Bibliography September 2007

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

  2. BERNTSON L, WERNROTH L, FASTH A, AALTO K, HERLIN T, NIELSEN S, NORDAL E, RYGG Met ZAK M: Assessment of disease activity in juvenile idiopathic arthritis. The number and the size of joints matter, J.Rheumatol., Vol. 34(10), 2106-2111., 2007
    Organism:From the Department of Women's and Children's Health, Uppsala University Children's Hospital, Uppsala, SwedenFAU - Berntson, Lillemor
    Abstract:    OBJECTIVE: Variables for assessment of disease activity of juvenile idiopathic arthritis (JIA) were studied, in order to develop a disease activity score for children with JIA. METHODS: One randomly chosen hospital visit was studied for each of 312 patients with JIA, with regard to disease activity variables. The physician global assessment score visual analog scale (physician GA) was used as a dependent variable in comparisons between potential disease activity variables. Previous studies have shown this variable to be the most sensitive to changes in JIA disease activity and to be comparable between patients. RESULTS: Based on Spearman's rank order correlation the number of active joints had a strong association with the physician GA. The median physician GA score rose markedly for each active large joint, but less for small joints, although small joints were also statistically important in assessing disease activity. Among the laboratory data, the erythrocyte sedimentation rate, C-reactive protein level, and platelet count showed weak correlations to the physician GA. CONCLUSION: In preparation of a disease activity score for children with JIA the importance of both the number and size of joints involved needs further evaluation

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

  4. CARL HD, SCHRAML A, SWOBODA Bet HOHENBERGER G: Synovectomy of the hip in patients with juvenile rheumatoid arthritis, J.Bone Joint Surg.Am., Vol. 89(9), 1986-1992., 2007
    Organism:Division of Orthopedic Rheumatology, Department of Orthopedic Surgery, Friedrich Alexander University of Erlangen-Nuremberg, Rathsberger Strasse 57, D-91054 Erlangen, Germany Hans-DieterCarl@ortho-rheumameduni-erlangendeFAU - Carl, Hans-Dieter
    Abstract:    BACKGROUND: There is a lack of data on the functional effect of open hip synovectomy in a large number of patients with juvenile rheumatoid arthritis evaluated with a validated assessment tool. METHODS: Between 1985 and 1997, sixty-seven open hip-joint synovectomies were carried out in fifty-six patients with juvenile rheumatoid arthritis. Fifty-five hips (82%) had radiographic changes that were stage III or higher according to the system of Larsen et al. Hip function was evaluated preoperatively and after a mean of fifty months with the Merle d'Aubigne hip score. RESULTS: Sixty-five (97%) of the sixty-seven hips were available for follow-up. The mean total Merle d'Aubigne hip score (and standard error of the mean) was significantly improved from 9.5 +/- 2.5 points at baseline to 16.3 +/- 1.0 points at the time of follow-up (p < 0.001). The individual scores for pain, mobility, and walking ability were significantly increased as well (all p < 0.001). Eighty-five percent of the hips were observed to have a very great or great improvement in function. A concomitant soft-tissue release was performed in seven hips, and nine hips required surgical dislocation. Surgical complications included two superficial wound hematomas that did not require intervention; osteonecrosis of the femoral head was not observed. Five hips required total hip arthroplasty during the follow-up period. Thus, the survival rate for the hips was 94% at a mean of four years following the synovectomy. CONCLUSIONS: Open hip synovectomy in patients with juvenile rheumatoid arthritis is a safe procedure that can improve hip-joint function for up to five years

  5. CESPEDES-CRUZ A, GUTIERREZ-SUAREZ R, PISTORIO A, RAVELLI A, LOY A, MURRAY KJ, GERLONI V, WULFFRAAT NM, OLIVEIRA S, WALSH J, CALVO P, I, ALPIGIANI MG, LAHDENNE P, SAAD-MAGALHAES C, CORTIS E, LEPORE L, KIMURA Y, WOUTERS C, MARTINI Aet RUPERTO N: Methotrexate improves the health-related quality of life of children with juvenile idiopathic arthritis, Ann.Rheum.Dis., Vol. ., 2007
    Organism:Centro Medico Nacional La Raza, Mexico
    Abstract:    OBJECTIVES: To examine the change in health-related quality of life (HRQOL) and its determinants in children with juvenile idiopathic arthritis (JIA) treated with methotrexate (MTX). METHODS: Patients were extracted from the PRINTO clinical trial aimed to evaluate the efficacy and safety profile of MTX administered in standard, intermediate or higher doses (10, 15 and 30 mg/m2/week respectively). Children with polyarticular-course JIA, who were less than 18 years and had a complete HRQOL assessment were included. RESULTS: A total of 521 children were included. At baseline, JIA patients showed poorer HRQOL (p<0.01) as compared to healthy children. In 207/412 (50%) and 63 (15%) children, HRQOL values were 2 standard deviation from the mean of healthy controls in the physical and psychosocial summary scale, respectively. After 6 months of therapy with standard-dose MTX, there was a statistical significant improvement in all HRQOL health concepts, particularly in the physical ones. Similar improvements were observed in the patients non-responders to standard dose MTX who were subsequently randomized to higher dose-MTX. The presence of marked disability at baseline was associated with a 5-fold increased risk to maintain a poor physical health after 6 months of active treatment with standard-dose MTX. Other less important determinants for maintenance of poor physical well being were the baseline level of systemic inflammation, the intensity of child's pain and an ANA-negative status. CONCLUSIONS: We report that MTX treatment produces a significant improvement across a wide range of HRQOL components, particularly in the physical domains, in patients with JIA

  6. DUZGUN N, SAHIN M, GENC Yet TUTKAK H: Antinucleosome antibodies and systemic lupus erythematosus, Ann.N.Y.Acad.Sci., Vol. 1109, 421-428., 2007
    Organism:Department of Clinical Immunology and Rheumatology, Ankara University Faculty of Medicine, Samanpazari 06100, Ankara, Turkey duzgun@medicineankaraedutrFAU - Duzgun, Nursen
    Abstract:    The aim of this study is to investigate the prevalence of antinucleosome antibody in systemic lupus erythematosus (SLE) and their association with disease activity and renal involvement. The study included 131 patients with SLE, 74 rheumatoid arthritis, 26 systemic sclerosis, and 50 healthy individuals. Antinucleosome antibody and anti-dsDNA antibody were measured by an enzyme-linked immunosorbent assay (ELISA). Antinuclear antibody was tested by immunofluorescence using HEp-2 cells. Out of 131 SLE patients, 72 (54.9%) were seropositive for antinucleosome antibody, which was significantly higher than only 3 of 74 (4%) patients with rheumatoid arthritis (chi(2) = 52.82, P < 0.001); none of the patients with systemic sclerosis and 50 healthy individuals were seropositive. The sensitivity and specificity of antinucleosome antibodies in SLE were 83.6% and 70%, respectively. Fifty-one (38.9%) of SLE patients had renal involvement. Among these patients, the rate of antinucleosome positivity and anti-dsDNA were 74.5% and 78.4%, respectively. Antinucleosome antibodies were found to be 31.4% positive in SLE patients lacking anti-dsDNA antibody. Antinucleosome antibodies significantly correlated with disease activity (r = 0.428, P < 0.001) and anti-dsDNA (r = 0518, P < 0.001). The positivity of antinucleosome antibodies was significantly higher in patients with renal disease than the subjects without renal disease (chi(2) = 12.89, P < 0.001). The results of our study have revealed that in SLE patients, antinucleosome antibody could be a useful parameter for the assessment of disease activity or renal involvement

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

  8. KAKATI P, SODHI KS, SANDHU MS, SINGH S, KATARIYA Set KHANDELWAL N: Clinical and ultrasound assessment of the knee in children with juvenile rheumatoid arthritis, Indian J.Pediatr., Vol. 74(9), 831-836., 2007
    Organism:Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaFAU - Kakati, Pratap
    Abstract:    OBJECTIVE: To correlate clinical features with ultrasound (USG) findings in the detection, quantification and follow up of inflammatory signs of knee in children with mono or pauciarticular juvenile rheumatoid arthritis (JRA). METHODS: Thirty patients (11 girls, 19 boys) with pauciarticular JRA (14 with monoarticular and 16 with bilateral knee involvement) were studied. Mean disease duration was 10 months (range 2 months to 5 yr). All knees were classified into two groups, according to the presence or absence of acute inflammation. Clinical assessment and ultrasound was done in all patients on the same day. All the patients received naproxen (15-20 mg/Kg/day) for a period of six months, after which clinical assessment and ultrasound study was repeated. RESULTS: Synovial proliferation and effusion, was demonstrated in a much higher frequency in those clinically active (Group A) as compared to these in clinical remission (Group B). Statistically significant differences between clinical and USG indices were seen. CONCLUSION: USG of knee is more sensitive than clinical assessment in detection of synovial effusion and thickening and plays a useful role in monitoring evolution of the inflammatory process, its quantification and for follow up

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

  10. KOLARZ B, TARGONSKA-STEPNIAK B, DARMOCHWAL-KOLARZ Det MAJDAN M: [Autoimmune aspects of treatment with TNF-alpha inhibitors], Postepy Hig.Med.Dosw.(Online.)., Vol. 61, 478-484., 2007
    Organism:Katedra i Klinika Reumatologii i Ukladowych Chorob Tkanki Lacznej Akademii Medycznej im prof F Skubiszewskiego w Lublinie, Lublin, Poland mariamajdan@amlublinplFAU - Kolarz, Bogdan
    Abstract:    Tumor necrosis factor-alpha (TNF-alpha) plays an important role in the pathogenesis of such diseases as rheumatoid arthritis, Crohn's disease, ankylosing spondylitis, psoriatic arthritis, and juvenile chronic arthritis. Recent years have brought improvement in the understanding of the pathogeneses of these diseases, resulting in the production of new groups of biological drugs, including, among others, anti-TNF-alpha antibodies. The use of TNF inhibitors has been a great advance in the treatment of patients with these inflammatory diseases. Infliximab and adalimumab are monoclonal antibodies that bind to and neutralize the activity of TNF-alpha. Infliximab is a mouse/human chimera that joins the variable regions of a mouse antibody to the constant region of human IgG1. Adalimumab is a fully human IgG1 antibody. Etanercept is a dimeric fusion protein that joins the human p75 TNF receptor to the Fc domain of human IgG1. The beneficial effects of the anti-TNF monoclonal antibodies infliximab and adalimumab and the soluble receptor fusion protein etanercept in the treatment of rheumatoid arthritis, especially in patients resistant to other disease-modifying antirheumatic drugs (DMARDs), are discussed. We observe stoppage of articular destruction during treatment with TNF-alpha inhibitors. Soon after the introduction of this therapy it was found that these agents have a propensity for stimulating the production of autoantibodies and antibodies against themselves. In this review, recent studies analyzing the effect of TNF-alpha blockade (infliximab, etanercept, and adalimumab) on the ANA, anti-dsDNA, and anticardiolipin antibody profiles in autoimmune diseases are discussed

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

  12. LEVY-CLARKE G, GANGAPUTRA Set NUSSENBLATT R: Treatment with TNF inhibitors for uveitis associated with juvenile idiopathic arthritis, Nat.Clin.Pract.Rheumatol., Vol. ., 2007
    Organism:G Levy-Clarke is the Director of the Uveitis and Ocular Immunology Fellowship, S Gangaputra is a Postdoctoral Fellow and R Nussenblatt is the Chief of the Laboratory of Immunology at the National Eye Institute, NIH, Bethesda, MD, USA
    Abstract:

  13. LOPEZ-HOYOS M, RODRIGUEZ-VALVERDE Vet MARTINEZ-TABOADA V: Performance of antinuclear antibody connective tissue disease screen, Ann.N.Y.Acad.Sci., Vol. 1109, 322-329., 2007
    Organism:Immunology Department, Hospital Universitario Marques de Valdecilla, Santander, Spain inmlhm@humvesFAU - Lopez-Hoyos, Marcos
    Abstract:    Antinuclear antibodies (ANAs) have become routine laboratory parameters in clinical hospitals. However, ANA testing by indirect immunofluorescence (IIF) assays is not an automated laboratory test. Efforts are being made to develop easy and semi- or automated methods to screen for ANAs. We evaluated the clinical performance of a new ELISA developed to screen for connective tissue disease related ANAs. The presence of serum ANA was studied with a commercial ELISA (Varelisa ANA CTD Screen) in 472 patients (202 SLE, 41 Sjogren syndrome, 11 CREST, 59 rheumatoid arthritis, 30 seronegative spondyloarthropaties, 77 inflammatory bowel disease, 13 reactive arthritis, 11 giant cell arteritis, 28 ankylosing spondilitis). A hundred and five sera from healthy subjects were used as controls. Receiver operator characteristics (ROC) analysis was carried out in order to optimize the cutoff. At target specificities of 80/90%, sensitivities of 80.8/ 73.9% were achieved. At the manufacturer's cutoff (ratio >or=1.0) sensitivity/specificity of 71.4/91.2% was found. At that cutoff, a positive likelihood ratio of 8.11 was found. For helping in the diagnosis of connective tissue diseases a test employing a subset of the most prevalent specificities reveals a good compromise as indicated by a high-positive likelihood ratio. However, the presence of ANAs in pathologies other than connective tissue diseases, such as SLE or Sjogren syndrome, may be of clinical significance as well. In these cases an IIF assay test is still mandatory, especially in autoimmune laboratories

  14. MARTINEZ A, OROZCO G, VARADE J, SANCHEZ LM, PASCUAL D, BALSA A, GARCIA A, DE LA CONCHA EG, FERNANDEZ-GUTIERREZ B, MARTIN Jet URCELAY E: Macrophage migration inhibitory factor gene: influence on rheumatoid arthritis susceptibility, Hum.Immunol., Vol. 68(9), 744-747., 2007
    Organism:Department of Clinical Immunology, Hospital Clinico San Carlos, Madrid, SpainFAU - Martinez, Alfonso
    Abstract:    The macrophage inhibitory factor (MIF) is a cytokine that has been implicated in several inflammatory and autoimmune diseases, including rheumatoid arthritis, systemic lupus, glomerulonephritis, and multiple sclerosis. In rheumatoid arthritis (RA), results ranging from lack of association of MIF polymorphisms with RA, to involvement in either severity or susceptibility to the disease have been reported in the past. We aimed at investigating the role of this gene in RA in the Spanish population. Two well-known MIF promoter polymorphisms were tested in 606 adult RA patients and 886 healthy controls: a single nucleotide polymorphism at -173G/C and a tetranucleotide repeat (CATT)(5-8) located at -794. We found a significant association of the allele -173C with RA (p = 0.01; odds ratio [OR] = 1.31; 95% confidence interval [CI] = 1.06-1.62). The -173C risk allele, previously reported to be transmitted in excess in patients with juvenile idiopathic arthritis, was significantly more frequent in early-onset adult RA patients than in healthy controls (p = 0.003; OR = 1.57; 95% CI = 1.14-2.15), whereas late-onset patients were not significantly different to controls (p = 0.6; OR = 1.09; 95% CI = 0.77-1.55). In conclusion, the -173C allele in the MIF promoter region is associated with increased RA predisposition, mainly in early-onset patients

  15. MOROZZI G, FABBRONI M, BELLISAI F, PUCCI Get GALEAZZI M: Cartilage oligomeric matrix protein level in rheumatic diseases: potential use as a marker for measuring articular cartilage damage and/or the therapeutic efficacy of treatments, Ann.N.Y.Acad.Sci., Vol. 1108, 398-407., 2007
    Organism:Department of Clinical Medicine and Immunology, Rheumatology Section, University of Siena, Siena, Italy morozzi@unisiitFAU - Morozzi, Gabriella
    Abstract:    Cartilage oligomeric matrix protein (COMP) is a tissue-specific noncollagenous protein that was first detected in the serum and the synovial fluid of patients suffering from rheumatic disorders, such as rheumatoid arthritis, reactive arthritis, juvenile chronic arthritis, and osteoarthritis. In this review, the authors consider serum COMP levels in different diseases and discuss their study of patients with rheumatoid arthritis treated with anti-TNF-alpha, to evaluate whether COMP is able to predict a rapid and sustained clinical response to these drugs. They observe that patients with high COMP levels have a lower ACR 70 response independently of the state of systemic inflammation, and conclude that COMP seems to have a pathogenetic role that is independent of the mechanisms regulating inflammatory processes

  16. MULHALL KJ, SALEH KJ, THOMPSON CA, SEVERSON EPet PALMER DH: Results of bilateral combined hip and knee arthroplasty in very young patients with juvenile rheumatoid arthritis, Arch.Orthop.Trauma Surg., Vol. ., 2007
    Organism:Department of Orthopaedic Surgery, Mater Misericordiae University Hospital, Eccles Street, Dublin 7, Ireland, kjm@indigoie
    Abstract:    INTRODUCTION: We evaluated the long-term outcomes for combined, bilateral total knee and hip arthroplasty performed on a group of very young patients with juvenile rheumatoid arthritis. MATERIALS AND METHODS: Six consecutive patients with a mean age of 14 years at the time of hip replacement and 16 years at knee replacement were analyzed. Five of the six patients were wheelchair dependent pre-operatively. All knee components had uncemented fixation, while the hip replacements were a mixed group of cemented and uncemented prostheses. RESULTS: Clinical and radiographic follow-up at a mean duration of 13.8 years for the hips and 17.3 years for the knees demonstrated four of the six patients were unlimited community ambulators, one a limited community ambulator and the remaining patient a household ambulator. Failure, defined as revision of any of the components or definite radiographic loosening, occurred in three knees (two patients) and five hips (three patients). CONCLUSIONS: These good long-term functional results in a relatively very young population indicate that an early and aggressive approach to multiple joint disease is an appropriate option at a young age for patients with juvenile rheumatoid arthritis with severe disability and pain refractory to conservative management

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

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

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

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

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

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

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

  24. SHANDWICK Wet BUDE S: [Abbott proposes in Europe and the U.S.A. the approval of Humira (adalimumab) for therapy of juvenile idiopathic arthritis. Pediatric clinical trial shows very promising results in juvenile rheumatoid arthritis], Kinderkrankenschwester., Vol. 26(8), 335-336., 2007
    Organism:

  25. STRINGER DE, GILBERT DH, HERFORD ASet BOYNE PJ: A method of treating the patient with postpubescent juvenile rheumatoid arthritis, J.Oral Maxillofac.Surg., Vol. 65(10), 1998-2004., 2007
    Organism:Department of Oral and Maxillofacial Surgery, Loma Linda University, Loma Linda, CA, USA omfsdale@aolcomFAU - Stringer, Dale E
    Abstract:    PURPOSE: Juvenile rheumatoid arthritis (JRA) is a perplexing and devastating disease for which establishment of a treatment protocol is difficult. The relatively low incidence and unknown cause of this disorder have made it difficult to establish when and how to intervene. Treatment protocols for the prepubescent patient (<12 years for girls and 14 years for boys), as well as for the adult, have been established. A protocol for postpubescence (ages 12 through 18 years) has yet to be established. This pilot study attempts to establish another treatment protocol for this particular subgroup of patients. PATIENTS AND METHODS: Five girls between the ages of 14 and 18 years with common facial deformities who were given a diagnosis of juvenile rheumatoid arthritis were reconstructed by orthognathic surgery and costochondral rib grafts. All underwent surgery performed by the first author and were followed for 4 to 14 years. Patients were in disease remission at the time of surgery, and all presented with the same skeletal/dental deformity and condylar destruction. RESULTS: Serial cephalograms were taken immediately presurgically (T1), immediately postsurgically (T2), and at latest recall (T3). Tracings were done by the same orthodontist with the use of Quick Ceph Image Pro software (Quick Ceph Systems, San Diego, CA). Mandibular position as it related to the success of costochondral and orthognathic surgery was assessed by gnathion position relative to nasion-basion at the cranial center, as described by Rickett's facial analysis. Patient long-term follow-up lasted from 4 to 14 years and had a mean duration of 9.6 years. The average increase in anterior/posterior direction (T1 to T2) was 22.7 mm with an average relapse (T2 to T3) of 1.5 mm. Four of 5 patients had a stable Class I occlusion on follow-up, and 1 developed a 3-mm open bite postoperatively. CONCLUSION: This pilot study offers a treatment protocol for the postpubescent juvenile patient with rheumatoid arthritis (aged 12 to 18 years) that is based on a single surgery with relative postoperative stability

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

  27. VIKEN MK, SOLLID HD, JONER G, DAHL-JORGENSEN K, RONNINGEN KS, UNDLIEN DE, FLATO B, SELVAAG AM, FORRE O, KVIEN TK, THORSBY E, MELMS A, TOLOSA Eet LIE BA: Polymorphisms in the cathepsin L2 (CTSL2) gene show association with type 1 diabetes and early-onset myasthenia gravis, Hum.Immunol., Vol. 68(9), 748-755., 2007
    Organism:Institute of Immunology, Faculty Division Rikshospitalet, University of Oslo, Oslo, Norway mkviken@medisinuionoFAU - Viken, Marte K
    Abstract:    Type 1 diabetes (T1D) is an autoimmune disease characterized by loss of beta cells in the pancreas. The CTSL2 gene encodes the cysteine protease cathepsin V involved in antigen presentation in human cortical thymic epithelial cells, and involvement of the protease in autoimmunity has been suggested. This study aimed to evaluate CTSL2 as a candidate gene for T1D, and test whether the gene predisposes more generally to autoimmune diseases. Four polymorphisms aiming at tagging the CTSL2 locus were genotyped in 421 T1D families, and subsequently in 861 rheumatoid arthritis patients, 530 juvenile idiopathic arthritis patients, and 559 controls of Norwegian origin. Additionally, DNA from 83 German myasthenia gravis (MG) patients and 244 controls were investigated. A polymorphism, rs16919034, situated downstream of CTSL2 was associated with T1D (60.8%T, p = 0.008; p(c) = 0.03). An association with early-onset MG (45% in cases vs 36.6% in controls; p = 0.03) was observed for another polymorphism (rs4361859) situated upstream of the gene, but within the same linkage disequilibrium block. No association was observed in rheumatoid arthritis or juvenile idiopathic arthritis. Our findings suggest that the CTSL2 gene is associated with T1D and with early-onset MG

  28. WEISSBRICH B, SUSS-FROHLICH Yet GIRSCHICK HJ: Seroprevalence of parvovirus B19 IgG in children affected by juvenile idiopathic arthritis, Arthritis Res.Ther., Vol. 9(4), R82, 2007
    Organism:Institute of Virology and Immunobiology, University of Wurzburg, Versbacher Str 7, 97078 Wurzburg, Germany weissbrich@vimuni-wuerzburgde
    Abstract:    ABSTRACT: Parvovirus (PV) B19 is the causative agent of the childhood disease erythema infectiosum. An association of PV B19 with chronic arthropathies, sometimes resembling rheumatoid arthritis or juvenile idiopathic arthritis (JIA), has repeatedly been described. Other studies, however, have failed to identify any such relationship. In order to study further whether there is a link between PV B19 and JIA, we determined the prevalence of PV B19 specific IgG antibodies in serum samples from children with rheumatoid diseases and compared it with the prevalence in unaffected children We reasoned that if there is an association between PV B19 and JIA, then the prevalence of PV B19 IgG in the children with JIA should be higher than in the control group. PV B19 IgG status was tested in 406 children with JIA and related diseases, and in 146 children constituting a control group. The percentage of PV B19 IgG positive children was not significantly elevated in the disease subgroups compared with age-matched control groups. In conclusion, our findings do not support the hypothesis that human parvovirus B19 is involved in the pathogenesis of JIA

  29. WRIGHT Tet CRON RQ: Pediatric rheumatology for the adult rheumatologist II: uveitis in juvenile idiopathic arthritis, J.Clin.Rheumatol., Vol. 13(4), 205-210., 2007
    Organism:Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4318, USAFAU - Wright, Tracey
    Abstract:    Uveitis is an important and frequent extra-articular manifestation of juvenile idiopathic arthritis (JIA) that may result in poor visual outcome. Without early detection and aggressive therapy, the uveitis and topical steroid therapy used to treat it may result in cataracts, glaucoma, and even blindness. Fortunately, a variety of systemically administered anti-inflammatory agents have been found useful for the treatment of JIA associated uveitis. Methotrexate is often the first line disease modifying systemic agent used to help wean topical corticosteroids, but when this is not sufficiently effective there are a variety of other systemic medicines available. In particular, one of the tumor necrosis factor-alpha inhibitors, infliximab, has shown some promising results in difficult to treat JIA associated uveitis. With early screening and detection combined with aggressive therapy in difficult to treat cases, the morbidity associated with uveitis as part of JIA is on the decline