Bibliography May 04

1. AREND WP, GABAY C: Cytokines in the rheumatic diseases. Rheumatic Disease Clinics of North America 30:1 41-67, 2004

2. DE KLEER IM, WEDDERBURN LR, TAAMS LS, PATEL A, VARSANI H, KLEIN M, DE JAGER W, PUGAYUNG G, GIANNONI F, RIJKERS G, ALBANI S, KUIS W, PRAKKEN B: CD4+CD25(bright) regulatory T cells actively regulate inflammation in the joints of patients with the remitting form of juvenile idiopathic arthritis. J Immunol 172:10 6435-6443, 2004
   Organism: University Medical Center Utrecht, Wilhelmina Children's Hospital, Department of Pediatric Immunology and Immunology Advanced Center on Preclinical Immuno-genomics Institute for Translational Medicine, Utrecht, The NetherlandsFAU - de Kleer, Isme M
   Abstract: This study investigates the role of CD4(+)CD25(+) regulatory T cells during the clinical course of juvenile idiopathic arthritis (JIA). Persistent oligoarticular JIA (pers-OA JIA) is a subtype of JIA with a relatively benign, self-remitting course while extended oligoarticular JIA (ext-OA JIA) is a subtype with a much less favorable prognosis. Our data show that patients with pers-OA JIA display a significantly higher frequency of CD4(+)CD25(bright) T cells with concomitant higher levels of mRNA FoxP3 in the peripheral blood than ext-OA JIA patients. Furthermore, while numbers of synovial fluid (SF) CD4(+)CD25(bright) T cells were equal in both patient groups, pers-OA JIA patients displayed a higher frequency of CD4(+)CD25(int) T cells and therefore of CD4(+)CD25(total) in the SF than ext-OA JIA patients. Analysis of FoxP3 mRNA levels revealed a high expression in SF CD4(+)CD25(bright) T cells of both patient groups and also significant expression of FoxP3 mRNA in the CD4(+)CD25(int) T cell population. The CD4(+)CD25(bright) cells of both patient groups and the CD4(+)CD25(int) cells of pers-OA JIA patients were able to suppress responses of CD25(neg) cells in vitro. A markedly higher expression of CTLA-4, glucocorticoid-induced TNFR, and HLA-DR on SF CD4(+)CD25(bright) T regulatory (Treg) cells compared with their peripheral counterparts suggests that the CD4(+)CD25(+) Treg cells may undergo maturation in the joint. In correlation with this mature phenotype, the SF CD4(+)CD25(bright) T cells showed an increased regulatory capacity in vitro compared with peripheral blood CD4(+)CD25(bright) T cells. These data suggest that CD4(+)CD25(bright) Treg cells play a role in determining the patient's fate toward either a favorable or unfavorable clinical course of disease

3. DONN R, ALOURFI Z, ZEGGINI E, LAMB R, JURY F, LUNT M, MEAZZA C, DE BENEDETTI F, THOMSON W, RAY D: A functional promoter haplotype of macrophage migration inhibitory factor is linked and associated with juvenile idiopathic arthritis. Arthritis Rheum 50:5 1604-1610, 2004
   Organism: Arthritis Research Campaign/Epidemiology Unit, University of Manchester, Stopford Building, Oxford Road, Manchester M13 9PT, UK Rachelle@fs1sermanacukFAU - Donn, Rachelle
   Abstract: OBJECTIVE: To establish linkage and replicate the association of macrophage migration inhibitory factor (MIF) with juvenile idiopathic arthritis (JIA). METHODS: Three hundred twenty-one Caucasian simplex families from the UK were genotyped for polymorphisms of MIF using SNaPshot ddNTP primer extension, or by a fluorescently labeled primer method, and capillary gel electrophoresis. The functional significance of the promoter polymorphisms was studied using luciferase-based reporter gene assays in human T lymphoblast and epithelial cell lines. RESULTS: MIF was linked and associated with JIA (P = 0.0016). Specifically, a 2-point promoter haplotype, CATT(7)-MIF-173*C, was found to be transmitted in excess (38 transmitted: 21 not transmitted) in the JIA patients. Conditional extended transmission disequilibrium test and pairwise extended transmission disequilibrium test predicted functional interaction between the 2 polymorphic positions. The interaction of the CATT repeat with MIF-173*G/C was found to be specific to the cell type. CONCLUSION: Replication of an association and linkage of MIF with JIA has been established. Functional interaction between the polymorphic positions on the linked haplotype has also been shown. The molecular mechanism of this interaction is currently being investigated

4. GARAVITO G, YUNIS EJ, EGEA E, RAMIREZ LA, MALAGON C, IGLESIAS A, DE LA CRUZ OF, URIBE O, NAVARRO E, MARTINEZ P, JARAQUEMADA D: HLA-DRB1 alleles and HLA-DRB1 shared epitopes are markers for juvenile rheumatoid arthritis subgroups in Colombian mestizos. Human Immunology (United States ) 65:4 359-365, 2004
   Abstract: We studied the association of human leukocyte antigen (HLA)-DRB1 and HLA-DQB1 alleles and HLA haplotypes with juvenile rheumatoid arthritis (JRA) in 65 patients and 65 controls from Colombia. The JRA subsets were distinguished on the basis of criteria established by the American College of Rheumatology. Two alleles were associated with protection, HLA-DRB1*1501 (p = 0.002) and HLA-DRB1*1402 (p = 0.01). HLA-DRB1*1602 (p = 0.0000002) was associated with susceptibility for systemic JRA and HLA-DRB1*1104 (p = 0.0002) for pauciarticular JRA. Amino acid sequences at residues 70-74 of DRB1 chain shared by HLA-DRB1 alleles (shared epitomes) were also informative. The polyarticular JRA subset revealed association with SUP70QRRAA SUP74, which includes HLA-DRB1*04, 01, and SUP70DRRAA SUP74, which includes DRB1*1601, 1602, 1101, and 1104. Two new findings of interest were the association of the haplotypes DRB1*1104, DQB1*0301(p = 0.0002) with pauciarticular JRA and DRB1*1602, DQB1*0301 (p = 0.0000002) association with systemic JRA. The DRB1 alleles of these two haplotypes share the epitope SUP70DRRAASUP74and were associated with both the pauciarticular and the systemic subset of JRA. Our results suggest that studies of disease susceptibility in populations of admixed genetic background should take into account the contribution of different ethnic groups or nationalities in the recruitment of controls and patients studied in order to rule out genetic stratification. (c) American Society for Histocompatibility and Immunogenetics, 2004. Published by Elsevier Inc

5. HARTMAN C, SHAMIR R, ESHACH-ADIV O, IOSILEVSKY G, BRIK R: Assessment of Osteoporosis by Quantitative Ultrasound versus Dual Energy X-Ray Absorptiometry in Children with Chronic Rheumatic Diseases. Journal of Rheumatology (Canada ) 31:5 981-985, 2004
   Abstract: Objective. To evaluate the validity of quantitative ultrasound bone sonometry QUBS) as a screening tool for the diagnosis of osteoporosis in children with chronic rheumatic diseases (CRD), compared to the conventional dual energy x-ray absorptiometry (DEXA). Methods. Forty children with CRD [32 with juvenile idiopathic arthritis (JIA), 6 with systemic lupus erythematosus, and 2 with dermatomyositis] aged 9.9 +/- 4.3 years, were evaluated by QUBS of radius and tibia and DEXA of the lumbar spine. Twenty-five (62.5%) patients were treated with corticosteroids. Measurements of the velocity of the ultrasound wave, expressed as speed of sound (SOS) in m/s, and the results of the bone mineral density (BMD) assessed by DEXA were compared to reference data from healthy age and sex matched Israeli children. Results. Compared to controls, patients with CRD had significantly lower values by QUBS and DEXA alike. BMD and SOS z scores < -1 SD were found in 45% and 38% of the patients, respectively. Reduced BMD and SOS values correlated with age at disease onset and corticosteroid treatment. BMD alone correlated negatively with disease duration and methotrexate therapy. BMD was significantly lower in patients with polyarticular JIA compared to patients with oligoarticular disease (p < 0.03). SOS values did not differ between subtypes of JIA. A significant positive correlation was found between the lumbar DEXA and radius SOS. Conclusion. QUBS evaluation of radius and tibia yielded results comparable to DEXA and may therefore be used for screening patients with CRD for osteoporosis. QUBS might represent a promising means of evaluating bone quality in at-risk children

6. HUANG CM, TSAI CH, TSAI JJ, KUNG PT, CHEN CL, TSAI FJ: The relationship between insulin-like growth factor-II gene Apa I polymorphism and rheumatoid arthritis. Scand J Rheumatol 33:2 126-127, 2004
   Organism: Division of Immunology and Rheumatology, Department of Internal Medicine, China Medical University Hospital, Taichung, TaiwanFAU - Huang, C M

7. KUULIALA A, TAKALA A, SIITONEN S, LEIRISALO-REPO M, REPO H: Cellular and humoral markers of systemic inflammation in acute reactive arthritis and early rheumatoid arthritis. Scand J Rheumatol 33:1 13-18, 2004
   Organism: Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki, Finland anttikuuliala@helsinkifiFAU - Kuuliala, A
   Abstract: OBJECTIVE: To compare systemic inflammation in reactive arthritis (ReA), rheumatoid arthritis (RA), and sepsis using novel markers of systemic inflammation, and to study whether they are helpful in distinguishing between ReA and RA. METHODS: In 28 patients with acute ReA, 16 patients with early untreated RA, and 25 patients with blood culture-positive sepsis, phagocyte CD 11b expression was measured by flow cytometry, serum procalcitonin (PCT) levels by immunoluminometric assay, and soluble E-selectin (sE-selectin) levels by enzyme-linked immunosorbent assay (ELISA). RESULTS: Neutrophil and monocyte CD11b expression and serum levels of PCT and sE-selectin were higher in patients with sepsis than patients with ReA or RA, or in healthy subjects (all p < 0.01). They were comparable in healthy subjects, ReA, and RA. CONCLUSION: Patients with acute ReA and early RA have normal CD11b expression levels on phagocytes and normal PCT and sE-selectin levels in serum. Elevated levels suggest possible sepsis

8. LOTITO APN, MUSCARA MN, KISS MHB, TEIXEIRA SA, NOVAES GS, LAURINDO IMM, SILVA CAA, MELLO SBV: Nitric Oxide-Derived Species in Synovial Fluid from Patients with Juvenile Idiopathic Arthritis. Journal of Rheumatology (Canada ) 31:5 992-997, 2004
   Abstract: Objective. To evaluate superoxide anion (OSUB2-), nitrite/nitrate (NOSUB2-/NOSUB3), and nitrotyrosine (NT) production and the contribution of myeloperoxidase (MPO) to the production of NT-containing proteins in the synovial fluid (SF) of patients with juvenile idiopathic arthritis (JIA). The affected tissues in inflammatory arthritis produce large amounts of nitric oxide (NO) or peroxynitrite (ONOO-) but there are no reports of NO or ON

9. MARUYAMA K, MURAMATSU H, ISHIGURO N, MURAMATSU T: Midkine, a heparin-binding growth factor, is fundamentally involved in the pathogenesis of rheumatoid arthritis. Arthritis Rheum 50:5 1420-1429, 2004
   Organism: Department of Biochemistry, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, JapanFAU - Maruyama, Kiyoko
   Abstract: OBJECTIVE: Midkine (MK), a heparin-binding growth factor, promotes growth, survival, and migration of various cells. The essential role of MK in migration of inflammatory cells has been shown using mice deficient in the MK gene (Mdk(-/-) mice). We undertook this study to investigate the role of MK in the pathogenesis of rheumatoid arthritis (RA). METHODS: MK levels in specimens from patients were determined by enzyme-linked immunosorbent assay, and localization of MK was revealed by immunohistochemical analysis. Susceptibility to antibody-induced arthritis was compared between Mdk(-/-) and wild-type (WT) mice. Osteoclast differentiation was monitored using macrophage-like cells isolated from human synovial tissue and macrophages from mouse bone marrow. RESULTS: MK levels in sera and synovial fluid were increased in most RA patients, indicating a strong correlation between MK expression and RA. MK was expressed in macrophage-like cells and fibroblast-like cells in synovial membranes from the patients. In antibody-induced arthritis, Mdk(-/-) mice seldom developed the disease, while most of the WT mice did. Administration of MK to the Mdk(-/-) mice increased the frequency of antibody-induced arthritis. Migration of inflammatory leukocytes to the synovial membranes in the disease model was suppressed in the Mdk(-/-) mice. Furthermore, MK was found to promote the differentiation of osteoclasts from macrophages. CONCLUSION: MK participates in each of the two distinct phases of RA development, namely, migration of inflammatory leukocytes and osteoclast differentiation, and is a key molecule in the pathogenesis of RA

10. MASI L, SIMONINI G, PISCITELLI E, DEL MONTE F, GIANI T, CIMAZ R, VIERUCCI S, BRANDI ML, FALCINI F: Osteoprotegerin (OPG)/RANK-L System in Juvenile Idiopathic Arthritis: Is There a Potential Modulating Role for OPG/RANK-L in Bone Injury? Journal of Rheumatology (Canada ) 31:5 986-991, 2004
    Abstract: Objective. To evaluate serum levels of osteoprotegerin (OPG) and receptor activator of nuclear factor kappaB-ligand (RANK-L) in patients with juvenile idiopathic arthritis (JIA); to correlate these values with disease activity variables, radiological bone damage, and bone mass; and to correlate OPG gene polymorphisms with bone mass. Methods. Eighty-four patients (66 girls and 18 boys) with JIA and 40 sex and age-matched controls were enrolled. Serum OPG and RANK-L were measured using an enzyme-linked immunosorbent assay. OPG genotyping was performed by polymerase chain reaction. Results. Patients with JIA had significantly higher levels of serum OPG than controls (p = 0.001) and lower levels of RANK-L in comparison with controls (p = 0.0003). The OPG/RANK-L ratio in patients was higher than in controls (p = 0.004). No significant correlations were found between disease duration, erythrocyte sedimentation rate, and C-reactive protein values with either OPG or RANK-L serum levels. A significant difference in serum OPG levels (but not in RANK-L) was found between patients with and without erosions (p = 0.008). No correlation was found between OPG and RANK-L levels and bone mass (DXA Z scores). A higher prevalence of OPG CC genotype was found in both patients (65.4%) and controls (82.5%) (p = 0.006). Subjects with CC genotype had a higher lumbar spine bone mineral density (LS-BMD). Conclusion. We evaluated for the first time levels of OPG and RANK-L in children with JIA. The higher OPG/RANK-L ratio in JIA might be the result of a compensatory production of OPG. The presence of the T allele of the OPG gene appears to be associated with low BMD

11. MCCARBERG B: Impact of guidelines on healthcare from the patient and payor perspective: Example of the American pain society guidelines. Disease Management and Health Outcomes (New Zealand ) 12:2 73-79, 2004
    Abstract: Clinical practice relies on structured learning that is applied to a patient care setting. Patients present with symptoms and providers try to fit these complaints into known disease categories. Providers depend on memorized algorithms to direct diagnosis and treatment. Well thought-out guidelines developed by professional societies and based on the best available evidence have become the standard in modern medical care. Guidelines are developed to assist practitioners in making appropriate healthcare decisions, to help standardize medical care and improve quality. Guidelines attempt to change practice behavior towards an established norm when evidence is available or toward a consensus opinion when randomized trials are lacking. Patients seeking medical care expect that their practitioner is providing up-to-date, quality medical care. The payors of this care are also interested in quality but must pay attention to medical costs. Both the patient and the payor are invested in providers who use the best available evidence in providing care that is clearly based on guideline development and dissemination. The American Pain Society (APS) has written three practice guidelines, the most recent of which is the Guideline for the Management of Pain in Osteoarthritis, Rheumatoid Arthritis, and Juvenile Chronic Arthritis, published in 2002. Based on high quality research, when available, and consensus opinion from opinion leaders, these guidelines are endorsed throughout the world's medical community. Like any other guidelines, the goal is to standardize care and improve quality. The future will bring more development of guidelines like those from the APS. As studies improve and trials show more effective treatment paradigms, well researched, appropriately designed guidelines will emerge which are structured for busy practices. But this will not be enough; providers need to be made aware of the guidelines, educated on their use, shown appropriate studies documenting efficacy, given simple strategies to implement these guidelines, repeatedly reminded of the appropriate care and, finally, monitored for compliance. For guidelines to be effective they must be tested, disseminated, and their impact on healthcare outcomes must be assessed. Payors want evidence of the value for their expenditures and guidance by professional societies in allocating limited resources. At the same time, payors are struggling with quality issues. Guidelines can help standardize care that may also be more cost effective as well as satisfy quality concerns from regulatory agencies and the public. Understanding where guidelines make an impact and why resistance develops to well meaning, expert based documents will help us in our disease management efforts

12. MCCORMACK PL, WELLINGTON K: Etanercept: in ankylosing spondylitis. BioDrugs 18:3 199-205, 2004
    Organism: Adis International Inc, Yardley, Pennsylvania, USAFAU - McCormack, Paul L
    Abstract: black triangle Etanercept is a dimeric fusion protein based on the p75 tumor necrosis factor (TNF) receptor. It binds to TNFalpha and blocks its biological activity. black triangle Subcutaneous etanercept is effective in the treatment of rheumatoid arthritis, psoriatic arthritis, and polyarticular-course juvenile rheumatoid arthritis. More recently, etanercept has shown efficacy in the treatment of adults with ankylosing spondylitis. black triangle In randomized, double-blind, placebo-controlled trials, subcutaneous etanercept 25mg twice weekly for 6-24 weeks significantly reduced disease activity in patients with active ankylosing spondylitis. In the largest trial, etanercept produced a response rate of 57% compared with 22% for placebo after 24 weeks (response was determined via the validated ASAS 20 response criteria developed by the Assessments in Ankylosing Spondylitis [ASAS] Working Group). black triangle Etanercept therapy significantly improved health-related quality of life in patients with ankylosing spondylitis compared with placebo. The greatest improvements in a 16-week study were seen in the domains of physical functioning, physical role, bodily pain, vitality, and social functioning. black triangle Etanercept was generally well tolerated, with few serious adverse events or treatment withdrawals. The most common adverse events were injection-site reactions and minor upper respiratory tract infections

13. MINDEN K, NIEWERTH M, ZINK A: National paediatric rheumatologic database
    KERNDOKUMENTATION RHEUMAKRANKER KINDER UND JUGENDLICHER. Medizinische Welt (Germany ) 55:4 73-77, 2004
    Abstract: The national paediatric rheumatologic database prospectively and continuously collects clinical and patient questionnaire data of children and adolescents with inflammatory rheumatic diseases who are undergoing rheumatology care. Almost all German paediatric rheumatology units take part in this documentation, recording approximately 5.000 children and adolescents per year. The database provides national data on the processes and outcomes of rheumatology care in Germany. It allows to describe the course and burden of several rheumatic diseases in children, to demonstrate standards and trends of health care provision, as well as to identify health care deficits. In times of limited resources and health care system reorganisations it can serve as a tool for health care policy issues and quality assessment

14. MUNRO JE, MURRAY KJ: Advances in paediatric rheumatology: Beyond NSAIDs and joint replacement. Journal of Paediatrics and Child Health (Australia ) 40:4 161-169, 2004
    Abstract: Over the previous three decades there have been a number of dramatic changes in our understanding of both the pathogenesis and epidemiology of the rheumatic diseases of childhood. Improvements in the classification of paediatric-onset arthritides and international collaboration in terms of multicentre research have led to the development of new therapeutic agents and better methods of outcome assessment for these chronic and often disabling conditions. Fortunately for children with paediatric rheumatic diseases treatment regimes are now available that provide excellent disease control for many and remission induction for some. Challenges include clearer definition of the genetics and pathogenesis of the diseases, delineation of reliable biological markers for diagnosis and monitoring of disease activity. The future should also herald early identification of those with a poorer prognosis, together with the design of more powerful, safer and cheaper remissioninducing agents, given to the right patients at the right time

15. NANDA S, BATHON JM: Etanercept: a clinical review of current and emerging indications. Expert Opin Pharmacother 5:5 1175-1186, 2004
    Organism: Johns Hopkins Medical Institutions, Baltimore, MD 21224, USAFAU - Nanda, Shikha
    Abstract: The recent development of inhibitors of TNF-alpha has provided the opportunity for a more targeted and highly effective approach to the treatment of chronic inflammatory illnesses such as rheumatoid arthritis. Since the initial approval of etanercept as a treatment for rheumatoid arthritis, additional indications, including psoriatic arthritis, juvenile rheumatoid arthritis and ankylosing spondylitis, have also received FDA approval. More than 220,000 patients have been treated with etanercept so far. This review summarises the body of knowledge accumulated so far on etanercept (Enbrel) since it entered the market 5 years ago

16. OZKAYA O, CANTURK F, ALAYLI G, AKPOLAT I, BELET U, DIREN B: An unusual presentation of familial Mediterranean fever with prolonged hip pain and amyloidosis. Scand J Rheumatol 33:2 123-125, 2004
    Organism: Department of Paediatric Nephrology, Ondokuz Mayis University, Samsun, Turkey ozanozkaya@yahoocomFAU - Ozkaya, O
    Abstract: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limiting attacks of joint, chest and abdominal associated with fever. We present an unusual case of FMF with prolonged arthritis and amyloidosis. Familial Mediterranean fever should be considered in the differential diagnosis of prolonged hip pain, even in the absence of symptoms or signs of FMF

17. PALERMO TM, WITHERSPOON D, VALENZUELA D, DROTAR DD: Development and validation of the Child Activity Limitations Interview: a measure of pain-related functional impairment in school-age children and adolescents. Pain 109:3 461-470, 2004
    Organism: Division of Behavioral Pediatrics and Psychology, Department of Pediatrics, Rainbow Babies and Children's Hospital, 11100 Euclid Avenue, Cleveland, OH 44106-6038, USAFAU - Palermo, Tonya M
    Abstract: Recurrent pain in childhood are common and frequently impact children's everyday functioning. However, there are currently limited tools available to measure the impact of recurrent pain on children's daily activities, in particular, that can be used to identify appropriate targets for intervention and measure response to such interventions. The purpose of this study was to develop and validate a new measure, the Child Activity Limitations Interview (CALI), to improve the assessment of functional impairment due to recurrent pain in school-age children and adolescents, and to compare this measure to the Functional Disability Inventory. Participants included 189 children, aged 8-16 years ( [Formula: see text] SD 2.5), 60% female, 40% minority, who were part of a longitudinal study of recurrent pain in children with headaches, juvenile idiopathic arthritis, and sickle cell disease. Measures of socio-demographics, pain, anxiety and depression, and functional disability were completed. A subset of participants (47%) were re-administered the CALI 1 month later and completed prospective ratings of pain and activity limitations using the CALI in daily diaries. Internal consistency of the CALI was excellent ( [Formula: see text] child version; [Formula: see text] parent version). One-month test-retest reliability ( [Formula: see text] child report) and cross-informant reliability [Formula: see text] were moderate. Results demonstrate support for face, construct, and concurrent validity as well as responsiveness to pain symptom fluctuation. Findings demonstrate that the CALI is a promising measure for assessing and monitoring subjective report of functional impairment in school-age children and adolescents with recurrent and chronic pain

18. RAVELLI A: Toward an understanding of the long-term outcome of juvenile idiopathic arthritis. Clinical and Experimental Rheumatology (Italy ) 22:3 271-275, 2004
    Abstract: Over the past four decades, a number of studies have evaluated the long-term outcome of juvenile idiopathic arthritis (JIA) and some of them have also attempted to identify early prognostic factors. This editorial addresses, by reviewing the surveys that have analyzed the outcome of JIA in term of clinical remission, physical disability, and radiographic damage, the clinical questions that are most relevant in this area of study. Altogether, the available data indicate that JIA is not a benign disease because a considerable number of patients still enter adulthood with persistently active disease and a significant proportion of them may develop severe physical disability. Among the different onset forms, the long-term outcome is best in persistent oligoarthritis and worst in RF-positive polyarthritis; the outcome of systemic arthritis is widely variable, perhaps reflecting the heterogeneity of this JIA subtype. The comparison of earlier studies with those published in the last decade shows a decline in the frequency of patients with severe physical disability over the years; however, the proportion of patients who enter adulthood with active disease does not seem to be diminished. Although there is considerable data on prognostic factors in JIA, prediction of long-term outcome early after disease presentation is still difficult because comparisons among studies are hindered for a variety of reasons. Thus, while a considerable body of data is accumulating, the definition of the long-term outcome of JIA remains imperfect. To increase the comparability of future analyses and to obtain generalizable information on the prognosis of JIA and its prediction, a great deal of effort should be directed toward standardizing the study design and the measurement of predictors and outcomes. (c) Copyright Clinical and Experimental Rheumatology 2004

19. ROSEN P, HOPKIN RJ, GLASS DN, GRAHAM TB: Another Patient with Chromosome 18 Deletion Syndrome and Juvenile Rheumatoid Arthritis. Journal of Rheumatology (Canada ) 31:5 998-1000, 2004
    Abstract: Previously, 4 children with deletion of the long arm of chromosome 18 and chronic arthritis were reported. We present an 8-year-old girl with arthritis, atrial septal defect, external auditory canal atresia, and developmental delay. She is the fifth child reported with 18q- syndrome and juvenile rheumatoid arthritis. Evidence is mounting that genetic loci on chromosome 18 may play a role in the expression of complex autoimmune diseases. Idiopathic arthritis should be considered as a potential additional feature in 18q- syndrome

20. RUTKOWSKA-SAK L, RYZKO J, WAGNER T, LEGATOWICZ-KOPROWSKA M: Gastrointestinal system involvement in juvenile idiopathic arthritis complicated with amyloidosis
    ZMIANY W UKl(stroke)ADZIE POKARMOWYM W PRZEBIEGU UKl(stroke)ADOWEJ POSTACI Ml(stroke)ODZIENCZEGO IDIOPATYCZNEGO ZAPALENIA STAWOW POWIKl(stroke)ANEGO AMYLOIDOZA. Reumatologia (Poland ) 42:1 5-19, 2004
    Abstract: Functional and morphological changes of the gastrointestinal system were assessed in patients suffering from juvenile idiopathic arthritis complicated with amyloidosis. The results of endoscopic investigation of the gastrointestinal tract showed amyloidosis and inflammatory changes, cardiac and pylorus atony and refluxes. Ultrasonography results suggested hepatic, bile ducts and pancreatic inflammatory changes, steatosis and/or amyloidosis, dyskinesis. The above - mentioned changes were confirmed by morphological investigation (biopsy and/or autopsy). The results of the capacity tests performed in patients revealed an impairment hypoacidity and reduced gastric juice secretion, high serum gastrin level, distrubance liver detoxication function and pancreas and bowel functional capacity as well. Additionally, intolerance against lactose, casein and alfa- and beta-lactoglobuline was found in most of the patients. Some symptoms of malnutrition and malabsorption were observed in all of them. Such factors as underlying disease severity, adverse drug effects, environmental factors (infections, nutrition) were taken into consideration as probable causes of the gastrointestinal system damage

21. SCHAED SG, EISENKEIL A, MAEDER U, TRCKA J_(REPRINT), BROECKER EB, SPEER CP, HAMM H, GIRSCHICK HJ: Activity of disease as an important new risk factor for naproxen-induced pseudoporphyria in juvenile idiopathic arthritis. Archives of Dermatological Research 295:8-9 360, 2004

22. SCHULZE ZUR WA, FOELL D, FROSCH M, VOGL T, SORG C, ROTH J: Myeloid related proteins MRP8/MRP14 may predict disease flares in juvenile idiopathic arthritis. Clinical and Experimental Rheumatology (Italy ) 22:3 368-373, 2004
    Abstract: Objective. An unsolved problem in juvenile idiopathic arthritis (JIA) is to identify patients at special risk for relapse. It is important to adjust anti-inflammatory and immunosuppressive therapy to the children's actual disease activity especially in times of remission. Our aim was to analyze if the serum levels of MRP8/MRP14 are reliable predictive markers for the risk of relapse in clinically inactive juvenile idiopathic arthritis. Methods. Serum concentrations of MRP8/MRP14 were determined by ELISA and correlated with laboratory and clinical parameters for disease activity in patients with JIA. 29 patients with changing disease activity were followed up for a mean time of 2.9 years. Two groups of patients - one before relapse (mean 3.7 months) but without clinical signs of disease reactivation, and one in remission for 12 further months - were compared. Results. MRP8/MRP14 serum levels in patients before relapses were significantly higher than the levels in patients in stable remission for one year (662 ng/ml versus 395 ng/ml; p < 0.05). Using a cut-off for MRP8/MRP14 of 450 ng/ml the likelihood ratio for relapse was 3.7 (positive predictive value 80%), while no differences were found for C-reactive protein and erythrocyte sedimentation rate between the two groups. Conclusion. MRP8/MRP14 correlate with individual disease activity in patients with JIA. Our data suggest that local disease activity may be present even months before flares become clinically apparent. Serum levels of MRP8/MRP14 can give a hint as to clinically occult disease activity, in this way helping to adjust therapy in times of low disease activity. (c) Copyright Clinical and Experimental Rheumatology 2004

23. SHAW KL, SOUTHWOOD TR, MCDONAGH JE: Transitional care for adolescents with juvenile idiopathic arthritis: a Delphi study. Rheumatology (Oxford) .: 2004
    Organism: Institute of Child Health, University of Birmingham, Birmingham, UK
    Abstract: Objective. To identify the ideal programme of transitional care for adolescents with juvenile idiopathic arthritis (JIA) as perceived by users and providers, and to examine the feasibility of achieving this within a UK National Health Service context. Methods. A modified two-stage Delphi study was undertaken with rheumatology health professionals, young people with JIA (aged 12-25 yr) and their parents. Participants were presented with statements about transitional care and asked to rate (i) the extent to which these constituted best practice, and (ii) their feasibility. Results. Second-round questionnaires were completed by 83 individuals, representing an overall response rate of 90%. Items strongly agreed to constitute best practice and highly feasible included: 'addressing young people's psychosocial and educational/vocational needs'; 'using an individualized approach'; 'providing honest explanations of the adolescent's condition and health-care'; 'providing opportunities for adolescents to express opinions and make informed decisions'; 'having continuity in health personnel'; and 'giving adolescents the option of being seen by professionals without their parents'. However, providing adolescent-focused environments, professionals knowledgeable in transitional care and opportunities for young people with JIA to meet similar others were seen as feasible in only a few hospitals. Conclusions. There is considerable agreement as to the most important elements of transitional care. Those that are easily achievable should be undertaken in all hospitals that care for adolescents with JIA. However, not all elements identified were perceived as easily feasible; further research is required to determine how to implement these elements

24. SMERDEL A, DAI KZ, LORENTZEN AR, FLATO B, MASLINSKI S, THORSBY E, FORRE O, SPURKLAND A: Genetic association between juvenile rheumatoid arthritis and polymorphism in the SH2D2A gene. Genes Immun 5:4 310-312, 2004
    Organism: Institute of Immunology, Rikshospitalet University Hospital, Oslo, Norway annasmerdel@klinmeduionoFAU - Smerdel, A
    Abstract: T-cell-specific adapter protein (TSAd) involved in the negative control of T-cell activation is encoded by the SH2D2A gene. Our recent studies indicate that homozygosity for short (ie GA(13) and GA(16)) alleles of the SH2D2A gene promoter is associated with development of multiple sclerosis. To study whether the same SH2D2A promoter polymorphism also contributes to the genetic susceptibility to develop juvenile rheumatoid arthritis (JRA), we examined 210 JRA patients and 558 healthy unrelated controls from Norway. The frequency of the short allele GA(13) was increased among the JRA patients compared to control (0.098 vs 0.05; P(n=8)=0.042). There was a significant increased frequency of HLA-DRB1(*)08-positive patients carrying two copies of 'short' alleles GA(13) and/or GA(16) compared to healthy controls (16% vs 6%; P(n=4)=0.016). Our data indicate that the 'short' alleles of the SH2D2A promoter could contribute to the genetic susceptibility to JRA

25. SUZUKI K, TANAKA H, ITO E, WAGA S: Therapy-related membranous nephropathy in juvenile idiopathic arthritis with Turner syndrome. Pediatr Int 46:3 377-379, 2004
    Organism: Department of Pediatrics, Hirosaki University School of Medicine, Hirosaki, Japan skoichi@infoaomorinejpFAU - Suzuki, Koichi