Bibliography September 2006

1. AL MAYOUF SM: Familial Arthropathy in Saudi Arabian Children: Demographic, Clinical, and Biochemical Features, Semin.Arthritis Rheum., Vol. %20;., 2006
   Organism:Consultant and Section Head, Rheumatology, Department of Pediatrics, Section of Rheumatology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
   Abstract:BACKGROUND: Familial arthropathy comprises a heterogeneous group of arthropathies. It can be either an inflammatory or a noninflammatory condition. The worldwide frequency of these disorders is unknown. OBJECTIVE: To study the demographic, clinical and biochemical features, and survival of a large series of children with familial arthropathies. METHODS: The medical records of children who had an arthropathy and a family history of a similar condition at the Pediatric Rheumatology Clinic at King Faisal Specialist Hospital-Riyadh between 1990 and 2005 were reviewed. These included children with familial juvenile idiopathic arthritis (FJIA), infantile systemic hyalinosis (ISH), the nodulosis-arthropathy-osteolysis (NAO) syndrome, and the camptodactyly-arthropathy-coxa vara (CAC) syndrome. Familial rheumatic diseases including spondyloarthropathies or known syndromes associated with articular manifestations were excluded. In each case age, gender, presenting symptoms, laboratory data, diagnostic procedures, and provisional and final diagnoses as well as treatment and outcome were reviewed. RESULTS: Sixty-two children with various familial arthropathies were reviewed. Twelve children (9 female/3 male) with FJIA presented with polyarthritis. These children belonged to 4 unrelated families, all of whom were from the same geographical area, with 2 families belonging to the same tribe. The mean age at onset was 2.4 years, and mean age at diagnosis was 3.5 years. All children had high inflammatory serologic markers. Nineteen children (11 male/8 female) with ISH presented in the neonatal period with painful joint contractures and typical mucocutaneous features. The referral diagnosis was inaccurate in 14 patients. Thirteen patients were the product of first-degree cousin marriages, and 5 families had more than 1 affected child. Radiological findings included periosteal reaction and osteolytic lesions. Tissue biopsy was performed in 8 patients and the findings were consistent with the diagnosis in all 8 patients. Despite aggressive management, 16 patients died. The mean age of the remaining 3 surviving children was 20 months. There were 15 children (9 female/6 male) with the NAO syndrome with a mean age at onset of 3.4 years. They were from 7 unrelated families; 5 families had more than 1 affected child. The referral diagnosis was juvenile idiopathic arthritis (JIA). Most children presented with painful deformed hands. Eleven children (70%) had advanced osteolytic changes. All children had normal inflammatory markers. There were 16 children (11 male/5 female) with the CAC syndrome who were diagnosed at a mean age of 3.7 years. Camptodactyly presented at birth or in first months of life, while other features developed in early childhood. JIA was the referral diagnosis. Fourteen children had bilateral coxa vara. Two children exhibited symptoms or signs of pericarditis. Inflammatory markers were normal in all children. CONCLUSION: Familial arthropathies are not uncommon conditions which may be easily confused with JIA, causing a delay in diagnosis and management. Careful evaluation of a child presenting with an arthropathy, particularly in a population where consanguinity is common, is required for timely and accurate diagnosis. Overall, the prognosis of these conditions remains guarded despite treatment

2. AVCIN T, TSE SHIRLEY ML, SCHNEIDER R, NGAN Bet SILVERMAN ED: Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood, Journal of Pediatrics, Vol. 148, 683-686., 2006
   Organism:Univ Toronto, Hosp Sick Children, Div Rheumatol, 555 Univ Ave, Toronto, ON M5G 1X8, Canada Canada
   Abstract:We describe 3 patients who presented with features of macrophage activation syndrome (MAS) at the time of presentation of systemic lupus eryhematosus (SLE), systemic juvenile idiopathic arthritis, and Kawasaki disease. Immunohistochemical,l studies in the patient with SLE demonstrated extensive expression of CD163 on hemophagocyte macrophages, suggesting a possible role as a marker of MAS

3. BEUKELMAN T, ARABSHAHI B, CAHILL AM, KAYE RDet CRON RQ: Benefit of Intraarticular Corticosteroid Injection Under Fluoroscopic Guidance for Subtalar Arthritis in Juvenile Idiopathic Arthritis, J.Rheumatol., Vol. ., 2006
   Organism:OBJECTIVE: To determine the demographics of subtalar arthritis, the response to intraarticular corticosteroid injection, and the injection complication rate in a clinic sample of children with juvenile idiopathic arthritis (JIA). METHODS: A chart review was performed of all patients at a tertiary medical center who underwent subtalar corticosteroid injection during the past 5 years. Injection of 1 ml of triamcinolone hexacetonide or acetonide into the midsubtalar joint was performed using a lateral oblique approach under fluoroscopic guidance. Improvement was defined by enhanced foot inversion and eversion at the following office visit. RESULTS: Thirty-eight patients underwent 55 subtalar injections during the study period. All 7 JIA subtypes were represented. Thirty-one patients (82%) had subtalar arthritis at time of JIA diagnosis and 32 (84%) had concomitant tibiotalar ankle arthritis. Improvement was observed following 34 (89%) of the initial 38 injections. The mean duration of improvement was 1.2 years (SD +/- 0.9). Twenty patients (53%) developed hypopigmentation or subcutaneous atrophy. This complication was associated with a higher volume of injected corticosteroid per patient weight (p = 0.02) and with less efficacious injections (p = 0.04). CONCLUSION: Subtalar arthritis in children with JIA is common. Similar to other joints, subtalar arthritis responds to corticosteroid injection in approximately 90% of cases and often remains improved for greater than one year. Hypopigmentation and subcutaneous atrophy are frequent complications and are likely related to the dose of injected corticosteroid and possibly the accuracy of needle placement

4. BREZA Set MAGRO CM: Lichenoid and granulomatous dermatitis associated with atypical mycobacterium infections, J.Cutan.Pathol., Vol. 33(7), 512-515., 2006
   Organism:College of Medicine, The Ohio State University, Columbus, OH 43221, USAFAU - S Breza, Thomas Jr
   Abstract:BACKGROUND: Lichenoid and granulomatous dermatitis defines a distinctive pattern of cutaneous inflammation that may be part of the morphologic spectrum of idiopathic lichenoid reactions such as lichen planus and as well may be seen with lichenoid drug reactions, endogenous T-cell dyscrasias and as a feature of certain systemic diseases especially Crohn's disease and rheumatoid arthritis. RESULTS: We encountered three cases of lichenoid and granulomatous dermatitis in which the basis was one of primary cutaneous Mycobacterium infection. In all three cases acid fast stains revealed pathogenic organisms and as well cultures were positive for Mycobacterium kansasii in one case and Mycobacterium marinum in another. Other features included a prominent perineural and periadnexal lymphocytic infiltrate. CONCLUSIONS: The differential diagnosis of lichenoid and granulomatous dermatitis should also encompass primary cutaneous Mycobacterium infection in addition to the other more characteristic entities associated wtih this distinctive reaction pattern. Infection with Mycobacterium induces a TH1 dominant response which would hence produce an infiltrate

5. BRUNNER H, I, TAYLOR J, BRITTO MT, CORCORAN MS, KRAMER SL, MELSON PG, KOTAGAL UR, GRAHAM TBet PASSO MH: Differences in disease outcomes between Medicaid and privately insured children: Possible health disparities in juvenile rheumatoid arthritis, Arthritis & Rheumatism, Vol. 55, 378-384., 2006
   Organism:Univ Cincinnati, Childrens Hosp, Med Ctr, William Rowe Div Rheumatol, E 4010,3333 Burnet Ave, Cincinnati, OH 45229 USA USA
   Abstract:Objective. To determine the relationship between health insurance status and disease outcome in children with juvenile rheumatoid arthritis (JRA).Methods. IRA patients followed at a tertiary pediatric rheumatology center were assessed for the number of active joints and number of joints with limited range of motion. Disease activity, patient well-being, and pain were measured. Disability was assessed by the Childhood Health Assessment Questionnaire, health-related quality of life by the Pediatric Quality of Life Inventory (PedsQL) Generic Core Scale, and the PedsQL Rheumatology Module. Health care resource utilization was estimated based on the number of billing events for health services coded in administrative databases; these databases a lso provided information on patient health insurance status. Children insured by Medicaid or similar state programs for low-income families were considered to have Medicaid status. Disease outcomes of children with Medicaid status was compared with that of children with private health insurance.Results. Forty (14%) of the 295 children with IRA had Medicaid status. Patients with Medicaid status were more often of nonwhite race (P <= 0.04) and more frequently had a polyarticular or systemic disease course (P = 0.04) compared with other patients (n = 255). After correction for differences in disease duration, race, IRA onset, and IRA course between groups, children with Medicaid status continued to have significantly higher disability (P < 0.0003), and lower mean PedsQL Generic Core Scale scores (P < 0.05), while health resource utilization appeared similar between groups.Conclusion. Despite apparently similar health resource utilization and joint involvement, Medicaid status is associated with significantly lower health-related quality of life and higher disability in IRA

6. BRUNNER Jet SITZMANN FC: The diagnostic value of anti-cyclic citrullinated peptide (CCP) antibodies in children with Juvenile Idiopathic Arthritis, Clin.Exp.Rheumatol., Vol. 24(4), 449-451., 2006
   Organism:Department of Pediatrics, Innsbruck Medical School, Austria juergenbrunner@uklibkacatFAU - Brunner, J
   Abstract:BACKGROUND: Antibodies against cyclic citrullinated peptide (anti-CCP) are considered to be specific for rheumatoid arthritis (RA). OBJECTIVE: To assess the clinical significance of anti-CCP antibodies in a cohort of patients with juvenile idiopathic arthritis (JIA) and if they can be used to identify patients with an unfavourable course of disease. METHODS: 68 serum samples were investigated. 45 patients were diagnosed with JIA (15 male and 30 female) aged 1.9-17.3 years (median 12.9 mean 11,0). 5 patients had polyarticular (RF negative), 2 polyarticular (RF positive), 25 oligoarticular JIA, 6 enthesitis related arthritis, 2 psoriatric arthritis, 3 patients had systemic disease and 2 unclassified arthritis. 23 samples were taken from patients with non-inflammatory cardiac diseases undergoing interventional cardiac therapy. Enzyme-linked immunosorbent assay (ELISA; Euroimmun, Lubeck, Germany) was used for the detection and quantification of anti-CCP antibodies in patients with JIA. RESULTS: Overall, anti-CCP antibodies were found in 2.9% (2/68) of all samples and in 4.4 % (2/45) patients with JIA. CONCLUSION: Anti-CCP antibodies are associated with RF positive polyarticular course of JIA. Anti-CCP antibodies are not relevant for other subgroups of JIA. Therefore anti-CCP Abs in patients with JIA should not be investigated routinely

7. BRUNNER JKHet SITZMANN FC: The diagnostic value of anti-cyclic citrullinated peptide (CCP) antibodies in children with juvenile idiopathic arthritis|, 2006
   Organism:Background. Antibodies against cyclic citrullinated peptide (anti-CCP) are considered to be specific for rheumatoid arthritis (RA). Objective. To assess the clinical significance of anti-CCP antibodies in a cohort of patients with juvenile idiopathic arthritis (JIA) and if they can be used to identify patients with an unfavourable course of disease. Methods. 68 serum samples were investigated. 45 patients were diagnosed with JIA (15 male and 30 female) aged 1.9-17.3 years (median 12.9 mean 11,0). 5 patients had polyarticular (RF negative), 2 polyarticular (RF positive), 25 oligoarticular JIA, 6 enthesitis related arthritis, 2 psoriatric arthritis, 3 patients had systemic disease and 2 unclassified arthritis. 23 samples were taken from patie nts with non-inflammatory cardiac diseases undergoing interventional cardiac therapy. Enzyme-linked immunosorbent assay (ELISA; Euroimmun, Lubeck, Germany) was used for the detection and quantification of anti-CCP antibodies in patients with JIA. Results. Overall, anti-CCP antibodies were found in 2.9% (2/68) of all samples and in 4.4 % (2/45) patients with JJA. Conclusion. Anti-CCP antibodies are associated with RF positive polyarticular course of JIA. Anti-CCP antibodies are not relevant for other subgroups of JIA. Therefore anti-CCP Abs in patients with JIA should not be investigated routin ely. (c) Copyright Clinical and Experimental Rheumatology 2006

8. BURGER D, DAYER JM, PALMER Get GABAY C: Is IL-1 a good therapeutic target in the treatment of arthritis?, Best.Pract.Res.Clin.Rheumatol., Vol. 20(5), 879-896., 2006
   Organism:Clinical Immunology Unit, Division of Immunology and Allergy, Department of Internal Medicine, University Hospital, 24 rue Micheli-du-Crest, CH-1211 Geneva 14, SwitzerlandFAU - Burger, Danielle
   Abstract:Inflammation is an important homeostatic mechanism that limits the effects of infectious agents. However, inflammation might be self-damaging and therefore has to be tightly controlled or even abolished by the organism. Interleukin 1 (IL-1) is a crucial mediator of the inflammatory response, playing an important part in the body's natural responses and the development of pathological conditions leading to chronic inflammation. While IL-1 production may be decreased or its effects limited by so-called anti-inflammatory cytokines, in vitro IL-1 inflammatory effects are inhibited and can be abolished by one particularly powerful inhibitor, IL-1 receptor antagonist (IL-1Ra). Recent research has shown that in the processes of rheumatoid arthritis (RA) IL-1 is one of the pivotal cytokines in initiating disease, and IL-1Ra has been shown conclusively to block its effects. In laboratory and animal studies the inhibition of IL-1 by either antibodies to IL-1 or IL-1Ra proved beneficial to the outcome. Because of its beneficial effects in many animal disease models, IL-1Ra has been used as a therapeutic agent in human patients. The recombinant form of IL-1Ra, anakinra (Kineret((R)), Amgen) failed to show beneficial effects in septic shock and displays weak effects in RA patients. However, IL-1 blockade by anakinra is dramatically effective in systemic-onset juvenile idiopathic arthritis, in adult Still's disease and in several autoinflammatory disorders, most of the latter being caused by mutations of proteins controlling IL-1beta secretion. Importantly, to be efficacious, anakinra required daily injections, suggesting that administered IL-1Ra displays very short-term effects. Better IL-1 antagonists are in the process of being developed

9. CEFLE A, CEFLE K, TUNACI M, OZTURK Set PALANDUZ S: A case of progressive pseudorheumatoid arthropathy of 'childhood' with the diagnosis delayed to the fifth decade, Int.J.Clin.Pract., Vol. 60(10), 1306-1309., 2006
   Organism:Kocaeli University, Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Kocaeli, Istanbul University, Istanbul, Turkey
   Abstract:Progressive pseudorheumatoid arthropathy of childhood (PPAC) is a rare single gene disorder which is frequently misdiagnosed as juvenile rheumatoid arthritis. It is characterised with arthralgia, joint contractures, bony swelling of metacarpophalangeal and interphalangeal joints and platyspondyly. Clinical and laboratory signs of joint inflammation such as synovitis, a high erythrocyte sedimentation rate and an elevated C-reactive protein level are usually absent. Although the disease begins early in life (usually between 3 and 8 years of age), the diagnosis may be delayed. In the present case report, we describe a male patient diagnosed with PPAC at the age of 46 years, although he had been exhibiting the typical radiological and clinical features of the disease since the age of 7 years

10. CHANG JH, MCCLUSKEY PJet GRIGG JR: Recurrent hypopyon in chronic anterior uveitis of pauciarticular juvenile idiopathic arthritis, Br.J.Ophthalmol., Vol. 90(10), 1327-1328., 2006
    Organism:Department of Ophthalmology, St Vincent's Clinic, 1004/438 Victoria Street, Darlinghurst, NSW 2010, Australia jhchang@optusnetcomau
    Abstract:

11. CHEN H, YAO Z-Jet TANG F-L: Study of the proteins associated with Sa antigen|, 2006
    Organism:Objective: To study the proteins associated with Sa antigen, a target of the anti-Sa antibodies specific for rheumatoid arthritis, and to elucidate the nature of these proteins. Methods: Sa antigen was extracted from fresh human placental tissue by anion exchange chromatography and subjected to SDS-PAGE electrophoresis. Serum samples were collected from 155 patients with connective tissue diseases, including rheumatoid arthritis (71 cases), ankylosing spondylitis (11 cases), psoriatic arthritis (7 cases), reactive arthr itis (7 cases), juvenile idiopathic arthritis (4 cases), osteoarthritis (5 cases), polymyalgia rheumatica (6 cases), gout (6 cases), systemic lupus erythematous (7 cases), Sjogren's syndrome (10 cases), adult onset still's disease (3 cases) and Sa antibodies were detected by immunoblotting. The gel bands corresponding to the stained bands were excised, trypsin-digested in gel, and analyzed by LC-ESI-MS/ MS. Once identified, the protein was recombinated and expressed in Escherichia coli, and the antibodies were detected by immunoblotting. Then the protein was citrullinated to detect the antibodies again. Results: Immunoblotting showed anti-Sa antibodies, band(s) with apparent molecular weight of 50 000 (and) 55 000, in 34 of the 71 patients of rheumatoid arthritis and 4 of the 84 patients of other rheumatic diseases, with a sensitivity rate of 47.9% and a specificity rate of 95.2%. The target protein was identified as vimentin. The positive rate of anti-vimentin antibody was statistically different between the RA patients and the patients with other rheumatic diseases (P = 0.005), with a sensitivity rate of 36.6% and a specificity rate of 83.3%, respectively. But there was no obvious correlation between anti-vimentin antibody and anti-Sa antibodies (Kappa = 0.316). The positive rate of anti-citrullinated vimentin antibody was significantly higher in the RA patients than in the patients with other rheumatic diseases (P < 0.01), with a sensitivity rate of 49.3%. There was a high correlation between anti-citrullinated vimentin antibody and anti-Sa antibodies(Kappa = 0.746), albeit a low specificity rate (86.9%). Conclusion: Ci trullinated vimentin is closely correlated with Sa antigen

12. CINEK O, HRADSKY O, AHMEDOV G, SLAVCEV A, KOLOUSKOVA S, KULICH Met SUMNIK Z: No independent role of the -1123 G>C and+2740 A>G variants in the association of PTPN22 with type 1 diabetes and juvenile idiopathic arthritis in two Caucasian populations, Diabetes Res.Clin.Pract., Vol. ., 2006
    Organism:University Hospital Motol, The 2nd Medical School, Charles University, Prague, Czech Republic
    Abstract:INTRODUCTION: The PTPN22 is a negative regulator of the T cell response. Its +1858C>T (R620W) polymorphism has been shown to associate with a risk for multiple autoimmune diseases, including type 1 diabetes (T1D) and juvenile idiopathic arthritis (JIA). The minor (susceptibility) allele is absent in Asian populations, but a recent study suggested an independent involvement of another polymorphism located within the promoter -1123 nucleotides relative to the translational start site. AIMS: We aimed to analyse the association of three PTPN22 polymorphisms in two distinct Caucasian populations, the Czechs (with T1D and with JIA) and Azeri (with T1D). METHODS: The single nucleotide polymorphisms (SNP) at positions -1123 (rs2488457), +1858 (rs2476601, the R620W substitution), and +2740 (rs1217412) were genotyped using TaqMan assays in 372 subjects with childhood-onset T1D, 130 subjects with JIA, and 400 control subjects of Czech origin, and in 160 subjects with T1D and 271 healthy controls of Azeri origin. RESULTS: In the Czechs, all three SNPs were in a tight linkage disequlibrium, while in the Azeri, the linkage disequlibrium was limited to between the promoter and 3'-UTR polymorphism, D'(-1123, +2740)=0.99, r(2)=0.72. Haplotype reconstruction via the expectation-maximization algorithm showed in both populations that only the haplotype containing the minor (W) allele at codon 620 was associated with T1D (OR=2.26, 95% CI 1.68-3.02 in Czechs, OR=14.8, 95% CI 2.0-651 in Azeri) or JIA (OR=2.43, 95% CI 1.66-3.56 in Czechs). The haplotypes having the wild-type (R) allele at codon 620 and minor alleles at -1123and/or +2740 were neutral as to the risk of autoimmune conditions in both populations. CONCLUSIONS: In two different Caucasian populations, the Czechs and the Azeri, no independent contribution can be detected either of the -1123 promoter SNP or the +2740 3'-UTR SNP, and only the minor allele at PTPN22 codon 620 contributes to the risk of autoimmunity

13. CUOMO A, PIRPIRIS Met OTSUKA NY: Case report: biceps tenosynovial rice bodies, J.Pediatr.Orthop.B., Vol. 15(6), 423-425., 2006
    Organism:aDepartment of Orthopaedic Surgery, University of California, Los Angeles bShriners Hospitals for Children, Los Angeles, Los Angles, California, USAFAU - Cuomo, Anna
    Abstract:Here we report the first documented case of rice body formation within the biceps tenosynovium in a patient with juvenile rheumatoid arthritis. We discuss the pathophysiology and the clinical and radiographic findings and correlate these to prior case studies. Lastly, we suggest that limited awareness is a barrier in achieving a timely diagnosis and appropriate management

14. DENMAN AM: Obituary - Leonard Glynn 1910-2005, Rheumatology (Oxford), Vol. 45, 927-928., 2006
    Organism:

15. DESAI SBet FURST DE: Problems encountered during anti-tumour necrosis factor therapy, Best.Pract.Res.Clin.Rheumatol., Vol. 20(4), 757-790., 2006
    Organism:Department of Rheumatology, University of California, Los Angeles, CA, USAFAU - Desai, Sheetal B
    Abstract:Worldwide, over 400 000 patients have been treated with tumour necrosis factor (TNF)-alpha antagonists for indications that include rheumatoid arthritis, juvenile rheumatoid arthritis, inflammatory bowel disease, psoriatic arthritis and ankylosing spondylitis. Since their approval, concerns regarding safety have been raised. There is a risk of re-activation of granulomatous diseases, especially tuberculosis, and measures should be taken for detection and treatment of latent tuberculosis infections. Preliminary data suggest that anti-TNF therapy may be safe in chronic hepatitis C. However, TNF-alpha antagonists have resulted in re-activation of chronic hepatitis B if not given concurrently with antiviral therapy. Solid tumours do not appear to be increased with anti-TNF therapy. Variable rates of increased lymphoma risk have been described with anti-TNF therapy compared with the general population, although no increased risk was found compared with a rheumatoid arthritis population. Large phase II and III trials with TNF-alpha antagonists in advanced heart failure have shown trends towards a worse prognosis, and should therefore be avoided in this population. Both etanercept and infliximab are associated with the formation of autoantibodies, and these autoantibodies are rarely associated with any specific clinical syndrome. Rare cases of aplastic anaemia, pancytopenia, vasculitis and demyelination have been described with anti-TNF therapy. This chapter will discuss the safety profile and adverse events of the three commercially available TNF-alpha antagonists: etanercept, infliximab and adalimumab. The data presented in this review have been collected from published data, individual case reports or series, package inserts, the Food and Drug Administration postmarketing adverse events surveillance system, and abstracts from the American College of Rheumatology and European Congress of Rheumatology meetings for 2005

16. FERREIRA RA, SILVA CH, SILVA DA, SOPELETE MC, KISS MH, MINEO JRet FERRIANI VP: Is measurement of IgM and IgA rheumatoid factors (RF) in juvenile rheumatoid arthritis clinically useful?, Rheumatol.Int., Vol. ., 2006
    Organism:Department of Pediatrics, School of Medicine of Ribeirao Preto, University of Sao Paulo, Av Bandeirantes, 3900, CEP: 14049-900, Ribeirao Preto, SP, Brazil, rosapferreira@uolcombr
    Abstract:The prevalence and clinical relevance of IgM and IgA RF detected by ELISA were studied in 91 patients with juvenile rheumatoid arthritis (JRA) and 45 healthy children. IgM and IgA RF were detected, respectively, in 33 and 44% of the patients, compared to 6.7 and 15.6% of the healthy children (p = 0.001 and 0.0006, respectively). The frequency of IgM RF was significantly higher in patients with polyarticular (52%) as compared to systemic onset JRA (21%; p = 0.04). Five out of ninety-one patients and none of the control group were IgM RF positive by the latex test. High levels of IgM RF were detected more frequently in patients with active disease (p = 0.01) and positive latex agglutination test (p < 0.001) and had a marginally significant association with severe radiological deformities (p = 0.05). The presence of IgA RF was associated with active disease in polyarticular onset JRA children (p = 0.04). In conclusion, high levels of IgM RF and the detection of IgA RF can be useful in assessing clinical activity in a subset of patients with JRA

17. FIFE MS, GUTIERREZ A, OGILVIE EM, STOCK CJ, SAMUEL JM, THOMSON W, MACK LF, LEWIS CMet WOO P: Novel IL10 gene family Associations with Systemic Juvenile Idiopathic Arthritis (sJIA), Arthritis Res.Ther., Vol. 8(5), R148, 2006
    Organism:ABSTRACT: Juvenile idiopathic arthritis (JIA) is the most common cause of chronic childhood disability and encompasses a number of disease subgroups. In this study we have focused on systemic JIA (sJIA), which accounts for approximately 11% of UK JIA cases. This study reports the investigation of three members of the Interleukin-10 (IL10) gene family as candidate susceptibility loci in children with sJIA. DNA from 473 unaffected controls and 172 patients with sJIA was genotyped for a single nucleotide polymorphism (SNP) in IL19 and IL20 and two SNPs in IL10. We examined evidence for association of the four SNPs by single marker and haplotype analysis. Significant differences in allele frequency were observed between cases and controls, for both IL10-1082 (p=0.031) and IL20-468 (p=0.028). Furthermore, examination of the haplotypes of IL10-1082 and IL20-468 reveals greater evidence for association (global p=0.0006). This study has demonstrated a significant increased prevalence of the low expressing IL10-1082 genotype in patients with sJIA. In addition, we have shown a separate association with an IL20 polymorphism, and the IL10-1082A/ IL20-468T haplotype. The two marker A-T haplotype confers an odds ratio of 2.24 for sJIA. This positive association suggests an important role for these cytokines in sJIA pathogenesis

18. FLEISCHMANN R: Anakinra in the treatment of rheumatic disease|, 2006
    Organism:Anakinra is a specific receptor antagonist of interleukin-1 that differs from naturally occurring interleukin-1 receptor antagonist by the presence of a methionine group. It is administered by daily subcutaneous injection. Anakinra improves the clinical signs and symptoms of rheumatoid arthritis, slows radiographic progression and improves patient physical function. The most common adverse event is an injection site reaction. Anakinra has been associated with an increased incidence of serious infections and has an increased standardized incidence ratio for lymphoma. It has not been found to be associated with the development of opportunistic infections, worsening of congestive heart failure or the development of demyelinating disease. It appears to be effective in treating adult Stills disease, systemic-onset juvenile idiopathic arthritis and chronic infantile neurological cutaneous and articular syndrome (also known as neonatal-onset multisystem inflammatory disease syndrome). (c) 2006 Future Drugs Ltd

19. FROMMELT PC: Echocardiographic measures of diastolic function in pediatric heart disease, Current Opinion in Cardiology, Vol. 21, 194-199., 2006
    Organism:Med Coll Wisconsin, Div Pediat Cardiol, Milwaukee, WI 53226 USA USA
    Abstract:Purpose of review The past year has seen a continued evolution in the echocardiographic assessment of diastolic function in children. This paper reviews published studies from the past year that have helped characterize diastolic function using echocardiography in children.Recent findings Characterization of diastolic function using Doppler and Doppler tissue imaging in the normal infant and child was a primary focus of pediatric echocardiographic investigation. These technologies appear to hold significant promise as tools to improve understanding of diastolic function in the normal child as the heart matures. Diastolic function in children with congenital heart disease has also been better characterized using these tools, specifically in patients with atrial septal defects, tetralogy of Fallot, single ventricle physiology, and following cardiac transplantation. Finally, diastolic function in acquired heart disease or with systemic disease in the child has been evaluated using echocardiography, with recent reports describing findings in children with dilated cardiomyopathy, chronic renal disease, obesity, type I diabetes, juvenile rheumatoid arthritis, obstructive sleep apnea, and after anthracycline exposure for childhood cancer.Summary Pediatric echocardiography has clearly become the primary tool for describing and characterizing diastolic function in infants and children both with and without heart disease. It is becoming an important noninvasive diastolic monitoring tool that allows serial assessment of pathologic diastolic disease in both pri mary myocardial and systemic disease states

20. GUMBREVICIUS G, MILASIUS Aet SVEIKATA A: [Nonsteroidal anti-inflammatory agents--choice between disturbances of gastrointestinal tract and cardiovascular toxicity], Medicina (Kaunas.)., Vol. 42(5), 429-439., 2006
    Organism:Department of Theoretical and Clinical Pharmacology, Kaunas University of Medicine, Kaunas, Lithuania gingum@takasltFAU - Gumbrevicius, Gintautas
    Abstract:Nonsteroidal anti-inflammatory agents are used more than 100 years. Most of them can cause undesirable effects on gastrointestinal tract: ulceration, bleeding and perforation of stomach and duodenum. Gastrointestinal toxicity is diminished when selective cyclooxygenase-2 inhibitors are used. However, recent clinical trials have showed that the use of cyclooxygenase-2 inhibitors increases the risk of cardiovascular event and cerebrovascular accident. According to the data such risk may be high using also nonselective nonsteroidal anti-inflammatory agents, however, it is lesser. Incidence rates of the cardiovascular events and cerebrovascular accidents increase due to activated thrombotic activity. Nonsteroidal anti-inflammatory agents are very useful in the management of rheumatic diseases and as pain relievers. Choosing appropriate nonsteroidal anti-inflammatory agent it is essential to consider the risk of gastrointestinal as well cardiovascular damage

21. HAVERKAMP D, EIJER H, PATT TWet MARTI RK: Multi directional intertrochanteric osteotomy for primary and secondary osteoarthritis--results after 15 to 29 years, Int.Orthop., Vol. 30(1), 15-20., 2006
    Organism:Department of Orthopaedic Surgery, Academic Medical Centre, Meibergdreef 15, 1105 AZ, Amsterdam, The Netherlands info@osteotomienlFAU - Haverkamp, D
    Abstract:Between 1974 and 1987, 276 intertrochanteric osteotomies were performed in 217 patients. In 48 hips the osteotomy was done for idiopathic osteoarthritis. In 166 hips the osteoarthritis was secondary to acetabular dysplasia, in 23 to trauma, in 14 to slipped capital femoral epiphysis, in five to Legg-Calve-Perthes' disease and in 20 to avascular necrosis of the femoral head. Good results were achieved in young females with mild osteoarthritis secondary to acetabular dysplasia, and in patients with posttraumatic osteoarthritis. All other indications showed a poorer long-term survival. Our study shows that acetabular dysplasia and posttraumatic arthritis remain valid indications for intertrochanteric osteotomy

22. HEAD AJ, MYERS LK, WATSKY MA, GREENWELL MW, BARROW KD, MICHELSON JAet CARBONE LD: Bone mineral density and turnover in non-corticosteroid treated African American children with juvenile rheumatoid arthritis, Rinsho Ganka, Vol. 33, 1001-1003., 2006
    Organism:Univ Tennessee, Ctr Hlth Sci, Dept Med, Div Rheumatol, Room G326,Coleman Bldg,956 Court Ave, Memphis, TN 38163 USA USA
    Abstract:Objective. To determine bone mineral content (BMC), bone mineral density (BMD), Z scores, and markers of bone turnover in African American children with juvenile rheumatoid arthritis (JRA).Methods. Eight children with JRA with no prior exposure to corticosteroids were evaluated. Lumbar spine (L1-L4) and total body and total hip BMC and BMD were determined using dual x-ray absorptiometry (DXA), and Z scores (BMD) were calculated. Serum samples of markers of bone turnover pyridinoline (PYR), N-terminal propeptide of type I procollagen (P1NP), osteocalcin (OC), and bone-specific alkaline phosphatase (BSAP) were measured.Results. The mean Z score (BMD) at the lumbar spine (L1-L4) in patients with JRA was -1.2 +/- 0.8. Z scores for total body and total hip were within 1 standard deviation of normal compared with healthy historical controls matched for age, sex, and race.Conclusion. BMD was normal for chronological age (defined as Z score >= 2.0) in African American children with JRA who had not previously been treated with corticosteroids. Further studies are needed on the effects of JRA on skeletal health in African American children

23. HELIN-SALMIVAARA A, HUUPPONEN R, VIRTANEN A, LAMMELA Jet KLAUKKA T: Frequent prescribing of drugs with potential gastrointestinal toxicity among continuous users of non-steroidal anti-inflammatory drugs, Eur.J.Clin.Pharmacol., Vol. 61(5-6), 425-431., 2005
    Organism:Centre for Pharmacotherapy Development, Box 55, FIN, 00301, Helsinki, Finland arjahelin-salmivaara@rohtofiFAU - Helin-Salmivaara, Arja
    Abstract:OBJECTIVE: A number of drugs used concurrently with non-steroidal anti-inflammatory drugs (NSAIDs) increase the risk of gastrointestinal (GI) haemorrhage. We studied the prescribing of NSAIDs with corticosteroids, oral anticoagulants or selective serotonin re-uptake inhibitors (SSRIs), as well as the use of gastroprotection among continuous and non-continuous users of NSAIDs in Finland. METHODS: Concurrent use of various drugs was analysed in a nested case-control study in a population-based cohort of NSAID users in 2000 using data in the National Prescription Database. RESULTS: Prescribing of any other drug with the potential to increase the risk of GI bleeding with NSAIDs was five times [5.2; 95% confidence interval (CI) 4.7-5.9] more common among continuous than non-continuous NSAID users, and the odds ratio for oral corticosteroids was 8.0 (95% CI 6.6-9.6). Of patients using continuous NSAIDs with oral corticosteroids, 73.3% had rheumatoid arthritis (RA). After excluding RA patients, the odds ratio remained high (4.5; 95% CI 3.3-6.1) and at the same level as for SSRIs (3.7; 3.1-4.4). Gastroprotective drugs were prescribed for 6.8% of the continuous users of NSAIDs alone, and for 20.4% of patients taking any of the studied drug combinations with NSAIDs. The continuous users of NSAIDs alone had gastroprotection 2.9 (2.5-3.3) times more often than other users of NSAIDs. With drug combinations (NSAID+corticosteroid, NSAID+SSRI, NSAID+anticoagulants), the use of gastroprotection did not differ from patients using lower amounts of NSAIDs. CONCLUSIONS: When prescribing NSAIDs, situations leading to habitual use should be avoided, potential complications due to clustering of risk factors recognised, and gastroprotection prescribed for patients with increased risk of GI haemorrhage

24. HERMAN CJ, DENTE JM, ALLEN Pet HUNT WC: Ethnic differences in the use of complementary and alternative therapies among adults with osteoarthritis, Prev.Chronic.Dis., Vol. 3(3), A80, 2006
    Organism:University of New Mexico Center on Aging, 1720 Louisiana NE, Suite 300, Albuquerque, NM 87110, USA cherman@saludunmeduFAU - Herman, Carla J
    Abstract:INTRODUCTION: The use of complementary and alternative medicine (CAM) in the United States has been rising steadily, especially among people with chronic conditions such as osteoarthritis. It has been suggested that ethnicity and acculturation may influence use of CAM. The purpose of this study was to assess the influence of ethnicity and acculturation on patterns of CAM use among Hispanic and non-Hispanic white adults with osteoarthritis. METHODS: We conducted interviews in person, in English or Spanish, using a 255-item survey. We randomly selected participants aged 18 to 84 years from patients at university-based primary care outpatient clinics who had been diagnosed with osteoarthritis during the previous year. Measures included prevalence and types of CAM use, sociodemographic factors, self-reported ethnicity, and degree of acculturation according to language use. RESULTS: The Hispanic (n = 218) and non-Hispanic white (n = 204) populations showed similar rates of overall current CAM use (65.5% Hispanic vs 67.8% NHW) at time of interview. However, although more Hispanics used oral herbs (P = .03) and magnets or copper jewelry (P = .03), more non-Hispanic whites used nutritional supplements (P < .001). Hispanics speaking primarily English mirrored patterns of CAM use among non-Hispanic whites. These effects persisted after controlling for age, sex, income, education, degree of disability, and disease duration. CONCLUSION: In this population, ethnicity was a significant influence on patterns of CAM use but did not affect overall rates of use. Some differences were more pronounced among Spanish-speaking Hispanics, reflecting the incorporation of folk or traditional remedies into their health care practices

25. HUSCHER D, MERKESDAL S, THIELE K, ZEIDLER H, SCHNEIDER Met ZINK A: Cost of illness in rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and systemic lupus erythematosus in Germany, Ann.Rheum.Dis., Vol. 65(9), 1175-1183., 2006
    Organism:German Rheumatism Research Centre, Berlin, GermanyFAU - Huscher, D
    Abstract:OBJECTIVE: To estimate and compare the direct and indirect costs of illness in rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis (PsA) and systemic lupus erythematosus (SLE), and to evaluate the effect of sex, disease duration and functional status on the various cost domains. METHODS: Data of outpatients, aged 18-65, with rheumatoid arthritis (n = 4351), ankylosing spondylitis (n = 827), PsA (n = 908) or SLE (n = 844), who were enrolled in the national database of the German collaborative arthritis centres in 2002, were analysed. Data on healthcare consumption, out-of-pocket expenses and productivity losses were derived from doctors and patients. For the calculation of indirect costs, the human capital approach (HCA) and the friction cost approach (FCA) were applied. RESULTS: Mean direct costs amounted to 4737 euros a year in rheumatoid arthritis, 3676 euros in ankylosing spondylitis, 3156 euros in PsA and 3191 euros in SLE. By using the HCA, total costs were calculated at 15,637 euros in rheumatoid arthritis, 13,513 euros in ankylosing spondylitis, 11,075 euros in PsA and 14,411 euros in SLE, whereas with the FCA the numbers were 7899 euros, 7204 euros, 5570 euros and 6518 euros, respectively. Costs increased with disease duration and were strongly dependent on functional status. In patients with the highest disability (<50% of full function), the total costs on applying the HCA were 34,915 euros in rheumatoid arthritis, 29,647 euros in alkylosing spondylitis, 37,440 euros in PsA and 32,296 euros in SLE. CONCLUSION: The costs of illness are high in all four diseases, with a strong effect of functional status on total costs. Indirect costs differ by the factor 3, based on whether the HCA or the FCA is used

26. JARVIS JN, PETTY HR, TANG Y, FRANK B, TESSIER PA, DOZMOROV I, JIANG K, KINDZELSKI A, CHEN Y, CADWELL C, TURNER M, MCGHEE JLet CENTOLA M: Evidence for chronic, peripheral activation of neutrophils in polyarticular juvenile rheumatoid arthritis, Arthritis Res.Ther., Vol. 8(5), R154, 2006
    Organism:ABSTRACT: Although strong epidemiologic evidence suggests an important role for adaptive immunity in the pathogenesis of polyarticular juvenile rheumatoid arthritis (JRA), there remain many aspects of the disease that suggest equally important contributions of the innate immune system. We used gene expression arrays and computer modeling to examine the function in neutrophils of 25 children with polyarticular JRA. Computer analysis identified 712 genes that were differentially expressed between patients and healthy controls. Computer-assisted analysis of the differentially expressed genes demonstrated functional connections linked to both IL-8 and IFNg regulated processes. Of special note is that the gene expression fingerprint of children with active JRA remained essentially unchanged even after children had responded to therapy. This result differed markedly from our previously reported work, in which gene expression profiles in buffy coats of children with polyarticular JRA reverted to normal after disease control was achieved pharmacologically. These findings suggest that JRA neutrophils remain in an activated state even during disease quiescence. Computer modeling of array data further demonstrated disruption of gene regulatory networks in clusters of genes modulated by IFNg and IL-8. These cytokines have previously been shown to independently regulate the frequency (IFNg) and amplitude (IL-8) of the oscillations of key metabolites in neutrophils, including NAD(P)H and superoxide ion. Using real-time, high-speed, single-cell photoimaging, we observed that 6/6 JRA patients displayed a characteristic defect in 12-23% of the neutrophils tested. Reagents known to induce only frequency fluctuations of NAD(P)H and superoxide ion induced both frequency and amplitude fluctuations in JRA neutrophils. This is a novel finding that was observed in children with both active (n=4) and inactive (n=2) JRA. A sub-population of polyarticular JRA neutrophils are in a chronic, activated state, a state that persists when the disease is well-controlled pharmacologically. Furthermore, polyarticular JRA neutrophils exhibit an intrinsic defect in the regulation of metabolic oscillations and superoxide ion production. Our data are consistent with the hypothesis that neutrophils play an essential role in the pathogenesis of polyarticular JRA

27. JONES OYet LOVELL DJ: Comparison of treatment-response criteria for juvenile idiopathic arthritis, Nat.Clin.Pract.Rheumatol., Vol. 2(9), 466-467., 2006
    Organism:Department of Pediatric Rheumatology of the Children's National Medical Center, George Washington University, Washington, DC 20010, USA oyjones@cnmcorgFAU - Jones, Olcay Y
    Abstract:

28. JONES OYet LOVELL DJ: Comparison of treatment-response criteria for juvenile idiopathic arthritis: Commentary|, 2006
    Organism:

29. KANEIDER NC, LEGER AJet KULIOPULOS A: Therapeutic targeting of molecules involved in leukocyte-endothelial cell interactions, FEBS J., Vol. 273(19), 4416-4424., 2006
    Organism:Molecular Oncology Research Institute, Tufts-New England Medical Center, Boston, MA, USAFAU - Kaneider, Nicole C
    Abstract:Inflammation is traditionally viewed as a physiological reaction to tissue injury. Leukocytes contribute to the inflammatory response by the secretion of cytotoxic and pro-inflammatory compounds, by phagocytotic activity and by targeted attack of foreign antigens. Leukocyte accumulation in tissues is important for the initial response to injury. However, the overzealous accumulation of leukocytes in tissues also contributes to a wide variety of diseases, such as atherosclerosis, chronic inflammatory bowel disease, rheumatoid arthritis, multiple sclerosis, vasculitis, systemic inflammatory response syndrome, juvenile diabetes and psoriasis. Many therapeutic interventions target immune cells after they have already migrated to the site of inflammation. This review addresses different therapeutic strategies, used to reduce or prevent leukocyte-endothelial cell interactions and communication, in order to limit the progression of inflammatory diseases

30. KIM SJ, JUNG KA, KIM JM, KWUN JDet KANG HJ: Arthroscopic synovectomy in wrists with advanced rheumatoid arthritis, Clin.Orthop.Relat Res., Vol. 449, 262-266., 2006
    Organism:Department of Orthopaedic Surgery, Yonsei University College of Medicine, Seoul, Korea sungjaikim@yumcyonseiackrFAU - Kim, Sung-Jae
    Abstract:We evaluated the effects of arthroscopic wrist synovectomy for patients with advanced rheumatoid arthritis. We retrospectively reviewed data for 11 patients with moderately advanced arthritis and joint space narrowing of 25% to 50% (Group A) treated from May 1993 to March 2000. We compared effects of these patients with effects of six patients with earlier disease and joint space narrowing of 0% to 25% (Group B). For Group A, the total modified Mayo wrist scores increased from an average of 26.36 points preoperatively to an average of 56.36 points postoperatively. Postoperative scores for pain and return to work status, and two components of the modified Mayo wrist score also showed differences compared with the respective preoperative scores. There was an increase in final followup scores in return to work status and total modified Mayo wrist scores. Group A showed larger differences than Group B. Our data suggest that arthroscopic synovectomy is a useful treatment for modest and moderate stages of rheumatoid arthritis. It is especially effective for decreasing pain and improving function, allowing patients to return to work. Level of Evidence: Therapeutic study, Level III. See the Guidelines for Authors for a complete description of levels of evidence

31. KONTTINEN L, HONKANEN V, UOTILA T, POLLANEN J, WAAHTERA M, ROMU M, PUOLAKKA K, VASALA M, KARJALAINEN A, LUUKKAINEN R, NORDSTROM DCet ROB-FIN sg: Biological treatment in rheumatic diseases: results from a longitudinal surveillance: adverse events, Rheumatol.Int. Berlin, Vol. 26, 916-922., 2006
    Organism:Univ Helsinki, Cent Hosp, Dept Internal Med, Haartmanink 4, Helsinki 00029, Finland Finland
    Abstract:The objective of this study was to assess the long-term safety and tolerability of biologicals in a clinical setting. Data on adverse events (AEs) have been collected over a 5-year period by means of detailed reports sent in to the National Register of Biological Treatment in Finland (ROB-FIN) and validated by information collected by the National Agency for Medicines. Three hundred and eight reports on AEs were filed, concerning a total of 248 patients; this corresponds to 17% of all patients in the ROB-FIN register who started biological treatments. Skin reactions and infections comprised 35 and 28% of the AEs, respectively. Some cases of tuberculosis and other infections, heart failure and demyelinating conditions were seen. Our work demonstrates no unexpected AEs in a Finnish patient cohort consisting of rheumatoid arthritis and spondylarthropathy patients, although many of them were treated with combination treatments in common use in Finland. Biological treatment appears safe in the hands of the Finnish rheumatologists

32. LANDGREN O, ENGELS EA, PFEIFFER RM, GRIDLEY G, MELLEMKJAER L, OLSEN JH, KERSTANN KF, WHEELER W, HEMMINKI K, LINET MSet GOLDIN LR: Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in Scandinavia, J.Natl.Cancer Inst., Vol. %20;98(18), 1321-1330., 2006
    Organism:Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892-7236, USA landgreo@mailnihgovFAU - Landgren, Ola
    Abstract:BACKGROUND: Personal history of autoimmune diseases is consistently associated with increased risk of non-Hodgkin lymphoma. In contrast, there are limited data on risk of Hodgkin lymphoma following autoimmune diseases and almost no data addressing whether there is a familial association between the conditions. METHODS: Using population-based linked registry data from Sweden and Denmark, 32 separate autoimmune and related conditions were identified from hospital diagnoses in 7476 case subjects with Hodgkin lymphoma, 18,573 matched control subjects, and more than 86,000 first-degree relatives of case and control subjects. We calculated odds ratios (ORs) and 95% confidence intervals (CIs) as measures of relative risks for each condition using logistic regression and also applied multivariable hierarchical regression models. All P values are two-sided. RESULTS: We found statistically significantly increased risks of Hodgkin lymphoma associated with personal histories of several autoimmune conditions, including rheumatoid arthritis (OR = 2.7, 95% CI = 1.9 to 4.0), systemic lupus erythematosus (OR = 5.8, 95% CI = 2.2 to 15.1), sarcoidosis (OR = 14.1, 95% CI = 5.4 to 36.8), and immune thrombocytopenic purpura (OR = infinity, P = .002). A statistically significant increase in risk of Hodgkin lymphoma was associated with family histories of sarcoidosis (OR = 1.8, 95% CI = 1.01 to 3.1) and ulcerative colitis (OR = 1.6, 95% CI = 1.02 to 2.6). CONCLUSIONS: Personal or family history of certain autoimmune conditions was strongly associated with increased risk of Hodgkin lymphoma. The association between both personal and family histories of sarcoidosis and a statistically significantly increased risk of Hodgkin lymphoma suggests shared susceptibility for these conditions

33. LEN CA, TERRERI MT, PUCCINI RF, WECHSLER R, SILVA EK, OLIVEIRA LM, BARBOSA CASSIA MP, PEDROSO GCet HILARIO MARIA OE: Development of a tool for early referral of children and adolescents with signs and symptoms suggestive of chronic arthropathy to pediatric rheumatology centers, Arthritis & Rheumatism, Vol. 55, 373-377., 2006
    Organism:Ave Brigadeiro Faria Lima 1826 cj 205, BR-01451001 Sao Paulo, SP, Brazil Brazil
    Abstract:Objective. To develop an easy, time-efficient tool to identify children and adolescents with signs and symptoms suggestive of chronic arthropathies, and to evaluate its interobserver reproducibility and reliability.Methods. The instrument used standardized techniques as required for the development of health-related instruments, targeting parents of apparently healthy children and adolescents ages 1-16 years. A multidisciplinary team was involved in the design of the instrument.Results. Each health professional generated 10-15 questions addressing musculoskeletal complaints that they considered to be the most relevant. A total of 60 questions were listed. During the reduction step, each health professional scored questions from 1 to 4 according to the question's relevance. The tool comprised 12 questions and was administered to the parents of 3 groups: patients with juvenile idiopathic arthritis (JIA; n = 48), children with diffuse musculoskeletal pain (n = 39), and a healthy control group (n = 42). The JIA group achieved the highest scores, followed by the diffuse musculoskeletal pain group and the control group. Nine (18.7%) of 48 patients with JIA and 2 (5.1%) of 39 children with musculoskeletal pain had a score of 5. The interobserver reproducibility was confirmed. All 12 questions were included in the final version of the instrument. We determined that children and adolescents with a score >= 5 should be referred for a rheumatologic evaluation (cluster analysis and logistic regression).Conclusion. Our questionnaire seems to be a useful tool for the early detection of musculoskeletal problems in children that may need a referral for a rheumatologic evaluation

34. LITTLE BS, WIXSON RLet STULBERG SD: Total hip arthroplasty with the porous-coated anatomic hip prosthesis: results at 11 to 18 years, J.Arthroplasty., Vol. 21(3), 338-343., 2006
    Organism:Indiana University Medical Center, Indianapolis, USAFAU - Little, Bryan S
    Abstract:The authors had previously reported good results with apparent fixation of a series of porous-coated anatomic hips at 2 to 4 years. In a larger series of 133 hips with porous-coated anatomic uncemented components, 91 hips in 82 patients were available for a follow-up of 11 years or greater. Although 3.1% of acetabular cups had migrated or had been revised at 2 to 4 years; at 11 to 18 years, there was 37% lysis, 21.7% migration, and 32.3% revision. For the femur at 2 to 4 years, there was 3.1% migration and 1.5% revision. At 11 to 18 years, there was 27.2% lysis (proximal only), 6.6% migration, and 3.2% revision. Survival analysis for migration or revision for the femoral was 94%, and for the acetabular component, 63%, deteriorating markedly after 10 years

35. LURATI A, PONTIKAKI I, TERUZZI B, DESIATI F, GERLONI V, GATTINARA M, CIMAZ Ret FANTINI F: A comparison of response criteria to evaluate therapeutic response in patients with juvenile idiopathic arthritis treated with methotrexate and/or anti-tumor necrosis factor alpha agents, Arthritis & Rheumatism, Vol. 54, 1602-1607., 2006
    Organism:Gaetano Pini Inst, Via G Pini 9, I-20122 Milan, Italy Italy
    Abstract:Objective. There are no validated criteria to evaluate clinical response in juvenile idiopathic arthritis (JIA). The purpose of this study was to compare 4 sets of criteria (2 from the American College of Rheumatology [ACR] and 2 from the European League Against Rheumatism [EULAR]) for clinical response evaluation in JIA patients treated with methotrexate and/or antitumor necrosis factor a drugs.Methods. Seventy-five patients with JIA were evaluated at baseline and after 6 months of therapy with second-line drugs. Mean age at study onset was 12.8 years (range 2-32.9 years). Diagnoses were systemic JIA (n = 16), rheumatoid factor-positive JIA (n = 5), rheumatoid factor-negative JIA (n = 9), persistent oligoarticular JIA (n = 10), extended oligoarticular JIA (n = 33), and psoriatic arthritis (n = 2). Clinical response was evaluated with the ACR Pediatric 30 criteria and the ACR 20% response criteria (ACR20), and with the EULAR Disease Activity Score (DAS) and 28-joint DAS (DAS28). Patients with EULAR criteria responses of "good" or "moderate" were classified as responders. Responders and nonresponders according to the different criteria were then compared.Results. For patients younge r than 16 years, Cohen's kappa varied between 0.51 and 0.72, with a good-to-excellent reproducibility index for all comparisons, except for the DAS28/ACR20 comparison. The best agreement was obtained by comparing the DAS and the ACR Pediatric 30. For patients older than 16 years, the reproducibility index was good or excellent in only 2 cases, i.e., comparing the DAS and the ACR Pediatric 30 and comparing the DAS and the DAS28 (as expected).Conclusion. Our study shows a good agreement overall for the different criteria tested. The highest concordance was observed between the DAS and the ACR Pediatric 30, the lowest between the DAS28 and the ACR20. Our data suggest that the ACR Pediatric 30 criteria can be used also in adult patients affected by JIA, and that the original DAS can be an alternative to the ACR Pediat ric 30 in both children and young adults with PA

36. MACKOOL RL, SHETYE P, GRAYSON Bet MCCARTHY JG: Distraction osteogenesis in a patient with juvenile arthritis, J.Craniofac.Surg., Vol. 17(2), 387-390., 2006
    Organism:The Institute of Reconstructive Plastic Surgery, New York, New YorkFAU - Mackool, Richard L
    Abstract:We present a 26-year-old patient with juvenile-onset arthritis, Alagille's syndrome, micrognathia, and progressive sleep apnea. Despite the presence of significant temporomandibular joint pathology, mandibular distraction was indicated to correct life-threatening sleep apnea. Before distraction, the patient had only 10 mm of maximal interincisal opening and bilateral temporomandibular joint symptomatology. After distraction, the patient's sleep apnea resolved. There was slight improvement in her maximal incisal opening (12 mm) with neither exacerbation nor improvement of her temporomandibular joint symptomatology

37. MANKI A, NISHIKOMORI R, NAKATA-HIZUME M, KUNITOMI T, TAKEI S, URAKAMI Tet MORISHIMA T: Tumor necrosis factor receptor-associated periodic syndrome mimicking systemic juvenile idiopathic arthritis|, 2006
    Organism:Background: We report two cases of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) in patients in whom systemic juvenile idiopathic arthritis (JIA) had initially been diagnosed or suspected. One patient, given a diagnosis of systemic JIA, was a 10-year-old boy who had presented with recurrent episodes of spike-fever, skin rash, arthritis, and myalgia. The other patient was his 7-year-old sister, who presented with similar symptoms and was suspected of having systemic JIA. Methods: Serum levels of soluble tumor necrosis factor receptor super family 1A (TNFRSF1A), TNF-alpha, Interleukin (IL) -6, and C-reactive protein (CRP) were measured in two siblings and JIA patients. In addition, DNA sequencing of the TNFRSF1A gene in two siblings was also performed. Results: A detailed family history showed that their mother had an episode of recurrent fever, arthritis, and myalgia with an increased serum CRP after the delivery of a daughter. Both siblings had serum levels of soluble TNFRSF1A that were below the normal reference range, and that did not reach a level corresponding to that of systemic JIA. On TNFRSF1A gene analysis, a single missense mutation resulting in C30Y was found in both siblings. Conclusions: Based on the clinical features and the TNFRSF1A mutation, both siblings were given a diagnosis of TRAPS. The serum levels of soluble TNFRSF1 A, measured along with the CRP level, may be a useful screening marker for differentiating TRAPS from systemic JIA. (c)2006 Japanese Society of Allergology

38. MANOLOVA Iet DANTCHEVA M: Antineutrophil cytoplasmic antibodies in Bulgarian patients with rheumatoid arthritis: characterization and clinical associations, Rheumatol.Int., Vol. 26(2), 107-114., 2005
    Organism:Laboratory of Clinical Immunology, Department of Molecular Biology and Immunology, University Hospital, Armeiska 11 Street, 6000 Stara Zagora, Bulgaria imanolova@mfuni-szbgFAU - Manolova, Irena
    Abstract:OBJECTIVE: The aim of this study was to study the prevalence, subspecificities, and immunoglobulin (Ig)G subclass distribution of antineutrophil cytoplasmic antibodies (ANCA) in 90 Bulgarian patients with rheumatoid arthritis (RA) and to investigate the clinical associations of ANCA in these patients. METHODS: The ANCA were detected by indirect immunofluorescence, while antigen specificities were determined by enzyme-linked immunosorbent assay (ELISA) directed against myeloperoxidase (MPO), proteinase 3 (PR3), bactericidal/permeability-increasing protein (BPI), lactoferrin (LF), leukocyte elastase (LE), and cathepsin G (CG). The IgG subclass reactivity of antibodies to BPI and LF was measured. RESULTS: Antineutrophil cytoplasmic antibodies were found in 18 RA patients. Only a P-ANCA fluorescence pattern was seen. Six sera reacted to BPI, five to LF, one to MPO, one to PR3, and one to CG by ELISA testing. Immunoglobulin-G1 was the predominant subclass for LF-ANCA, whereas IgG1/3 contributed mainly to BPI-ANCA. Compared to P-ANCA-negative RA patients, the P-ANCA-positive patients exhibited significantly higher inflammatory activity, as estimated by disease activity score, C-reactive protein, erythrocyte sedimentation rate, and higher levels of IgM rheumatoid factor. CONCLUSION: Twenty percent of Bulgarian patients with RA have P-ANCA in their sera. These antibodies are directed against variable antigen specificities, while ANCA positivity in RA reflects disease and inflammatory activity

39. METIVIER Het HASSON SM: Use of the faces pain scale to evaluate pain of a pediatric patient with pauciarticular juvenile rheumatoid arthritis, Physiother.Theory Pract., Vol. 22(2), 91-96., 2006
    Organism:Sletcher Allen Health Care, Sports and Orthopaedic Rehabilitation Center, Colchester, VT, USAFAU - Metivier, Heather
    Abstract:The Faces Pain Scale developed by Wong and Baker is a common assessment tool that uses cartoon-like faces to assess self reported pain in children. The purpose of this case report is to explore the appropriateness of the Faces Pain Scale as an outcome measure for a young child with pauciarticular juvenile rheumatoid arthritis. The patient was a four-year-old boy who had undergone a synovectomy on his right knee secondary to pauciarticular JRA. Each session the patient was asked to rate his pain using the Faces Pain Scale. The patient gainedfull knee range of motion and his functional mobility improved compared to initial visit. His subjective pain rating remained constant at "no hurt" throughout three weeks of visits. His functional mobility did not match his subjective rating of pain via the Faces Pain Scale. Further research is needed to determine the relationship of pain, stiffness, and function in children with rheumatic diseases

40. MIRKINSON LJ, NAGALE D, KADOM Net JONES OY: Erratum: Anakinra therapy in a child with systemic-onset juvenile rheumatoid arthritis after human herpesvirus 6 encephalitis (Journal of Clinical Rheumatology (2006) 12, (83-86))|, 2006
    Organism:

41. MONTANO-LOZA A, CZAJA AJ, CARPENTER HA, PIETTE A, MURPHY D, SHUMS Z, BURLINGAME Ret NORMAN GL: Frequency and significance of antibodies to cyclic citrullinated peptide in type 1 autoimmune hepatitis, Autoimmunity., Vol. 39(4), 341-348., 2006
    Organism:Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN 55905, USAFAU - Montano-Loza, Aldo
    Abstract:OBJECTIVES: Determine the frequency, clinical phenotype, and prognostic implications of antibodies against cyclic citrullinated peptides in patients with type 1 autoimmune hepatitis. METHODS: Three hundred and ninety-five serum samples from 179 patients were tested by enzyme-linked immunosorbent assay, and findings correlated with clinical and histological features, frequency of HLA DR3 and DR4, and treatment outcome. RESULTS: Twenty patients (11%) had antibodies against cyclic citrullinated peptides. Seropositivity was associated with a higher frequency of rheumatoid arthritis (RA) (25 vs. 0%, P < 0.001). Patients with antibodies against cyclic citrullinated peptides also had a significantly greater occurrence of histological cirrhosis at presentation (47 vs. 20%, P = 0.01) and death from hepatic failure than seronegative patients (25 vs. 9%, P = 0.04). Cirrhosis at presentation occurred more commonly in the patients with antibodies against cyclic citrullinated peptides and RA than in the other patients (100 vs. 21%, P = 0.005). CONCLUSIONS: Antibodies against cyclic citrullinated peptides occur in a subgroup of patients with type 1 autoimmune hepatitis who have a greater occurrence of cirrhosis at presentation and death from hepatic failure. Their presence with RA at accession characterizes a subgroup with cirrhosis

42. OKUDA Yet TAKASUGI K: Successful use of a humanized anti-interleukin-6 receptor antibody, tocilizumab, to treat amyloid A amyloidosis complicating juvenile idiopathic arthritis, Arthritis Rheum., Vol. 54(9), 2997-3000., 2006
    Organism:Center for Rheumatic Diseases, Dohgo Spa Hospital, Ehime, Japan
    Abstract:We report an excellent clinical response to treatment with a humanized anti-interleukin-6 receptor antibody, tocilizumab, in a patient with progressive amyloid A (AA) amyloidosis complicating very active juvenile idiopathic arthritis. Treatment with tocilizumab immediately normalized the serum AA (SAA) level, and subsequently all of the clinical symptoms of AA amyloidosis disappeared. Serial gastrointestinal biopsy specimens showed marked lasting regression of AA protein deposits. The patient's functional ability score improved dramatically, she maintains her mobility, and she has regained her previous quality of life. Tocilizumab appears to have an excellent ability to suppress SAA levels and could therefore be an important therapeutic strategy in AA amyloidosis secondary to rheumatic diseases

43. PANCEWICZ SA, WIELGAT P, HERMANOWSKA-SZPAKOWICZ T, KONDRUSIK M, ZAJKOWSKA J, GRYGORCZUK S, POPKO Jet ZWIERZ K: Activity of lysosomal exoglycosidases in the serum of patients with chronic Lyme arthritis, Int.J.Med.Microbiol., Vol. 296 Suppl 40, 280-282., 2006
    Organism:Department of Infectious Diseases and Neuroinfections, Medical University of Bialystok, Zurawia 14, PL-15540 Bialystok, Poland spancewicz@interiaplFAU - Pancewicz, Slawomir A
    Abstract:To examine the role of lysosomal exoglycosidases in the pathogenesis of Lyme arthritis we studied a group of 18 patients aged 18-72 (mean: 46 yr) diagnosed with chronic arthritis in the course of borreliosis. The control group was composed of 20 healthy volunteers (health service employees) aged 25-65 (mean: 45 yr) with no detectable serum anti-Borrelia burgdorferi antibodies. We found that N-acetyl-beta-d-hexosaminidase (HEX) was significantly increased and beta-galactosidase and alpha-mannosidase also showed an increase in Lyme arthritis patients compared to healthy persons and normalised after treatment with doxycycline. Our results suggest that HEX is a sensitive enzymatic marker of Lyme arthritis and it may be used to monitor the course of the disease and the efficiency of treatment

44. PAVLOV-DOLIJANOVIC S, DAMJANOV N, OSTOJIC P, SUS c, STOJANOVIC R, GACIC Det GRDINIC A: The prognostic value of nailfold capillary changes for the development of connective tissue disease in children and adolescents with primary Raynaud phenomenon: A follow-up study of 250 patients|, 2006
    Organism:To assess the prognostic value of capillaroscopy findings for the development of connective tissue disease in children and adolescents with Raynaud phenomenon, we followed up a group of 250 (mean age 15 years) for 1 to 6 years after the first capillaroscopy was performed. Every 6 months they were screened for signs and symptoms of connective tissue disease. Analysis was performed on capillary changes registered 6 months before the development of connective tissue disease. Capillary changes were classified into three types: normal, nonspecific, and sclerodermatous. At the end of the follow-up period, 191 (76%) subjects had primary Raynaud phenomenon, 27 (10.8%) were d iagnosed as having undifferentiated connective tissue disease, and 32 (12.8%) fulfilled the criteria for a diagnosis of a specific connective tissue disease. Systemic lupus erythematosus was found in nine (3.6%) patients, rheumatoid arthritis in 10 (4%) patients (six of them with juvenile onset rheumatoid arthritis), and scleroderma spectrum disorders in 13 (5.2%). The mean time for the evolution of Raynaud phenomenon into undifferentiated connective tissue disease or a form of the disease was 2 years. Most of the subjects with primary Raynaud phenomenon (173/191, 91%), undifferentiated connective tissue disease (22/27, 81%), juvenile onset rheumatoid arthritis/rheumatoid arthritis (7/10, 70%), and systemic lupus erythematosus (6/9, 67%) had normal capillary findings. Nonspecific capillary changes occurred in 3 of 10 (30%) patients with rheumatoid arthritis, 2 of 9 (22%) with systemic lupus erythematosus, 4 of 27 (15%) with undifferentiated connective tissue disease, and 18 of 191 (9%) with primary Raynaud phenomenon. Of all the subjects, only 10 (4%) showed sclerodermatous disease type capillary changes 6 months before the expression of a particular disease: eight (62%) of these developed scleroderma spectrum disorders, one expressed systemic lupus erythematosus, and one had undifferentiated connective tissue disease. We concluded that there were no specific capillary changes predictive for future dev elopment of systemic lupus erythematosus, juvenile onset rheumatoid arthritis/rheumatoid arthritis, and undifferentiated connective tissue disease in children and adolescents with Raynaud phenomenon. Most of our study subjects with Raynaud phenomenon who developed these diseases had normal capillary findings or nonspecific changes. Children and adolescents who developed scleroderma spectrum disorders showed a sclerodermatous type of capillary changes 6 months before the expression of the disease, indicating that this type of capillary changes in children and adolescents with Raynaud phenomenon highly correlated with further development of scleroderma spectrum disorders. (c) 2006 The Authors

45. PHILLIPS B: Towards evidence based medicine for paediatricians, Arch.Dis.Child., Vol. 91, 532, 2006
    Organism:Univ Oxford, Warneford Hosp, Dept Psychiat, Ctr Evidence Based Med, Oxford OX3 7JX, UK UK
    Abstract:

46. PIPINOS I, I, HOPP R, EDWARDS WDet RADIO SJ: Giant-cell temporal arteritis in a 17-year-old male, Journal of Vascular Surgery, Vol. 43, 1053-1055., 2006
    Organism:Creighton Univ, Med Ctr, Dept Pediat, 601 N 30th St,Suite 6820, Omaha, NE 68131 USA USA
    Abstract:Temporal arteritis, particularly in its classic form, is exceedingly rare in individuals < 50 years old. We report the youngest case of biopsy-proven giant cell temporal arteritis. A 17-year-old male presented with a progressively expanding and pulsatile but otherwise asymptomatic mass in his forehead. The patient's medical history was significant for uveitis since the age of 3, and severe allergic rhinitis, mild asthma, and juvenile rheumatoid arthritis as a young adolescent. Admission laboratory values included a mildly elevated erythrocyte sedimentation rate and C-reactive protein level. A computed tomography evaluation demonstrated aneurysmal degeneration of the frontal branch of the right superficial temporal artery and confirmed no other cerebrovascular changes. Histologically, the aneurysmal arterial segment demonstrated subacute temporal arteritis. The arterial wall had a primarily lymphoplasmacytic infiltrate with rare giant cells and focally marked medial destruction. Additionally, severely obstructive intimal hyperplasia with chronic adventitial and periadvential dense fibrosis was noted. The diagnosis of classic giant cell temporal arteritis was established front the biopsy result. Postoperatively, the patient was treated with prednisone for 3 months. Three years after surgery, the patient remains well and reports no recurrence of temporal artery disease

47. PIZZUTILLO PD: Infections and Inflammatory Conditions of the Cervical Spine in Children, Instr.Course Lect., Vol. 55, 655-659., 2006
    Organism:St Christopher's Hospital for Children, Philadelphia, Pennsylvania
    Abstract:Because infections and inflammatory disorders of the cervical spine are uncommon in children and adolescents, diagnosis and treatment are frequently delayed. Intractable pain, limitation of neck motion, and neurologic compromise may occur as the result of these pathologic processes. It is important to identify these symptoms for early diagnosis and treatment of conditions such as bacterial and tuberculous infections, intervertebral disk calcification, juvenile rheumatoid arthritis, and Grisel's syndrome

48. REIFF A, LOVELL DJ, VAN ADELSBERG J, KISS MARIA HB, GOODMAN S, ZAVALER MF, CHEN P, BOLOGNESE JA, CAVANAUGH PF, REICIN ASet GIANNINI EH: Evaluation of the comparative efficacy and tolerability of rofecoxib and naproxen in children and adolescents with juvenile rheumatoid arthritis: A 12-week randomized controlled clinical trial with a 52-week open-label extension, Rinsho Ganka, Vol. 33, 985-995., 2006
    Organism:Merck and Co Inc, Merck Res Labs, RY34-B280,POB 2000, Rahway, NJ 07065 USA USA
    Abstract:Objective. To compare the safety and efficacy of rofecoxib* to naproxen for the treatment of juvenile rheumatoid arthritis (JRA).Methods. This was a 12-week, multicenter, randomized, double-blind, double-dummy, active comparator-controlled, non-inferiority study with a prespecified 52-week open-label active comparator-controlled extension. Children (ages 2-11 yrs) and adol escents (ages 12-17 yrs) received lower-dose (LD)rofecoxib [0.3 mg/kg/day up to 12.5 mg/day (base study only)]; or higher-dose (HD)-rofecoxib (0.6 mg/kg/day up to 25 mg/day) or naproxen 15 mg/kg/day as oral suspensions. Adolescents received daily rofecoxib (LD) 12.5 (base study only) or (HD) 25 mg, or naproxen 15 mg/kg/day (maximum 1000 mg/day) as tablets. The primary endpoint was the time-weighted average proportion of patients meeting the American College of Rheumatology Pediatric-30 (ACR Pedi 30) response criteria. A prespecified bound for the 95% confidence interval for the ratio of the percentage of ACR Pedi 30 responders was used to assess non-inferiority of treatment response between groups. Safety was assessed throughout the study.Results. A total of 310 patients ages 2-17 years (181 <= age 11) were randomized to receive LD-rofecoxib (N = 109), HD-rofecoxib (N = 100), or naproxen (N = 101). The ACR Pedi 30 response rates following 12 weeks of treatment were 46.2%, 54.5%, and 55.1%, respectively. The relative rates of response compared to naproxen were 0.81 (95% CI 0.61, 1.07) and 0.98 (95% CI 0.76, 1.26) for

49. RKAIN H, ALLALI F, JROUNDI Iet HAJJAJ-HASSOUNI N: Socioeconomic impact of rheumatoid arthritis in Morocco, Joint Bone Spine., Vol. 73(3), 278-283., 2006
    Organism:Rheumatology B Department, El Ayachi Hospital, Rabat-Sale Teaching Hospital, Sale, Morocco hananrkain@yahoofrFAU - Rkain, Hanan
    Abstract:OBJECTIVE: To estimate the socioeconomic impact of rheumatoid arthritis (RA) in Morocco. MATERIALS AND METHODS: We identified 100 consecutive patients (88 women and 12 men) with RA receiving follow-up either at a teaching hospital or from office-based physicians. For each patient, we recorded direct costs, indirect costs (productivity losses), and intangible costs (deterioration in the social domain of quality of life). RESULTS: Mean age at symptom onset was 31+/- 13.6 years and mean disease duration was 12.8 +/- 7.8 years. RA-related expenses caused financial difficulties for 90% of patients, resulting in poor treatment compliance (61% of cases) and school absenteeism in the children (19% of cases). Of the 34 patients who had paid jobs at symptom onset, 65% stopped working, 6.9 years on average after the diagnosis. Older age, male gender, and a physically strenuous job were associated with stopping work. Six women (10% of married patients) divorced because of their disease. Sexual problems were reported by 67% of patients. The ability to perform domestic chores was affected in 84% of cases and participation in leisure activities in 46% of cases. CONCLUSION: RA has a major socioeconomic impact on affected families. In addition to the disease itself, the low socioeconomic status of many patients and the inadequate social welfare and health insurance systems contribute to the burden

50. RODRIGUEZ-MAHOU M, LOPEZ-LONGO FJ, SANCHEZ-RAMON S, ESTECHA A, GARCIA-SEGOVIA A, RODRIGUEZ-MOLINA JJ, CARRENO Let FERNANDEZ-CRUZ E: Association of anti-cyclic citrullinated peptide and anti-Sa/citrullinated vimentin autoantibodies in rheumatoid arthritis, Arthritis Rheum., Vol. 55(4), 657-661., 2006
    Organism:Hospital General Universitario Gregorio Maranon, Madrid, Spain mrodriguezmahgugm@saludmadridorgFAU - Rodriguez-Mahou, Margarita
    Abstract:

51. ROZKOVA D, HORVATH R, BARTUNKOVA Jet SPISEK R: Glucocorticoids severely impair differentiation and antigen presenting function of dendritic cells despite upregulation of Toll-like receptors, Clin.Immunol., Vol. 120(3), 260-271., 2006
    Organism:Institute of Immunology, Charles University, 2nd Medical School, V Uvalu 84, Prague 5, Czech RepublicFAU - Rozkova, Daniela
    Abstract:Glucocorticoids (GCs) are widely used as anti-inflammatory and immunosuppressive agents. Effects of GC have mainly been attributed to the suppression of T cells. Recently, several studies have indicated the role of dendritic cells (DC) in GC-mediated immunosuppression. We investigated the effect of GC on characteristics of DC. Given the crucial role of Toll-like receptor (TLR) triggering for the initiation of DC maturation program, we analyzed the expression of TLR2, 3, 4 by GC-treated DC. To extend our in vitro findings, we analyzed the distribution of DC subsets in the blood of patients treated with high-dose corticosteroids. DC differentiation in presence of GC was skewed to a qualitatively distinct population incapable of inducing an efficient immune response, whereas GC presence during the process of maturation significantly reduced DC IL-12 p70 and TNF production and T cell stimulatory function. Despite the fact that GC increased expression of TLR2, 3 and 4 on DC, their stimulation with TLR-derived signals did not induce maturation. Administration of high-dose GC to the patients with systemic autoimmunity induced a decrease of circulating myeloid DC and abrogated plasmacytoid DC. These findings provide further insights into the mechanisms of GC immunosuppressive functions and reveal additional mechanisms of their therapeutic efficiency

52. SANDBORG C, HOLMES TH, LEE T, BIEDERMAN K, BLOCH DA, EMERY H, MCCURDY Det MELLINS ED: Candidate Early Predictors for Progression to Joint Damage in Systemic Juvenile Idiopathic Arthritis, J.Rheumatol., Vol. ., 2006
    Organism:OBJECTIVE: To assess if joint damage at 2 years after diagnosis in patients with systemic juvenile idiopathic arthritis (SJIA) can be predicted by clinical or laboratory features assessed up to 3 or 6 months after diagnosis. METHODS: Medical records from 70 children were retrospectively reviewed. The primary outcome measure was presence of joint damage at 2 years after diagnosis (JD2) as defined by presence of erosions or fusion in one or more joints. Potential predictor variables for JD2 in the first 3 and 6 months after diagnosis consisted of the highest observed white blood cell count, platelet count, erythrocyte sedimentation rate, active joint count, and presence of symptomatic pulmonary or cardiac disease or macrophage activation syndrome, and treatment data. RESULTS: The outcome of interest, JD2, was identified in 15/70 patients. Classification-tree analysis identified a pair of variables (highest observed platelet count and number of active joints) measured within the first 3 months after diagnosis that together predicted progression to JD2 with an estimated sensitivity of 87%, specificity of 82%, and positive predictive value of 57%. Multivariate logistic regression analyses at 3 months found that higher quantities of joints with active arthritis and early use of methotrexate (MTX) were factors significantly associated with increased odds of progression to JD2 (active joints odds ratio = 1.08, 95% CI 1.00-1.16, p = 0.04; MTX OR = 11.85, 95% CI 1.89-74.26, p = 0.01). Unsupervised cluster analysis identified 2 major phenotypes of patients at 3 months characterized by different ages at onset, acute phase markers, active joint counts, and presence of serositis. These phenotypes differed 3-fold in proportion of subjects progressing to JD2 (p < 0.05). CONCLUSION: By 3 months after diagnosis, a clinical phenotype based on active joint count and platelet count may be prognostic of an increased risk of progression to JD2. Use of corticosteroids did not appear to change the risk of joint damage. In contrast, the presence of serositis appeared to be associated with decreased risk of joint damage

53. SCHMELING Het HORNEFF G: Tumour necrosis factor alpha promoter polymorphisms and etanercept therapy in juvenile idiopathic arthritis, Rheumatol.Int., Vol. ., 2006
    Organism:Department of Pediatrics, Martin-Luther University Halle-Wittenberg, 06097, Halle, Germany, heinrikeschmeling@medizinuni-hallede
    Abstract:The objective of this study was to investigate the influence of TNF-alpha promoter alleles on clinical response to etanercept therapy in JIA. TNF-alpha promoter polymorphisms at positions -163, -238, -244, -308, -376 were determined in 137 JIA patients treated with etanercept for at least 3 months. A PCR fragment of about 500 bp of the TNF gene promoter was amplified. Polymorphisms were detected by a single sequencing procedure. Patients with the genotype -308GG achieved an ACR-JRA 30 response at month 6 more frequently than patients with the genotype -308GA or AA. This was already notable at month 3 of therapy. This difference in the total patient group is attributable to the JIA subgroup with rheumatoid factor negative polyarthritis. In this subgroup, patients with the -308GG genotype achieved an ACR-JRA 30 response more frequently than those with the -308GA or AA genotype (84 vs. 33% at months three, P < 0.01, 93 vs. 67% at months six, P < 0.05). There was no influence of the -238 TNF-alpha promoter alleles on clinical response. The rare alleles at position -376 or at positions -163 and -244 were too infrequent. There is an association between TNF gene promoter polymorphisms and response to etanercept in rheumatoid factor negative polyarticular JIA

54. SEYAHI E, UGURLU S, CUMALI R, BALCI H, SEYAHI N, YURDAKUL Set YAZICI H: Atherosclerosis in Takayasu arteritis, Ann.Rheum.Dis., Vol. 65(9), 1202-1207., 2006
    Organism:Division of Rheumatology, Department of Medicine, Cerrahpasa Medical Faculty, Istanbul University, Aksaray Istanbul, TurkeyFAU - Seyahi, E
    Abstract:OBJECTIVE: Chronic inflammatory diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis are associated with accelerated atherosclerosis. We hypothesised that atherosclerosis may also be increased in Takayasu arteritis. METHODS: The frequency of atherosclerotic plaques and the intima-media thickness (IMT) were investigated in 30 female patients with Takayasu arteritis (mean age (standard deviation), 35.4 (8.0) years), along with 45 sex-matched and age-matched patients with SLE (37.4 (6.8)) and 50 healthy controls (38.2 (5.7)). Plaques were scanned and IMT was measured at both sides of the common carotids, carotid bulb, and internal and external carotid arteries by B-mode ultrasonography. Traditional risk factors for atherosclerosis were also assessed. RESULTS: Most of the atherosclerotic risk factors were comparable between patients with Takayasu arteritis and SLE. More atherosclerotic plaques were observed among patients with Takayasu arteritis (8/30; 27%) and those with SLE (8/45; 18%) than among the healthy controls (1/50; 2%; p = 0.005). Logistic regression analyses showed that the presence of a plaque was associated only with age in both Takayasu arteritis and SLE (p = 0.04 and 0.02, respectively). The mean overall IMT was significantly higher among patients with Takayasu arteritis (0.95+/-0.31 mm) than among the patients with SLE (0.58+/-0.10 mm) and the healthy controls (0.59+/-0.08 mm; p<0.001). CONCLUSION: Patients with Takayasu arteritis have a high rate of atherosclerotic plaques, at least as frequent as that observed among patients with SLE

55. SINGH-GREWAL D, SCHNEIDER R, BAYER Net FELDMAN BM: Predictors of disease course and remission in systemic juvenile idiopathic arthritis - Significance of early clinical and laboratory features, Arthritis & Rheumatism, Vol. 54, 1595-1601., 2006
    Organism:Hosp Sick Children, Div Rheumatol, 555 Univ Ave, Toronto, ON M5G 1X8, Canada Canada
    Abstract:Objective. To determine whether the disease course in systemic juvenile idiopathic arthritis (JIA) can be characterized as monophasic, polycyclic, or persistent, and to determine whether early clinical and laboratory characteristics can be used to predict the disease course and time to remission.Methods. Forty-five children with systemic JIA diagnosed between 1996 and 2000 were followed up with a standardized data collection protocol, including data on clinical and laboratory features (mean followup 4.9 years). Disease was considered inactive if the clinical and laboratory features were normal. Three definitions of remission were applied to classify disease course. P redictors of disease course were evaluated using multiple logistic regression. Predictors of time to remission were evaluated using Cox proportional hazards regression.Results. When applying a definition of remission requiring inactive disease while not receiving any medications for a period of 3 months, 42.2%, 6.7%, and 51.1% of the patients were classified as having monophasic, polycyclic, and persistent disease, respectively. Fever and active arthritis at 3 months (R-2 = 0.42, area under the receiver operating characteristics curve [AUC] = 0.76) and an erythrocyte sedimentation rate (ESR) > 26 mm/hour and corticosteroid use at 6 months (R-2 = 0.49, AUC = 0.92) were predictive of a non-monophasic course. Absence of active arthritis, an ESR of < 26 mm/hour, and no requirement for corticosteroid therapy at 3 and 6 months were predictors of an earlier time to remission.Conclusion. The disease course in systemic JIA can be characterized as monophasic, polycyclic, or persistent using a definition of remission requiring 3 months of inactive disease while not receiving any therapy. Features at 3 and 6 months are predictive of the disease course and time to remission

56. STABILE A, BERTONI B, ANSUINI V, LA T, I, SALLI Aet RIGANTE D: The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age, Eur.Rev.Med.Pharmacol.Sci., Vol. 10(2), 53-59., 2006
    Organism:Department of Pediatric Sciences, Catholic University Sacro Cuore, Rome, Italy astabile@rmunicattitFAU - Stabile, A
    Abstract:Macrophage activation syndrome is a rare and potentially fatal complication of many childhood pathological settings, most frequently reported in systemic onset-juvenile idiopathic arthritis. The disruption of the macrophage-lymphocyte interaction leads to uncontrolled proliferation of highly activated macrophages and T lymphocytes. The syndrome comprises a heterogeneous group of disorders featuring sepsis-like characteristics typically combined with impaired function of natural killer cells and cytotoxic T-cells, haemophagocytosis and hypercytokinemia, often resulting in fatal multiple organ failure. The clinical picture shows high grade fever, hepatosplenomegaly, pancytopenia, lymphoadenopathy, central nervous system involvement and consumptive coagulopathy. Macrophage activation syndrome is associated with high mortality: even though diagnostic criteria have been proposed, definite diagnosis can be a challenge for clinicians, especially in early phases. There is no standardized therapeutic protocol for macrophage activation syndrome, but it is widely recognized that aggressive treatment strategies might strongly influence prognosis. First line-therapy is usually represented by parenteral administration of high dose-corticosteroids, whilst cyclosporine is added in the steroid-resistant cases. In this paper we provide clinical clues and summarize the most recent studies about pathophysiology and management suggestions for macrophage activation syndrome

57. STEIN MB, COX BJ, AFIFI TO, BELIK SLet SAREEN J: Does co-morbid depressive illness magnify the impact of chronic physical illness? A population-based perspective, Psychol.Med., Vol. 36(5), 587-596., 2006
    Organism:Department of Psychiatry, University of California San Diego, La Jolla, CA 92093-0985, USA mstein@ucsdeduFAU - Stein, Murray B
    Abstract:OBJECTIVE: To examine the relative and combined impact of depressive and chronic physical conditions on functional status and health-care use in the general population. METHOD: Canadian, representative, population-based cross-sectional survey (n=130,880). Major depressive disorder (MDD) in the past 12 months was assessed by structured interview, and physical disorders, activity reduction, role impairment and work absence by self-report. The relative impact of MDD and six common chronic physical illnesses (asthma, arthritis, back problems, chronic obstructive pulmonary disease, heart disease and diabetes) was estimated using multivariate regression, adjusting for sociodemographic characteristics and overall chronic physical illness burden. RESULTS: After adjusting for sociodemographic characteristics, alcohol dependence and chronic physical illness burden, the presence of co-morbid MDD was associated with significantly greater (approximately double the) likelihood of health-care utilization and increased functional disability and work absence compared to the presence of a chronic physical illness without co-morbid MDD. This impact of MDD was seen across each of the six chronic physical illnesses examined in this study, with the strongest associations seen for work absence. CONCLUSIONS: These observations confirm prior findings of a strong association at the population level between major depression and health-care use and role impairment among persons with chronic physical disorders. They also point to the significant impact of co-morbid major depression on health-care seeking, disability and work absence in persons with chronic physical illness, underscoring the need for greater efforts to design and test the impact of detection and treatment programs for such individuals

58. TAKEYAMA J, SATO A, NAKANO K, ABUKAWA D, ICHINOHAZAMA Ret IMAIZUMI M: Epstein-Barr Virus Associated Hodgkin Lymphoma in a 9-Year-Old Girl Receiving Long-Term Methotrexate Therapy for Juvenile Idiopathic Arthritis, J.Pediatr.Hematol.Oncol., Vol. 28(9), 622-624., 2006
    Organism:Departments of *Pathology daggerHematology and Oncology double daggerPediatrics, Miyagi Children's Hospital section signDepartment of Hematologic Pathology, Tohoku University School of Medicine, Sendai, Japan
    Abstract:We describe a case of Hodgkin lymphoma developing in a 9-year-old girl with polyarticular, rheumatoid factor-positive juvenile idiopathic arthritis treated with methotrexate (MTX), prednisone, and naproxen for 5 years. Pathologic and molecular analyses revealed that the Hodgkin cells contained Epstein-Barr virus and the viral DNA was monoclonal. She achieved complete remission after MTX withdrawal, chemotherapy, and radiation. To the best of my knowledge, this is the sixth report of Hodgkin lymphoma in patients with juvenile idiopathic arthritis receiving low dose MTX therapy

59. TAN X, CAI D, WU Y, LIU B, RONG L, CHEN Zet ZHAO Q: Comparative analysis of serum proteomes: discovery of proteins associated with osteonecrosis of the femoral head, Transl.Res., Vol. 148(3), 114-119., 2006
    Organism:Department of Orthopedics, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China tanxiaoyun@126comFAU - Tan, Xiaoyun
    Abstract:No means exist to evaluate the activity status, turnover, and prognosis of idiopathic osteonecrosis of the femoral head (IONFH) except for X-ray evidence of segmental collapse as a very good marker for prognosis. Moreover, the only current method for diagnosis of this disease is through physical examination and diagnostic imaging results, and no serum biochemical markers exist. A comparative analysis of serum proteomes was performed to discover proteins associated with osteonecrosis of the femoral head. Two-dimensional electrophoresis (2-DE) patterns of human sera from 10 patients with IONFH and 10 normal subjects were analyzed. The differentially expressed spots were identified by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS), and 7 proteins were found. The expression levels of tissue-type plasminogen activator (t-PA), bone-carboxyglutamate protein (BGP), c-sis, and an unknown protein were downregulated in the sera of patients with IONFH, whereas the other 3 proteins, including plasminogen activator inhibitor type 1 (PAI-1), crosslaps, and anti-p53 antibody, were upregulated. To examine their applicability as diagnostic markers, levels of the 6 identified proteins in serum were validated from patients with IONFH, osteoarthritis (OA), rheumatoid arthritis (RA), and fracture using the enzyme-linked immunosorbent assay (ELISA) method. It was found that only serum levels of t-PA, PAI-1, crosslaps, and anti-p53 antibody in patients with IONFH were always significantly different from those in patients with OA, RA, and fracture. These results suggest that serum levels of t-PA, PAI-1, crosslaps, and anti-p53 antibody could be used as noninvasive diagnostic biomarkers for IONFH

60. TSE SHIRLEY ML, LAXER RM, BABYN PSet DORIA AS: Radiologic improvement of juvenile idiopathic arthritis-enthesitis-related arthritis following anti-tumor necrosis factor-alpha blockade with etanercept, Rinsho Ganka, Vol. 33, 1186-1188., 2006
    Organism:Hosp Sick Children, Div Rheumatol, Dept Pediat, 555 Univ Ave, Toronto, ON M5G 1X8, Canada Canada
    Abstract:Children with juvenile spondyloarthropathy, classified as enthesitis-related arthritis (ERA) under the International League of Associations for Rheumatology classification of juvenile idiopathic arthritis, usually experience both arthritis and enthesitis. Treatment with anti-tumor necrosis factor-alpha (TNF-alpha) agents has resulted in dramatic clinical improvement. Radiologic imaging methods useful in the detection, diagnosis, and grading of arthritis and enthesitis include magnetic resonance imaging and ultrasonography. We describe the serial radiologic improvements of arthritis and enthesitis in a child with ERA in response to treatment with the anti-TNF-alpha agent etanercept

61. TSURUMARU M, KAWASAKI E, IDA H, MIGITA K, MORIUCHI A, FUKUSHIMA K, FUKUSHIMA T, ABIRU N, YAMASAKI H, NOSO S, IKEGAMI H, AWATA T, SASAKI Het EGUCHI K: Evidence for the role of small ubiquitin-like modifier 4 as a general autoimmunity locus in the Japanese population, J.Clin.Endocrinol.Metab., Vol. 91(8), 3138-3143., 2006
    Organism:Department of Metabolism/Diabetes and Clinical Nutrition, Nagasaki University Hospital of Medicine and Dentistry, 1-7-1 Sakamoto, Nagasaki 852-8501, JapanFAU - Tsurumaru, Masako
    Abstract:CONTEXT: Recently, an association of a single nucleotide polymorphism, 163A>G encoding M55V, in the gene SUMO4, which has been shown to be a negative feedback regulator for nuclear factor kappaB, has been reported in type 1 diabetes. OBJECTIVE: To establish whether SUMO4 locus contributes to the genetic susceptibility to other autoimmune disorders, a case-control analysis was carried out using genomic DNA from type 1 diabetes, autoimmune thyroid disease (AITD), rheumatoid arthritis (RA), and primary Sjogren's syndrome. SUBJECTS: A total of 1480 samples, including 929 cases (411 patients with type 1 diabetes, 292 AITD, 172 RA, and 54 primary Sjogren's syndrome) and 551 healthy control subjects of Japanese origin participated in the study. METHODS: The 163A>G (rs237025, M55V) polymorphism of SUMO4 was genotyped. RESULTS: SUMO4 M55V variant was associated not only with type 1 diabetes [odds ratio (OR), 1.42; 95% confidence interval (CI), 1.09-1.84; P = 0.0072], but also with increased risk of other autoimmune diseases, AITD (OR, 1.52; 95% CI, 1.14-2.03; P = 0.0041) and RA without amyloidosis (OR, 1.53; 95% CI, 1.65-2.24; P = 0.027), but not primary Sjogren's syndrome. Furthermore, the association of SUMO4 M55V variant was stronger in type 1 diabetic patients complicated with AITD (OR, 1.62; 95% CI, 1.06-2.47; P = 0.023) and in patients who have neither type 1 diabetes-susceptible class II HLA, DRB1*0405 nor DRB1*0901 (OR, 2.28; 95% CI, 1.34-3.87; P = 0.0018). CONCLUSIONS: These results indicate that the SUMO4 is a more common autoimmune disease gene and a supplementary risk factor to type 1 diabetes in conjunction with class II HLA

62. URAKAMI T, MANKI A, INOUE T, ODA M, TANAKA Het MORSHIMA T: Clinical significance of decreased serum concentration of cartilage oligomeric matrix protein in systemic juvenile idiopathic arthritis, Rinsho Ganka, Vol. 33, 996-1000., 2006
    Organism:Okayama Univ, Grad Sch Med Dent and Pharmaceut Sci, Dept Pediat, 2-5-1 Shikata Cho, Okayama 7008558, Japan Japan
    Abstract:Objective. Serum cartilage oligomeric matrix protein (COMP) concentration is elevated in patients with early osteoarthritis and early rheumatoid arthritis, and may be a biomarker of cartilage turnover. We investigated whether serum COMP concentration could be a clinically significant marker of arthritis and/or growth impairment in juvenile idiopathic arthritis (JIA).Methods. Specimens were collected from 82 healthy blood donors under 22 years of age with no growth impairment who served as healthy controls, and from 24 patients with JIA (6 with oligoarthritis, 10 with polyarthritis, 8 with systemic JIA) presenting with active arthritis. Serum COMP concentration was de termined using a human COMP assay kit.Results. Serum COMP concentrations were significantly higher in all controls less than 16 years of age than in all controls aged 16 years or older. There was a significant negative correlation between serum COMP concentration and serum C-reactive protein in patients with JIA. Serum COMP concentrations in patients with systemic JIA were significantly lower than those in controls.Conclusion. Serum COMP concentrations in healthy children reflected increased cartilage turnover in the growth phase. Because the serum COMP concentration was decreased in cases of systemic JIA in which growth impairment was pronounced, the systemic inflammation Occurring in systemic JIA may have an effect on cartilage turnover, which plays an important role in growth. Serum COMP concentration may prove to be a marker that indicates growth impairment in systemic JIA

63. ZHA Q, HE Y, LU Yet LU A: Relationship between platelet counts and cartilage erosion in 436 cases of rheumatoid arthritis, Clin.Chim.Acta., Vol. 371(1-2), 194-195., 2006
    Organism: