Belg., Vol. 73(5), 641-647., 2007
    Organism:Balgrist University Hospital, Zurich, Switzerland leonhardramseier@balgristchFAU - Ramseier, Leonhard E
    Abstract:Late recurrence of idiopathic clubfoot deformity in adults after prior successful surgery in childhood remains a rarity and only case reports exist. No study has yet clarified the results of triple arthrodesis in such cases. Complete clinical and radiological review of 7 patients (7 feet) after a follow-up time of 43 months following triple arthrodesis was undertaken. The time interval between the last surgical intervention and the triple arthrodesis averaged 27 years. The American Orthopaedic Foot and Ankle Society (AOFAS) score was used as an outcome measure. Average age at time of review was 36 years (range 18-45). All patients were examined clinically and radiologically. The AOFAS-score improved from 43 points preoperatively to 61 points at follow-up (p = 0.004). If adjusted by excluding subtalar motion, the relative score improved by 19% (from 46% to 65%; p = 0.0043). Although not significantly altered (p = 0.1), pain scores remained fair (25 points) but were improved compared with the preoperative evaluation (13 points). Ankle motion was not changed. Although statistically not significant, there was an increase in degree of ankle arthritis in 67% of patients (one patient had ankle fusion) and mid- and forefoot degenerative changes in 57%. Hindfoot alignment remained fair after surgical intervention. Triple arthrodesis is a palliative means to correct recurrent deformity in patients with idiopathic clubfoot. Despite residual symptoms and degenerative changes at the ankle, 86% of all patients were satisfied with the postoperative result

41. RASKOVIC S, BOGIC M, PERIC-POPADIC Aet TOMIC-SPIRIC V: [Sjogren's syndrome], Srp.Arh.Celok.Lek., Vol. 125(1-2), 54-58., 1997
    Organism:Sjogren's syndrome is a chronic inflammatory disease of unknown aethiology. It is characterized by decreased secretion of salivary and lacrimal glands, which induces keratoconjunctivitis sicca and xerostomia. Sjogren's syndrome is a central autoimmune disease, and it has characteristics of both organ-specific and generalized autoimmune diseases. It can exist as a primary disease or is associated with other autoimmune diseases (most freyuently with systemic lupus erythematosus or rheumatoid arthritis) and is classified as a secondary Sjogren's syndrome. The aethiology is multifactorial, and it has not yet been completely explained. In the pathogenesis of the disease the important role have genetic predisposition, chronic oestrogen stimulation, end viral infections, especially of the herpes virus group (EBV, CMV, HHV6) and retroviruses. In the clinical picture xerostomia, xerophtalmia and non-erosive arthritis are the most common features, with the whole spectrum of extraglandular manifestations of respiratory, gastrointestinal, skin, and haematologic, neurologic and endocrinologic disturbances. Pathohistological findings of minor labial salivary gland lymphocyte infiltration is the most specific and the most sensitive diagnostic criterion of Sjogren's syndrome. The diagnosis of keratoconjunctivitis sicca is made by Schrimer's test, Rose bengal dye staining and by the "tear break up time". Differential diagnosis of Sjogren's syndrome includes an extremely large number of various pathologic states. The treatment of Sjogren's syndrome consists of symptomatic treatment of dry mucosas (artificial tears, etc.) and also of antiinflammatory drugs, glucocorticoids, immunosuppressive drugs. Plasmapheresis and intravenous administration of immunoglobulins are used for immunosuppression in these patients

42. RIISE OR, HANDELAND KS, CVANCAROVA M, WATHNE KO, NAKSTAD B, ABRAHAMSEN TG, KIRKHUS Eet FLATO B: Incidence and Characteristics of Arthritis in Norwegian Children: A Population-Based Study, Pediatrics., Vol. ., 2008
    Organism:aDepartments of Rheumatology
    Abstract:OBJECTIVE. The purpose of this work was to assess the annual incidence of arthritis in children and describe early disease and patient characteristics, microbiologic features, and immunogenetic factors in children with different subgroups of childhood arthritis. PATIENTS AND METHODS. A population-based multicenter study was performed in southeastern Norway between June 1, 2004, and May 31, 2005. The total population of children under 16 years of age was 255303. Physicians were asked to refer their patients with suspected arthritis to the local department of pediatrics or rheumatology. The children were assessed on the basis of clinical, radiologic, and laboratory examinations at inclusion and followed up at 6 weeks, 6 months, and thereafter as long as clinically indicated. A chart review was performed to identify patients with arthritis who had not been included prospectively. RESULTS. The total annual incidence of arthritis was 71 per 100000 children. Transient arthritis, juvenile idiopathic arthritis, postinfectious arthritis, and infectious arthritis were found in 43, 14, 9, and 5 of 100000 children, respectively. The incidence was higher in children under the age of 8 years than in older children (107 vs 34 per 100000). Arthritis occurred more frequently in boys than in girls before the age of 8 years but not thereafter. The median age of onset was lower in children with infectious arthritis than in those with other types of arthritis. Monarthritis was less frequent in patients with juvenile idiopathic arthritis than in the other subgroups (64% vs 83%-100%). Ten percent of the patients had poststreptococcal reactive arthritis, and only 1 had enteropathic arthritis. Autoantibodies and the presence of HLA-B27 were associated with juvenile idiopathic arthritis. CONCLUSIONS. The annual incidence of childhood arthritis was 71 per 100000 children. We found several factors that may help in differentiating between subgroups of arthritis

43. ROTH J, BECHTOLD S, BORTE G, DRESSLER F, GIRSCHICK Het BORTE M: [Diagnosis, prophylaxis and therapy of osteoporosis in juvenile idiopathic arthritis: consensus statement of the German Association for Pediatric Rheumatology], Z.Rheumatol., Vol. 66(5), 434-440., 2007
    Organism:Padiatrische Pneumologie und Immunologie, SPZ Rheumatologie, Charite Virchow Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany johannesroth@charitedeFAU - Roth, J
    Abstract:In all subgroups of juvenile idiopathic arthritis (JIA), a pathologic loss of bone or the lack of increase in bone mass has been described in a high percentage of cases, even with new therapeutic approaches. The decrease in bone mass is correlated with the duration of active disease and the number of affected joints (cytokines, inactivity). In several studies, muscle mass was the strongest predictor of bone mass. A standardized diagnostic approach to the musculoskeletal system including measures of prophylaxis and therapy therefore seems to be mandatory for all children with JIA who do not achieve rapid remission. In this review, the diagnostic and therapeutic options are described and summarized in an algorithm

44. ROTH J, BECHTOLD S, BORTE G, DRESSLER F, GIRSCHICK HJet BORTE M: Osteoporosis in juvenile idiopathic arthritis--a practical approach to diagnosis and therapy, Eur.J.Pediatr., Vol. 166(8), 775-784., 2007
    Organism:Department of Pediatric Pneumology and Immunology, Charite University Medicine, Berlin, Germany johannesroth@charitedeFAU - Roth, Johannes
    Abstract:In all subgroups of juvenile idiopathic arthritis, a decrease in bone mass has been described in a high percentage of children. Recently, new pathogenetic concepts have identified muscle mass as the strongest predictor of bone mass and bone is now recognized as part of the musculoskeletal system. In addition, the sophisticated use of bone densitometry in pediatrics, including new measurement techniques, has provided the tools for a reliable assessment. A standardized diagnostic approach to the musculoskeletal system, including prophylaxis and therapy, is, therefore, mandatory in all children with JIA who do not achieve rapid remission. In this review, diagnostic and therapeutic options are being described and possibilities to incorporate them into clinical practice are suggested

45. SCHEIBEL I, VEIT T, NEVES AG, SOUZA L, PREZZI S, MACHADO S, KOHEM C, ICARELLI M, XAVIER R, BRENOL JCet CHIES JA: Differential CCR5Delta32 allelic frequencies in juvenile idiopathic arthritis subtypes: evidence for different regulatory roles of CCR5 in rheumatological diseases, Scand.J.Rheumatol., Vol. 37(1), 13-17., 2008
    Organism:Division of Rheumatology, Clinical Hospital of Porto Alegre (HCPA)FAU - Scheibel, I
    Abstract:Background: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of childhood and is characterized by persistent arthritis for at least 6 weeks. Its aetiopathogenesis is unknown but there is strong evidence that there is a substantial genetic component. Chemokine receptors genes are among the candidate genes for association with arthritis and other inflammatory diseases. The CC chemokine receptor 5 (CCR5)Delta32 polymorphism has been associated with rheumatoid arthritis (RA), conferring a protective effect. Objective: To determine whether the CCR5Delta32 polymorphism is associated with JIA and RA in Brazilian patients. Methods: We investigated 203 RA patients, 101 JIA patients, and 104 healthy individuals by amplification of the CCR5Delta32 deletion. We compared the allelic frequencies among these groups, as well as among different JIA subtypes. Results: The frequency of the Delta32 allele was higher in JIA patients (9.4%) as compared to control subjects (3.8%) and RA patients (3.2%). Grouping the patients according to JIA subtypes, we observed a higher CCR5Delta32 allelic frequency in the subtypes with a greater inflammatory component: 4.1% in oligoarticular (n = 49), 11.2% in polyarticular (n = 40) [9.5% in rheumatoid factor negative (RF-) and 33.3% in RF positive (+)], and 25% in systemic JIA (n = 12). Conclusions: This study suggests that in JIA, unlike in RA, CCR5Delta32 does not have a protective effect, but instead it could be a factor associated with more inflammatory forms of the disease. These observations give rise to new questions about the mechanism and the cellular types involved in JIA as well as about the aetiology of JIA

46. SGHIRI R, BOUAGINA E, ZAGLAOUI H, MESTIRI H, HARZALLAH L, HARRABI I, GHANNOUCHI M, MOKHTAR Fet GHEDIRA I: Diagnostic performances of anti-cyclic citrullinated peptide antibodies in rheumatoid arthritis, Rheumatol.Int., Vol. 27(12), 1125-1130., 2007
    Organism:Laboratory of Immunology, Farhat Hached Hospital, Sousse, Tunisia Sghiririm@yahoocomFAU - Sghiri, Rim
    Abstract:To evaluate the rheumatoid arthritis (RA) diagnostic performances of anti-cyclic citrullinated peptide antibodies (anti-CCP). Anti-CCP was detected by an enzyme linked immunosorbent assay in 164 patients with RA and 343 controls. In addition, anti-CCP predictive value for radiological damage were investigated in 37 recent-onset RA patients followed up prospectively for 2 years. Radiological damages were assessed by Sharp method modified by van der Heijde. The sensitivity of anti-CCP was 78.7% and the specificity was 95.6%. The positive predictive value and the negative predictive value were 90.2% and 90.3%, respectively. Anti-CCP were detected in sera of 79.3% of patients with recent onset RA and 78.3% of patients with long disease duration. In univariate and multivariate analyses, anti-CCP were not predictive for radiological damage. Our study confirms the high diagnostic performances of anti-CCP in RA. They are very useful to aid the diagnostic of RA in clinical practice

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

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

49. SIJSSENS KM, RIJKERS GT, ROTHOVA A, STILMA JS, SCHELLEKENS PAet DE BOER JH: Cytokines, chemokines and soluble adhesion molecules in aqueous humor of children with uveitis, Exp.Eye Res., Vol. 85(4), 443-449., 2007
    Organism:FC Donders Institute of Ophthalmology, University Medical Center Utrecht, E03136, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands ksijssens@umcutrechtnlFAU - Sijssens, Karen M
    Abstract:Uveitis in childhood is a visual threatening disease with a complication rate of more than 75%. Despite extensive research, the etiology of uveitis is still unclear although the general opinion is now that uveitis is a T-cell mediated disease. The purpose of this study was to investigate the profile of cytokines, chemotactic cytokines (chemokines) and soluble adhesion molecules in the aqueous humor (AqH) of children with uveitis in order to identify the factors that control the immune response in the eye. In this clinical laboratory investigation we analyzed, with a multiplex immunoassay, 16 immune mediators in the AqH of 25 children with uveitis and 6 children without uveitis. Increased levels of interleukin-2 (IL-2), IL-6, IL-10, IL-13, IL-18, interferon-gamma, tumor necrosis factor-alpha, soluble intercellular adhesion molecule-1, RANTES, IL-8 and interferon-inducible 10-kDa protein were found in the AqH of children with uveitis compared with controls. No significant differences were found for IL-1 beta, IL-4, IL-12 p-70, soluble vascular cell adhesion molecule 1 and Eotaxin. Lower levels of IL-10 and IL-8 were found in quiet stage uveitis (surgical) samples compared with active uveitis (diagnostic) samples and in samples of patients treated with methotrexate (MTX) compared with samples of patients not treated with MTX. Lower levels of IL-10 were as well found in samples taken during the first 3 months after the diagnosis of uveitis than samples taken later during the disease process. No significant differences were found between patients treated with or without topical or systemic (perioperative and long term) corticosteroids. In conclusion, in children with uveitis, multiple intraocular cytokines, chemokines and soluble adhesion molecules are increased in the AqH regardless of active or inactive inflammation. Whether the IL-8 and IL-10 levels in AqH of children with uveitis are correlated with uveitis activity, early or late phase of the course of the disease and systemic treatment with MTX needs further investigation in a bigger study population

50. SOBRIN L, CHRISTEN Wet FOSTER CS: Mycophenolate Mofetil after Methotrexate Failure or Intolerance in the Treatment of Scleritis and Uveitis, Ophthalmology., Vol. ., 2008
    Organism:Massachusetts Eye Research and Surgery Institute, Cambridge, Massachusetts
    Abstract:PURPOSE: To evaluate the outcomes of treatment with mycophenolate mofetil in patients with scleritis and uveitis refractory to or intolerant of methotrexate. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Eighty-five patients with scleritis and/or uveitis who failed with or did not tolerate methotrexate and were subsequently treated with mycophenolate mofetil between 1998 and 2006. METHODS: We reviewed medical records of patients who were treated with mycophenolate mofetil after methotrexate intolerance or failure at one tertiary uveitis referral practice. We recorded dose and duration of methotrexate and mycophenolate mofetil therapy, inflammation grade, Snellen visual acuity (VA), use of other immunomodulatory therapy, and adverse events. Multivariate logistic regression was used to identify factors associated with inflammation control. MAIN OUTCOME MEASURES: Control of inflammation, steroid-sparing effect, VA, and adverse effects were assessed. RESULTS: Inflammation was controlled with mycophenolate mofetil in 47 patients (55%), with 5 achieving durable remission off all medication. In multivariate logistic regression analysis that adjusted for gender and age, the odds of inflammation control were lower for patients with scleritis (odds ratio [OR], 0.19; 95% confidence interval [CI], 0.04-0.93; P = 0.04) than for patients without scleritis. Among patients without scleritis, the odds of inflammation control were lower for patients with juvenile idiopathic arthritis (JIA)-associated uveitis (OR, 0.14; CI, 0.02-0.81, P = 0.03) compared to patients without JIA-associated uveitis. Eight of the 11 patients (73%) who were taking concomitant prednisone were able to taper their dose to <10 mg daily. Visual acuity declined in a greater percentage of patients who were unresponsive to mycophenolate mofetil (29%) compared with that of patients who responded to mycophenolate mofetil (9%). Side effects requiring discontinuation of mycophenolate mofetil occurred in 18 patients (21%). CONCLUSIONS: Mycophenolate mofetil was effective in controlling inflammation in approximately half of the patients who had previously failed with or did not tolerate methotrexate. The odds of inflammation control were less in patients with the diagnoses of scleritis and JIA

51. SPADARO A, RICCIERI V, SCRIVO R, ALESSANDRI Cet VALESINI G: Anti-cyclic citrullinated peptide antibody determination in synovial fluid of psoriatic arthritis, Clin.Exp.Rheumatol., Vol. 25(4), 599-604., 2007
    Organism:Dipartimento di Clinica e Terapia Medica - Rheumatology Unit, Divisione di Reumatologia, Universita di Roma La Sapienza, Azienda Policlinico Umberto I, Rome, Italy aspadaroreuma@ virgilioitFAU - Spadaro, A
    Abstract:OBJECTIVE: To assess the role of anti-CCP antibodies in synovial fluid (SF) of psoriatic arthritis (PsA) patients by analysing their association with different clinical patterns of the disease. METHODS: Seventy-five patients with a knee-joint effusion were studied, including 31 PsA patients, 29 rheumatoid arthritis (RA) and 15 osteoarthritis (OA) patients. SF and paired serum samples were stored at -70 degrees C until IgG anti-CCP and total IgG determination. The pattern of PsA articular involvement was defined as mono-, oligo-, polyarticular or axial. RESULTS: Lower levels of IgG anti-CCP antibodies in SF (p < 0.01) and serum (p < 0.005) were found in PsA respect to RA patients without difference with OA. We found a higher SF/serum ratio for anti-CCP compared to the SF/serum ratio for total IgG in PsA (p < 0.0005) as well as in RA and OA. The correction of anti-CCP concentration in SF as IgG anti-CCP (unit) / total IgG revealed lower (p < 0.002) values in PsA patients with respect to RA patients. In PsA group, values of anti-CCP antibodies, SF/serum ratio of anti-CCP and anti-CCP/IgG above the cut-off were found in 5, 6 and 2 SF samples respectively. The presence or absence of anti-CCP antibodies did not discriminate a particular clinical subset. CONCLUSIONS: In conclusion, strengthening the concept of local production of anti-CCP antibodies within the joint space, our results suggest that anti-CCP antibody detection in SF should take into account corrections such as total amount of corresponding immunoglobulin or SF/serum ratio. In our study, the presence or absence of anti-CCP antibodies did not discriminate a particular clinical subset, but further longitudinal studies are required to clarify the clinical role of anti-CCP in PsA

52. SPERLING JW, COFIELD RH, SCHLECK CDet HARMSEN WS: Total shoulder arthroplasty versus hemiarthroplasty for rheumatoid arthritis of the shoulder: results of 303 consecutive cases, J.Shoulder.Elbow.Surg., Vol. 16(6), 683-690., 2007
    Organism:Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN 55905, USA sperlingjohn@mayoeduFAU - Sperling, John W
    Abstract:Between January 1, 1976 and December 31, 1991, 195 total shoulder arthroplasties and 108 hemiarthroplasties were performed in 247 patients in patients with rheumatoid arthritis. One hundred and eighty-seven total shoulder arthroplasties and 95 hemiarthroplasties with complete preoperative evaluation, operative records, and minimum 2-year follow-up (mean, 11.6 years) or follow-up until revision were included in the clinical analysis. Twenty patients had died and 1 was lost to follow-up. All 303 shoulders were included in the survival analysis. There was significant long term pain relief (P < .0001), improvement in active abduction (P < .0001), and external rotation (P < .0001) with both hemiarthroplasty and total shoulder arthroplasty (TSA). There was not a significant difference in improvement in pain and motion comparing hemiarthroplasty and TSA for patients with a thin or torn rotator cuff. However, among patients with an intact rotator cuff, improvement in pain and abduction were significantly greater with TSA. Additionally, among patients with an intact rotator cuff, the risk for revision was significantly lower for TSA (P = .04). Radiographs were available for 152 total shoulder arthroplasties and 63 hemiarthroplasties with a minimum 2-year follow-up. Glenoid erosion was present in 62 of 63 hemiarthroplasties (98%). Glenoid periprosthetic lucency was present in 110 of 152 total shoulder arthroplasties (72%). The data from this study indicate that there is marked long-term pain relief and improvement in motion with shoulder arthroplasty. Among patients with an intact rotator cuff, TSA appears to be the preferred procedure for pain relief, improvement in abduction, and lower risk of revision surgery

53. SUZUKI M, ROSS GF, WIERS K, NELSON S, BENNETT M, PASSO MH, DEVARAJAN Pet BRUNNER HI: Identification of a urinary proteomic signature for lupus nephritis in children, Pediatr.Nephrol., Vol. 22(12), 2047-2057., 2007
    Organism:Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USAFAU - Suzuki, Michiko
    Abstract:The quest for reliable biomarkers of systemic lupus erythematosus (SLE) nephritis is an area of intense contemporary research. In this study, surface-enhanced laser desorption/ionization time-of-flight mass spectrometry (SELDI-TOF-MS) technology was used for urinary proteomic profiling of patients with SLE nephritis. Clinical, laboratory, and kidney biopsy data from pediatric patients with SLE (n = 32) were analyzed. Children with juvenile idiopathic arthritis (n = 11) served as controls. SELDI-TOF-MS was performed using ProteinChips with different chromatographic surfaces. The resulting spectra were analyzed with Bio-Rad Biomarker Wizard software. A consistent urinary proteomic signature for SLE nephritis was found, comprising eight biomarker proteins with peaks at m/z of 2.7, 22, 23, 44, 56, 79, 100, and 133 kDa. The peak intensities of these biomarkers were significantly greater in patients with SLE nephritis compared with controls and SLE patients without nephritis. These biomarkers were strongly correlated with renal disease activity and moderately with renal damage. For the diagnosis of active nephritis, the area under the receiver operating characteristic curve was > or =0.90 for 22, 23, 44, 79, and 100 kDa biomarkers. Thus, SELDI-TOF-MS has identified a urine proteomic signature strongly associated with SLE renal involvement and active SLE nephritis

54. SUZUKI M, WIERS KM, KLEIN-GITELMAN MS, HAINES KA, OLSON J, ONEL KB, O'NEIL K, PASSO MH, SINGER NG, TUCKER L, YING J, DEVARAJAN Pet BRUNNER HI: Neutrophil gelatinase-associated lipocalin as a biomarker of disease activity in pediatric lupus nephritis, Pediatr.Nephrol., Vol. 23(3), 403-412., 2008
    Organism:Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
    Abstract:We hypothesized that neutrophil gelatinase-associated lipocalin (NGAL) is an early predictive biomarker of disease activity in lupus nephritis. NGAL in serial plasma (PNGAL) and urine (UNGAL) samples was measured by enzyme-linked immunosorbent assay (ELISA) in 85 participants with pediatric systemic lupus erythematosus (pSLE), healthy children (n = 50), and children with juvenile idiopathic arthritis (JIA) (n = 30). Disease activity was measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Plasma and urinary NGAL were significantly increased in subjects with pSLE compared with those with JIA or with healthy controls (all p < 0.03), and unrelated to subjects' age, weight, or height. Plasma and urinary NGAL were stable in pSLE subjects with unchanged disease activity. The pSLE subjects with worsening global or renal disease activity had a mean +/- standard error (SE) increase of UNGAL (in ng/ml) of 11.5 +/- 6.4 or 36.6 +/- 12.1 (p < 0.01), corresponding to a 156% or 380% increase, respectively. PNGAL increased with worsening disease but to a much lesser degree than UNGAL [global disease activity (mean +/- SE): 7.3 +/- 6.2 or 21%; renal disease activity: 20.2 +/- 6.0 or 51%; both p = not significant]. In conclusion, NGAL in urine but not in plasma represents a novel biomarker for renal disease activity in pSLE

55. TAKAHASHI H, SHIGEHARA K, YAMAMOTO M, SUZUKI C, NAISHIRO Y, TAMURA Y, HIROHASHI Y, SATOH N, SHIJUBO N, SHINOMURA Yet IMAI K: Interferon gamma assay for detecting latent tuberculosis infection in rheumatoid arthritis patients during infliximab administration, Rheumatol.Int., Vol. 27(12), 1143-1148., 2007
    Organism:First Department of Internal Medicine, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-ku, Sapporo 060-8543, Japan htakahas@sapmedacjpFAU - Takahashi, Hiroki
    Abstract:In rheumatoid arthritis (RA) patients treated with infliximab (IFX), QuantiFERON-TB Gold (QFT-G), an interferon gamma assay for diagnosing tuberculosis infection, was performed to compare its effectiveness to conventional diagnostic procedures (tuberculin skin test, imaging and medical history) in diagnosing latent tuberculosis infection (LTBI). QFT-G was measured bimonthly in 14 rheumatoid arthritis patients during IFX treatment. Seven of 14 patients were confirmed as LTBI positive by at least one method. Of these, four were positive on QFT-G during the study period, and two were positive before the start of IFX administration. For two of the four QFT-G-positive patients, LTBI was diagnosed only by QFT-G. The rate of agreement between QFT-G and conventional procedures was 64.3%. A total of 5% of QFT-G tests were impossible to judge due to decreased reactions in the positive control. These results suggest that QFT-G is able to detect LTBI in RA patients overlooked by conventional methods. Conventional procedures and QFT-G should be employed in parallel, and LTBI should be assumed when one technique gives a positive result

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

57. TROULIS MJ, TAYEBATY FT, PAPADAKI M, WILLIAMS WBet KABAN LB: Condylectomy and costochondral graft reconstruction for treatment of active idiopathic condylar resorption, J.Oral Maxillofac.Surg., Vol. 66(1), 65-72., 2008
    Organism:Department of Oral and Maxillofacial Surgery, Massachusetts General Hospital, Harvard School of Dental Medicine, Boston, MA 02114, USAFAU - Troulis, Maria J
    Abstract:PURPOSE: The purpose of this study was to evaluate the outcomes in patients with active bilateral idiopathic condylar resorption treated by condylectomy and costochondral graft (CCG) reconstruction. PATIENTS AND METHODS: This was a retrospective evaluation of 15 consecutive patients treated at Massachusetts General Hospital from 1999 to 2004 who had: 1) active bilateral idiopathic condylar resorption confirmed by clinical examination, plain radiographs, and technetium-99 bone scan; 2) adequate documentation; and 3) a minimum of 12 months follow-up. Patients with an identifiable cause of condylar resorption such as rheumatoid or degenerative arthritis, trauma, or steroid use, or who had less than 12 months follow-up were excluded. Preoperative, immediate postoperative, 6-month, 12-month, and latest follow-up clinical examinations, lateral cephalograms, and panoramic radiographs were used to evaluate the outcomes. Inferior alveolar and marginal mandibular nerve function, jaw motion, and occlusion were evaluated by history and physical examination. All patients underwent bilateral condylectomy and CCG reconstruction. RESULTS: There were 13 female and 2 male patients with a mean age of 24 years. Mean follow-up was 34 months (range, 12 to 84). Preoperatively, all patients had Class II malocclusion: mean overjet 6.2 mm and mean anterior open bite -2.65 mm. At latest postoperative follow-up, all patients showed Class I occlusion with no anterior open bite, a stable and reproducible occlusion, and a normal range of TMJ motion with a mean maximal incisal opening of 39 mm. CONCLUSION: The results of this study indicate that a stable and satisfactory outcome is achievable in patients with active idiopathic condylar resorption treated by condylectomy and CCG reconstruction

58. UZIEL Y, CHAPNICK G, ROTHSCHILD M, TAUBER T, PRESS J, HAREL Let HASHKES PJ: Nitrous Oxide sedation for intra-articular injection in juvenile idiopathic arthritis, Pediatr.Rheumatol.Online.J., Vol. 6(1), 1, 2008
    Organism:ABSTRACT: BACKGROUND: Intra-articular corticosteroid injection in juvenile idiopathic arthritis (JIA) is often associated with anxiety and pain. Recent reports advocate the use of nitrous oxide (NO), a volatile gas with analgesic, anxiolytic and sedative properties. OBJECTIVE: To prospectively evaluate the effectiveness and safety of NO analgesia for intra-articular corticosteroid injection in JIA, and to assess patients and staff satisfaction with the treatment. METHODS: NO was administered to JIA patients scheduled for joint injection. The patient, parent, physician and nurse completed visual-analog scores (VAS) (0-10, 10 is worst) for pain, and a 5-point Likert satisfaction scale (5 is best). Change in heart rate (HR) during the procedure was recorded in order to examine physiologic response to pain and stress. Patient's behavior and adverse reactions were recorded. RESULTS: 54 procedures (72 joints) were performed, 41 females, 13 males; 39 Jewish, 13 Arab; mean age was 12.2 +/- 4.7 year. The median VAS pain score for patients, parents, physicians and nurses was 3. The HR increased [greater than or equal to]15% in 10 patients. They had higher VAS scores as evaluated by the staff. The median satisfaction level of the parents and staff was 3.0 and 5.0 respectively. Adverse reactions were mild. CONCLUSIONS: NO provides effective and safe sedation for JIA children undergoing intra-articular injections

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

60. VAN DER NJ, VAN DER TP, ENGELBERT RH, ENGELEN V, VAN ZON F, TAKKEN Tet HELDERS PJ: Motor performance and functional ability in preschool- and early school-aged children with Juvenile Idiopathic Arthritis: a cross-sectional study, Pediatr.Rheumatol.Online.J., Vol. 6(1), 2, 2008
    Organism:ABSTRACT: OBJECTIVE: To describe the level of motor performance and functional skills in young children with JIA. METHODS: In a cross-sectional study in 56 preschool-aged (PSA) and early school- aged children (ESA) with JIA according to ILAR classification, motor performance was measured with the Bayley Scales of Infant Development II (BSID2) and the Movement Assessment Battery for Children (M-ABC). Functional skills were measured with the Pediatric Evaluation of Disability Inventory (PEDI). Disease outcome was measured with a joint count on swelling/range of joint motion, functional ability and joint pain. RESULTS: Twenty two PSA children (mean age 2.1 years) with a mean Developmental Index of the BSID2 of 77.9 indicating a delayed motor performance; 45% of PSA children showed a severe delayed motor performance. Mean PEDI scores were normal, 38% of PSA scored below -2 SD in one or more domains of the PEDI. Thirty four ESA children (mean age 5.2 years) with a mean M-ABC 42.7, indicating a normal motor performance, 12% of ESA children had an abnormal score. Mean PEDI scores showed impaired mobility skills, 70% of ESA children scored below -2 SD in one or more domains of the PEDI. Disease outcome in both age groups demonstrated low to moderate scores. Significant correlations were found between age at disease onset, disease duration and BSID2 or M-ABC and between disease outcome and PEDI in both age cohorts. CONCLUSION: More PSA children have more impaired motor performance than impaired functional skills, while ESA children have more impairment in functional skills. Disease onset and disease duration are correlated with motor performance in both groups. Impaired motor performance and delayed functional skills is primarily found in children with a polyarticular disease course. Clinical follow up and rehabilitation programs should also focus on motor performance and functional skills development in young children with JIA

61. VAN HOLSBEECK MT: A role for US screening in juvenile idiopathic arthritis, Pediatr.Radiol., Vol. 37(7), 623-624., 2007
    Organism:Department of Radiology, Musculoskeletal Radiology, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202, USA vanholsbeeck@comcastnetFAU - van Holsbeeck, Marnix T
    Abstract:

62. VERGUNST CE, GERLAG DM, DINANT H, SCHULZ L, VINKENOOG M, SMEETS TJ, SANDERS ME, REEDQUIST KAet TAK PP: Blocking the receptor for C5a in patients with rheumatoid arthritis does not reduce synovial inflammation, Rheumatology (Oxford)., Vol. 46(12), 1773-1778., 2007
    Organism:Division of Clinical Immunology and Rheumatology, Academic Medical Centre/University of Amsterdam, Meibergdreef 9, NL-1105 AZ Amsterdam, The NetherlandsFAU - Vergunst, C E
    Abstract:OBJECTIVES: All complement pathways lead to the formation of C5a, which is believed to contribute to the influx and activation of C5a-receptor (C5aR) bearing cells into the joints of patients with rheumatoid arthritis (RA). Studies in animal models of RA have suggested therapeutic potential of C5aR blockade. In this study, we examined the effects of the C5aR blockade on synovial inflammation in RA patients. METHODS: We performed a double-blind, placebo-controlled study using an orally administered C5aR-antagonist. Twenty-one patients with active RA were randomized 2:1 to treatment with a C5aR-antagonist AcF- (OpdChaWR) (PMX53) vs placebo for 28 days. Serum concentrations of PMX53 were determined. Synovial tissue was obtained at baseline and after 28 days of treatment for pharmacodynamic analysis using immunohistochemistry and digital image analysis. RESULTS: All patients completed the study. Areas under the curve (AUCs) of PMX53 in patients' blood samples showed a mean of 40.8 nmol h/l. There was neither decrease in cell infiltration, nor changes in key biomarkers associated with clinical efficacy after active treatment. In addition, there was no trend towards clinical improvement in the C5aR-antagonist-treated group compared with placebo nor was there a correlation between the AUC and clinical response. CONCLUSIONS: Treatment with PMX53 did not result in a reduction of synovial inflammation despite reaching serum levels of PMX53 that block C5aR-mediated cell activation in vitro. The data suggest that C5aR blockade does not result in reduced synovial inflammation in RA patients

63. VIJAYANATHAN V, SMITH AK, ZEBALA JA, KAMEN BAet COLE PD: High-performance liquid chromatography separation of aminopterin-polyglutamates within red blood cells of children treated for acute lymphoblastic leukemia, Transl.Res., Vol. 150(6), 367-373., 2007
    Organism:Pediatric Hematology/Oncology, The Cancer Institute of New Jersey, Robert Wood Johnson Medical School/UMDNJ, New Brunswick, NJ, USAFAU - Vijayanathan, Veena
    Abstract:Aminopterin (AMT), like the related compound methotrexate (MTX), is a drug with anticancer and antiinflammatory efficacy that works by interfering with synthetic reactions dependent on the vitamin folic acid. Red blood cell (RBC) precursors will accumulate antifolates like AMT and MTX through the same mechanism by which they take up folate. Intracellular folate and antifolates are then metabolized to polyglutamates that remain within the mature RBCs. RBC MTX has been correlated with toxicity and/or treatment efficacy among patients with acute lymphoblastic leukemia (ALL) or rheumatoid arthritis. Because AMT may offer clinically relevant advantages over MTX, we are testing whether it can be administered safely in multiagent therapy to children with ALL. Total RBC AMT was measured to monitor compliance with this oral, outpatient regimen, and to estimate AMT exposure to the bone marrow. Here we describe methods for quantifying each AMT-polyglutamate species within the RBCs of patients. The assay was linear over a concentration range of 62.5-500 nmol/L. Recovery of individual AMT-polyglutamates ranged from 85% to 92%, and the intraday coefficients of variation were 1.3% to 3.6%. Long-chain AMT-polyglutamates (triglutamate and tetraglutamate forms) accounted for over 40% of intracellular AMT within the RBCs of patients. Patients with long-chain AMT polyglutamate concentrations above the median tended to have lower mean neutrophil counts during weekly AMT therapy, which suggests that RBC AMT polyglutamate accumulation may correlate with hematologic toxicity. As AMT continues to be tested in clinical trials, the methods described here will be useful to define relationships between clinical response to AMT and RBC accumulation of AMT-polyglutamates

64. YOKOTA S, MORI M, IMAGAWA T, TAKEI S, MURATA T, TOMIITA M, ITO Yet FUJIKAWA S: Proposal for juvenile idiopathic arthritis guidance on diagnosis and treatment for primary care pediatricians and nonpediatric rheumatologists (2007), Mod.Rheumatol., Vol. 17(5), 353-363., 2007
    Organism:Department of Pediatrics, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan syokota@medyokohama-cuacjpFAU - Yokota, Shumpei
    Abstract:The Pediatric Standing Committee of the Japan College of Rheumatology, in collaboration with the Pediatric Rheumatology Association of Japan, produced guidance on the diagnosis and treatment for juvenile idiopathic arthritis (JIA) for primary care pediatricians and nonpediatric rheumatologists in Japan. This guidance aims to achieve early diagnosis and treatment for JIA, which is similar to adult rheumatoid arthritis (RA), based on recent progress in rheumatology, and to resolve arthritis at an early stage and improve the prognosis of the affected inflammatory joints. It describes clinical symptoms and laboratory findings characteristic to JIA in order to make early diagnosis and treatment possible, and also serves as a triage of patients who are refractory to the treatment protocol described here and need more aggressive interventions. However, because JIA is a complicated and heterogeneous disease and the optimal treatment approach can be diverse and different patient by patient, these guidelines should be viewed as recommendations and be individualized according to the condition of the patient. Finally, we hope that this guidance will trigger exploration for further information by referring to the textbooks and literature listed at the end of these guidelines