November 2006

Bibliography November 2006

Anonymous, [The role of regulatory T-cells in autoimmune rheumatic diseases], Vestn.Ross.Akad.Med.Nauk., Vol. (9-10), 74-82., 2006
Organism:Regulatory T-cells CD4+CD25+Foxp3, possessing suppressory activity, play the key role in the development of autoimmune diseases, maintenance of peripheral tolerance in transplantation immunity, and the prevention of a pathological immune response to intestinal microflora or microbial infection. A decrease in the total number of circulating CD4+CD25+ regulatory T-cells and their suppressive activity have been found in patients suffering from various autoimmune diseases, such as type 1 diabetes, multiple sclerosis, autoimmune hepatitis, psoriatic arthritis, juvenile idiopathic arthritis, and systemic lupus erythematosus (SLE). The authors of this study investigated the phenotypic characteristics of peripheral blood lymphocytes in 31 SLE patients and the effect of treatment on the content of CD4+CD25+ T-cells before and after pulse therapy with methylprednisolone and cyclophosphan. The total number of regulatory T-cells in the group of untreated patients was almost twice lower vs. the group of healthy donors. As a result of the therapy, the proportion of regulatory T-cells increased significantly, although it did not reach the values in the control group. The data from this research confirm the development of a defect of CD4+CD25+ T-cells at the active phase of SLE, and a possibility to partially correct this defect with an effective therapy
Anonymous, SOFREMIP Congress, Strasbourg, FRANCE, April 21 -22, 2006, 1149-1153., 21-04-2001
AGGARWAL P, AGGARWAL A, GUPTA Set MISRA R: Osteopenia is common in adult male patients with active juvenile idiopathic arthritis, Rinsho Ganka, Vol. 33, 1642-1645., 2006
Organism:Sanjay Gandhi Postgrad Inst Med Sci, Dept Immunol, Lucknow 226014, Uttar Pradesh, India India
Abstract:Objective. To assess the prevalence of osteopenia in Southeast Asian men with active juvenile idiopathic arthritis (JIA) and to identify predictors of reduced bone mineral density (BMD).Methods. BMD of 30 men with active JIA and 23 healthy men was assessed by dual energy x-ray absorptiometry scans. Clinical variables that influence bone mass were also analyzed. T scores were calculated based on Caucasian normative data.Results. Absolute BMD (g/cm(2)) was significantly lower in men with active JIA compared to controls at all measured sites, i.e., lumbar spine (p = 0.018), hip (p = 0.018), and distal third of forearm (p = 0.044). More subjects in the JIA group had low BMD (T score <= -1.0) than controls at hip (22/30 vs 9/23; p < 0.05) and di stal third of forearm (27/30 vs 10/23; p < 0.001), while at lumbar spine region the difference was not statistically significant (22/30 vs 13/23). A significant negative correlation of BMD was found with joint deformities, limitation of joint movement, Health Assessment Questionnaire score, and erythrocyte sedimentation rate. BMD at the hip and distal third of forearm was significantly lower in patients having arthritis at these sites. A positive correlation of BMD was found with body mass index.Conclusion. A majority of Southeast Asian men with active JIA have reduced BMD. More patients in our cohort had low BMD compared to reports from Western countries. This finding may be attributed to use of Caucasian normative data, uncontrolled disease activity, severity of disease, poor nutritional status, or an ethnic variation
AKAY AP, UNSAL E, OZBEK Aet BAYKARA B: Psychosocial aspects of Turkish mothers of children with juvenile idiopathic arthritis, Rheumatol.Int., Vol. 26(7), 685-686., 2006
Organism:
APRIL KT, FELDMAN DE, PLATT RWet DUFFY CM: Comparison between children with juvenile idiopathic arthritis and their parents concerning perceived treatment adherence, Arthritis & Rheumatism, Vol. 55, 558-563., 2006
Organism:Univ Montreal, Ecole Readaptat, CP 6128,Succ Centreville, Montreal, PQ H3C 3J7, Canada Canada
Abstract:Objective. Adherence to treatment in juvenile idiopathic arthritis (JIA) may be associated with better outcomes. Clinicians must be aware of possible divergence between parents and children regarding adherence, in order to gain a better understanding of adherence and factors associated with it. The objective was to determine the level of agreement between children with JIA and their parents concerning perception of the child's adherence to the treatment regimen (for both medications and exercises).Methods. Fifty patients and their parents, who attended the JIA clinic at the Montreal Children's Hospital, completed the Child Adherence Report Questionnaire and the Parent Adherence Report Questionnaire. Paired t-tests were used to compare parents' and children's scores for adherence questions and agreements were analyzed by intraclass correlation coefficients (ICCs).Results. Parents reported that their children showed more negative reactions to taking medication and doing exercises, more helpfulness from the medication, and more difficulty to carry out the exercise program than their children reported. ICCs (95% confidence interval) for medications and exercises were, respectively, 0.32 (0.04, 0.56) and 0.77 (0.61, 0.87) for overall adherence, 0.33 (0.05, 0.57) and 0.39 (0.09, 0.62) for perceived difficulty to following treatment, and 0.37 (0.09, 0.60) and 0.45 (0.17, 0.67) for how often children had negat ive reactions following treatment. Levels of agreement for perceived helpfulness of treatments were quite low.Conclusion. Agreement between parents and children concerning adherence was at best moderate, and generally better for the exercise program than for prescribed medications
ARNOLD I: [Intra-articular injections of corticosteroids--contra], Dtsch.Med.Wochenschr., Vol. 131(41), 2287, 2006
Organism:Abteilung fur operative Rheumatologie und Orthopadie im Rheumazentrum Bremen, Rotes Kreuz-Krankenhaus Bremen arnold@roteskreuzkrankenhausdeFAU - Arnold, I
Abstract:
BRUNNER H, I, MUELLER M, RUTHERFORD C, PASSO MH, WITTE D, GROM A, MISHRA Jet DEVARAJAN P: Urinary neutrophil gelatinase-associated lipocalin as a biomarker of nephritis in childhood-onset systemic lupus erythematosus, Arthritis & Rheumatism, Vol. 54, 2577-2584., 2006
Organism:Univ Cincinnati, Childrens Hosp, Ctr Med, William Rowe Div Rheumatol, E 4010,3333 Burnet Ave, Cincinnati, OH 45229 USA USA
Abstract:Objective. Renal involvement in systemic lupus erythematosus (SLE) is associated with poor prognosis. Currently available renal biomarkers are relatively insensitive and nonspecific for diagnosing SLE nephritis. Previous research suggests that neutrophil gelatinase-associated lipocalin (NGAL) is a high-quality renal biomarker of acute kidney injury, while its usefulness in SLE is unclear. We undertook this study to determine the relationship between urinary NGAL excretion and SLE disease activity or damage, with a focus on nephritis.Methods. A cohort of 35 patients diagnosed as having SLE prior to age 16 years (childhood-onset SLE) was assessed for disease activity (using the Systemic Lupus Erythematosus Disease Activity Index 2000 update) and damage (using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology SLE Damage Index) in a double-blind, cross-sectional study. Information on current markers of renal function and disease was obtained and compared with NGAL levels (ng/mg of urinary creatinine) measured by enzyme-linked immunosorbent assay. Eight children with juvenile idiopathic arthritis (JIA) served as controls.Results. NGAL levels did not differ with the age, weight, height, sex, or race of the patients. Patients with childhood-onset SLE had significantly higher NGAL levels than did those with JIA (P < 0.0001). NGAL levels were strongly to moderately correlated with renal disease activity and renal damage (Spearman's r >= 0.47, P < 0.0001 for both comparisons), but not with extrarenal disease activity or extrarenal damage. NGAL levels of > 0.6 ng/mg urinary creatinine were 90% sensitive and 100% specific for identifying childhood-onset SLE patients with biopsy-proven nephritis.Conclusion. Urinary NGAL is a promising potential biomarker of childhood-onset SLE nephritis. The results of the current study require validation in a larger cohort to more accurately delineate urinary NGAL excretion in relation to the diverse SLE phenotypes
CALGUNERI M, URETEN K, AKIF OM, ONAT AM, ERTENLI I, KIRAZ Set AKDOGAN A: Extra-articular manifestations of rheumatoid arthritis: results of a university hospital of 526 patients in Turkey, Clin.Exp.Rheumatol., Vol. 24(3), 305-308., 2006
Organism:Hacettepe University Department of Rheumatology, Ankara, TurkeyFAU - Calguneri, M
Abstract:OBJECTIVE: Presence of extra-articular manifestations (EAM) in rheumatoid arthritis (RA) is associated with more severe disease and increased mortality. Prevalence of EAM may vary in different geographic areas and in different ethnic populations. In this study we investigated the frequency of EAM in 526 RA patients from a single university hospital in Turkey. METHODS: The hospital records of patients who had been diagnosed as RA in Hacettepe University Department of Rheumatology between the years 1988 and 2003 were retrospectively evaluated. There were 73 males and 453 females, and mean age of the patients was 48.0 +/- 12.3 years. The mean follow-up period was 4.8 +/- 4.1 years. Three hundred and fifty-nine patients were rheumatoid factor (RF) positive (68.3%). RESULTS: The overall frequency of EAM was 38.4% (202 patients). The most common EAM was rheumatoid nodules (18.1%). Sicca symptoms, pulmonary findings, Raynaud's phenomenon, livedo reticularis, carpal tunnel syndrome, vasculitis, amyloidosis, and Felty syndrome were present in 11.4%, 4.8%, 3%, 4.8%, 2.8%, 1.3%, 1.1%, and 0.3% of the patients, respectively. Overall EAM and rheumatoid nodules were significantly more common in RF positive patients than RF negative patients. The frequency of rheumatoid nodules was significantly higher in males than in females. CONCLUSION: The prevalence of EAM in Turkey is higher than East Asia and Africa, and lower than UK and North America. Excluding secondary Sjogren's syndrome, our results are similar to other Mediterranean populations like Italy
CIMAZ Ret DESCLOUX E: Pediatric antiphospholipid syndrome, Rheumatic Disease Clinics of North America, Vol. 32, 553-573., 2006
Organism:Hop Edouard Herriot, Dept Pediat, Pavillon S,5 Pl dArsonval, F-69437 Lyon, France France
Abstract:Similar as in adults, antiphospholipid antibodies (aPL) have been recognized in a number of childhood conditions including pediatric autoimmune diseases, various infections, as well as in a small percentage of apparently healthy children [1-7]. Pathogenic mechanisms involved in pediatric antiphospholipid syndrome (APS) have not been thoroughly investigated, but it is generally assumed that they are similar to adults. However, there clearly are differences in the frequency of specific clinical events, because children do not have risk profiles that could be predisposing factors for thrombosis (eg, atherosclerosis, cigarette smoking, contraceptive medications).Most of the clinical features that can occur in adults with aPL have also been described in children, but often just as individual case reports or small case series from single institutions. Large cohorts or multicenter studies on APS in pediatric population are rare, and consequently, there is very little accurate information on the pediatric aspects of APS. The aim of this review is to highlight relevant clinical advances in the field of pediatric APS
CLEMENT SA, BURROWS NP, SANSOME A, HAZLEMAN BLet OSTOR AJ: Harlequin ichthyosis and juvenile idiopathic arthritis: a rare combination, Clin.Rheumatol., Vol. ., 2006
Organism:Rheumatology Research Unit, Box 194, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Rd, Cambridge, CB2 2QQ, UK, andrewostor@addenbrookesnhsuk
Abstract:
CONNELLY Met SCHANBERG L: Opioid therapy for the treatment of refractory pain in children with juvenile rheumatoid arthritis, Nat.Clin.Pract.Rheumatol., Vol. 2(12), 636-637., 2006
Organism:M Connelly is the Co-Director of Integrative Pain Management at Children's Mercy Hospital in Kansas City, and an Assistant Professor of the School of Medicine at University of Missouri, Kansas City, MOFAU - Connelly, Mark
Abstract:
FEUCHTENBERGER M, KNEITZ Cet TONY H-P: Treatment of rheumatoid arthritis (RA) with anticytokines|, 2006
Organism:In the hands of an experienced rheumatologist and in adherence to the contraindications named in the article, anticytokines such as TNF-alpha blockers or interleukin-1 antagonists are regarded as relatively reliable, are well tolerated and in many cases, are very effective. Especially when used in combination with methotrexate, they demonstratively lower the disease activity score and significantly slow the radiographic progression. Thus, anti-cytokines are currently the most effective therapy for RA. An additional advantage compared to conventional DMARD is the rapid onset of action (usually within two to four weeks). TNF-alpha blockers are also presently employed in numerous other chronic inflammatory diseases. The efficacy of anticytokines in psoriasis and psoriatic arthritis, ankylosing spondylitis, juvenile arthritis and Crohn's disease has been proven
GARDNER-MEDWIN JM, KILLEEN OG, RYDER CAJ, BRADSHAW Ket JOHNSON K: Magnetic resonance imaging identifies features in clinically unaffected knees predicting extension of arthritis in children with monoarthritis|, 2006
Organism:Objective. A proportion of children with oligoarthritis have an aggressive disease course. Identifying these children at presentation would help guide prognosis and management. We examined if magnetic resonance imaging (MRI) of clinically unaffected joints is more sensitive than clinical assessment in identifying those at risk of developing arthritis in more than one joint. Methods. Ten children were recruited; they had a mean age of 9.4 (range 5.2-14.2) years at presentation of a monoarthritis. MRI of a clinically unaffected knee was performed within 4 months of presentation, and was reported by 2 pediatric radiologists blinded to the clinical findings. All MR exami nations included post-gadolinium sequences. Joints with clinically apparent arthritis were recorded regularly over a median of 37.0 (range 6.6-47.0) months by a median of 6.0 (range 2-8) pediatric rheumatologists blinded to the MR result. Results. Four children developed arthritis in other joints over a median of 3.9 (range 3-6) months after the MRI scan; all had abnormal MRI scans at presentation. Three of these developed clinical features in the previously normal knee 4-11 months after MRI identified small joint effusions, synovial hypertrophy, and lymph node enhancement. One child developed a polyarthritis, but never developed clinical features in the imaged knee over 3.8 years of followup. Four other children had a persistent monoarthropathy with a median followup of 29.5 (range 6.6-42.0) months. All 4 had normal MRI. Two children had reactive arthritis. Conclusion. MRI distinguished between patients with a persistent monoarthritis and those who developed further clinical arthritis up to 1 year later. The results suggest a widespread inflammatory process may exist in children whose arthritis extends, and this has implications for our understanding of disease and the design and timing of therapeutic interventions
GATTORNO M, CHICHA L, GREGORIO A, FERLITO F, ROSSI F, JARROSSAY D, LANZAVECCHIA A, MARTINI Aet MANZ MG: Distinct expression pattern of IFN-{alpha} and TNF-{alpha} in juvenile idiopathic arthritis synovial tissue, Rheumatology (Oxford)., Vol. ., 2006
Organism:Second Division of Pediatrics, 'G Gaslini' Institute for Children and University of Genoa, Genoa, Italy
Abstract:Objectives. Recent laboratory and clinical data suggest that two prototype autoimmune diseases, systemic lupus erythematosus and rheumatoid arthritis are mainly driven by distinct cytokines, interferon (IFN)-alpha and tumour necrosis factor (TNF)-alpha, respectively. We here investigated the presence and characteristics of natural type I IFN-producing cells (IPCs), as well as IFN-alpha and TNF-alpha expression at sites of inflammation in juvenile idiopathic arthritis (JIA). Methods. Peripheral blood (PB) and synovial fluid (SF) mononuclear cells (MNCs) (n = 25 each) from JIA patients with active disease were studied. IPCs were identified as BCDA-2(+)CD123(+)HLA-DR(+)CD45RA(+) cells, and dendritic cells (DCs) as CD11c(+)CD14(-/low)lin(-) cells by flow cytometry. IPCs and DCs were analysed for Toll-like receptor-7 and -9 mRNA expression by real-time polymerase chain reaction. IFN-alpha was measured by enzyme-linked immunosorbent assay in serum, SF and in supernatants of influenza virus-infected, cultured IPCs. Synovial tissues of n = 6 additional JIA patients were analysed by immunohistochemistry using mAbs against CD123, IFN-alpha, TNF-alpha, CD3, CD19 and CD138. Results. IPCs were enriched in SF MNCs compared with PB MNCs in all JIA patients. Influenza-induced, but no spontaneous IFN-alpha release was detected from SF IPCs, and serum and SF IFN-alpha levels were not elevated. Nonetheless, in synovial tissue IFN-alpha producing cells accumulated at inflammatory lymph-follicular-like structures, while TNF-alpha producing cells were mostly found at the lining and sublining layers. Conclusions. These data suggest that besides TNF-alpha-expressing cells, IFN-alpha-producing IPCs are involved in initiation, maintenance or regulation of the inflammatory response in JIA
GOLDMUNTZ EAet WHITE PH: Juvenile idiopathic arthritis: a review for the pediatrician, Pediatr.Rev., Vol. 27(4), e24-e32, 2006
Organism:Children's National Medical Center, Department of Pediatrics, George Washington University School of Medicine and Health Sciences, Washington, DC, USAFAU - Goldmuntz, Ellen A
Abstract:
HANZLICEK P, ZVAROVA Jet DOSTAL C: Information technology in clinical research in rheumatology domain, Stud.Health Technol.Inform., Vol. 124, 187-192., 2006
Organism:EuroMISE Centre of Charles University, Academy of Sciences CR, Institute of Computer Science AS CR, Prague, Czech Republic hanzlicek@euromiseczFAU - Hanzlicek, Petr
Abstract:The development of a functional clinical database of rheumatic diseases represents an essential step in the process of acquiring the necessary epidemiological and other information on disorders under study. In 1999-2005 the Institute of Rheumatology in cooperation with the EuroMISE Center has developed the Clinical database/National Register of selected systemic inflammatory rheumatic diseases inclusive of a bank of sera and DNA. Aims of this phase of the pilot research were gathering clinical, laboratory, genetic, pharmaco-and socio-economic data in a representative sample of patients with systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, mixed connective tissue disease; rheumatoid arthritis, juvenile chronic arthritis, ankylosing spondylitis, psoriatic arthritis and reactive arthritis. In 2002 the preset number of over 2000 registered patients had been achieved with collaboration of 34 territorial and 20 institutional rheumatologists in the whole covering the majority of the Czech Republic. Based on experiences gathered, the systems for other related studies are being developed and implemented using modern information technologies
HARJACEK M, OSTOJIC Jet DJAKOVIC RO: Juvenile spondyloarthropathies associated with Mycoplasma pneumoniae infection, Clin.Rheumatol., Vol. 25(4), 470-475., 2006
Organism:Department of Pediatrics, Division of Pediatric Rheumatology/Immunology, Children's Hospital Zagreb, Klaiceva 16, 10 000, Zagreb, Croatia miroslavharjacek@zghtnethrFAU - Harjacek, Miroslav
Abstract:BACKGROUND: Reactive arthritis (ReA) has been sporadically reported as triggered by Mycoplasma pneumoniae. This study examined the potential relationship between the acute M. pneumoniae infection and juvenile spondyloarthropathy (jSpA) in children. PATIENTS AND METHODS: Twelve patients with ReA secondary to acute M. pneumoniae were examined. M. pneumoniae-specific IgM, IgG and IgA antibodies were serologically confirmed by enzyme-linked immunosorbent assay (ELISA) tests (Savyon Diagnost., Israel). Due to the early appearance and relatively short life time of M. pneumoniae-specific IgM antibodies, their detection allowed the diagnosis of acute infection using single serum sample, confirmed by parallel serum in 7 of 12 patients. Specific IgM and IgG titers higher than 10 U/l were considered positive and those higher than 50 U/l as highly positive. Specific IgA antibodies were detected in only one patient. RESULTS: Four patients were female and eight were male. The mean age at onset was 9 years, and the mean duration of follow-up was 24.1 months (range 18-32). The mean number of involved joints was 2.8, and the knee joints were involved in 7 of 12 patients. The mean recovery time was 4.5 weeks (range 1-28) in eight reactive arthritis (ReA) cases; three patients developed enthesitis-related arthritis, and in one patient, genuine juvenile ankylosing spondylitis (jAS) was diagnosed. Two patients were HLA-B27-positive, and one patient was HLA-B7/B27-positive. Six patients had preceding respiratory symptoms, and five were treated with antibiotics. CONCLUSIONS: Our findings provide clear evidence of ReA diagnosis following an acute M. pneumoniae infection that in four patients progressed to chronic jSpA. Our results suggest that detecting M. pneumoniae-specific antibodies in serological screening of jSpA patients might be useful. It is presently unclear whether antibiotic treatment would change the disease course in those patients
HASHKES PJet LAXER RM: Update on the medical treatment of juvenile idiopathic arthritis, Curr.Rheumatol.Rep., Vol. 8(6), 450-458., 2006
Organism:Department of Rheumatic Diseases A50, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA hashkep@ccforgFAU - Hashkes, Philip J
Abstract:Many exciting developments in the treatment of juvenile idiopathic arthritis (JIA) have emerged recently, including new tools to assess the results of clinical trials (eg, the definition of remission and a radiologic scoring tool). New controlled studies examined the equivalence of meloxicam to naproxen, the efficacy of leflunomide but the superiority of methotrexate, and the use of infliximab in polyarthritis JIA. Initial studies have shown the potential of anti-interleukin (IL)-1 and anti-IL-6 receptor antibody therapy for systemic JIA. Corticosteroid-sparing medications including the use of "biologic modifiers" for JIA-associated uveitis have been described. Evidence-based guidelines for the main subtypes of JIA have been published. However, good evidence on the treatment of several disease subtypes is still lacking. Studies of new medications and the use of combination therapy, including aggressive induction therapy early in the disease course, are necessary to continue improving the outcome of JIA patients
HESSNER MJ, LIANG Met KWITEK AE: The application of microarray analysis to pediatric diseases, Pediatr.Clin.North Am., Vol. 53, 579-590., 2006
Organism:Med Coll Wisconsin, 8701 Watertown Plank Rd, Milwaukee, WI 53226 USA USA
Abstract:DNA microarrays are a tool for simultaneously assessing the expression levels of thousands of genes in a single experiment, making them well-suited to understanding the complex gene response patterns and the regulatory pathways involved in human disease. This article reviews the basics of array technology and analysis, and highlights its application to pediatric diseases
HOFF AL, PALERMO TM, SCHLUCHTER M, ZEBRACKI Ket DROTAR D: Longitudinal relationships of depressive symptoms to pain intensity and functional disability among children with disease-related pain|, 2006
Organism:Objective: To examine the longitudinal relationship between depressive symptoms at study entry (T1) on pain intensity (PI) and functional disability over a 1-year period among children with either sickle cell disease (SCD) or juvenile idiopathic arthritis (JIA). Methods: 119 children, ages 8-17 years, completed measures of depression at T1 as well as pain and functional disability at T1, 6-month (T2), and 12-month (T3) follow-ups. Caregivers also rated their child's pain and disability at each time point. General linear mixed modeling was employed to examine longitudinal relationships between study variables. Results: For children with JIA, T1 pain significantly mode rated the effects of T1-depressive symptoms on T2 and T3 pain where T1-depressive symptoms predicted future child-reported pain only when T1 pain was relatively mild. Similarly, T1-depressive symptoms predicted future child-reported disability only when initial reports of disability were relatively low. Only family income significantly predicted T2 and T3 pain in children with SCD. Conclusions: Study findings suggest that T1-depressive symptoms play a role in the longitudinal course of pain symptoms in children with JIA but not in children with SCD. (c) The Author 2005. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved
KAMPHUIS S, HRAFNKELSDOTTIR K, KLEIN MR, DE JAGER W, HAVERKAMP MH, VAN BILSEN JH, ALBANI S, KUIS W, WAUBEN MHet PRAKKEN BJ: Novel self-epitopes derived from aggrecan, fibrillin and matrix-metalloproteinase 3 drive distinct autoreactive T cell responses in juvenile idiopathic arthritis and in health, Arthritis Res.Ther., Vol. 8(6), R178, 2006
Organism:ABSTRACT: Juvenile idiopathic arthritis (JIA) is a heterogeneous autoimmune disease characterized by chronic joint inflammation. Knowing which antigens drive the autoreactive T cell response in JIA is crucial for the understanding of disease pathogenesis and additionally may provide targets for antigen-specific immune therapy. In this study we tested 9 self-peptides derived from joint-related autoantigens for T cell recognition (T cell proliferative responses and cytokine production) in 36 JIA patients and 15 healthy controls. Positive T cell proliferative responses (SI>/=2) to one or more peptides were detected in PBMC of 69% JIA patients irrespective of MHC genotype. The peptides derived from aggrecan, fibrillin and mmp3 yielded the highest frequency of T cell proliferative responses in JIA patients. The aggrecan-peptide induced T cell proliferative responses in both the oligoarticular and polyarticular subtype of JIA that were inversely related with disease duration. The fibrillin-peptide, to our knowledge, is the first identified autoantigen that is primarily recognized in polyarticular JIA patients. Finally, the epitope derived from mmp3 elicited immune responses in both subtypes of JIA and healthy controls. Cytokine production in short-term peptide-specific T cell lines revealed production of IFN-gamma (aggrecan/mmp3), IL-17 (aggrecan) and inhibition of IL-10 production (aggrecan). Herewith we have identified a triplet of self-epitopes, each with distinct patterns of T cell recognition in JIA patients. Additional experiments need to be performed to explore their qualities and role in disease pathogenesis in further detail
KIRWAN J: Early rheumatoid arthritis: combination therapy with disease-modifying antirheumatic drugs and low-dose glucocorticoids?, Nat.Clin.Pract.Rheumatol., Vol. 2(4), 182-183., 2006
Organism:Rheumatic Diseases at the University of Bristol, Bristol, UK johnkirwan@bristolacukFAU - Kirwan, John
Abstract:
KRENN V, MORAWIETZ L, BURMESTER GR, KINNE RW, MUELLER-LADNER U, MULLER Bet HAUPL T: Synovitis score: discrimination between chronic low-grade and high-grade synovitis, Histopathology., Vol. 49(4), 358-364., 2006
Organism:Institute for Pathology, Trier, and Department for Rheumatology, Charite University Hospital, Berlin, Germany krenn@patho-trierdeFAU - Krenn, V
Abstract:AIMS: To standardize the histopathological assessment of synovial membrane specimens in order to contribute to the diagnostics of rheumatic and non-rheumatic joint diseases. METHODS AND RESULTS: Three features of chronic synovitis (enlargement of lining cell layer, cellular density of synovial stroma, leukocytic infiltrate) were semiquantitatively evaluated (from 0, absent to 3, strong) and each feature was graded separately. The sum provided the synovitis score, which was interpreted as follows: 0-1, no synovitis; 2-4, low-grade synovitis; 5-9, high-grade synovitis. Five hundred and fifty-nine synovectomy specimens were graded by two independent observers. Clinical diagnoses were osteoarthrosis (n=212), post-traumatic arthritis (n=21), rheumatoid arthritis (n=246), psoriatic arthritis (n=22), reactive arthritis (n=9), as well as controls (n=49) from autopsies of patients without joint damage. Median synovitis scores when correlated with clinical diagnoses were: controls 1.0, osteoarthritis 2.0, post-traumatic arthritis 2.0, psoriatic arthritis 3.5, reactive arthritis 5.0 and rheumatoid arthritis 5.0. The scores differed significantly between most disease groups, especially between degenerative and rheumatic diseases. A high-grade synovitis was strongly associated with rheumatic joint diseases (P<0.001, sensitivity 61.7%, specificity 96.1%). The correlation between the two observers was high (r=0.941). CONCLUSION: The proposed synovitis score is based on well-defined, reproducible histopathological criteria and may contribute to diagnosis in rheumatic and non-rheumatic joint diseases
KRUITHOF E, VAN dB, V, DE RYCKE L, VANDOOREN B, JOOS R, CANETE JD, TAK PP, BOOTS ANNEMIEKE MH, VEYS EMet BAETEN D: Distinct synovial immunopathologic characteristics of juvenile-onset spondylarthritis and other forms of juvenile idiopathic arthritis, Arthritis & Rheumatism, Vol. 54, 2594-2604., 2006
Organism:Univ Amsterdam, Acad Med Ctr, Div Clin Immunol and Rheumatol, Meibergdreef 9, NL-1105 AZ Amsterdam, Netherlands Netherlands
Abstract:Objective. To characterize the synovial immunopathologic features of juvenile-onset spondylarthritis (SpA) in relation to adult SpA and other forms of juvenile idiopathic arthritis (JIA).Methods. Synovial biopsy samples were obtained from 10 patients with juvenile-onset SpA, 23 with adult SpA, 19 with rheumatoid arthritis (RA), 8 with juvenile polyarthritis, and 12 with juvenile oligoarthritis. Synovial immunopathologic features were studied by extensive histologic and immunohistochemical analyses.Results. Synovitis in juvenile SpA was characterized by marked lining layer hyperplasia, clear hy pervascularity, and pronounced inflammatory cell infiltration with lymphocytes and macrophages, independent of disease duration or time of sampling. The immunopathologic features of juvenile SpA resembled those of adult SpA in terms of hypervascularity and absence of RA-specific intracellular citrullinated proteins and HLA-DR4/human cartilage glycoprotein 39(263-275) complexes, but differed markedly by a stronger lining layer hyperplasia and lower numbers of CD163+ macrophages. Accordingly, class prediction analysis failed to classify juvenile SpA synovitis in the SpA group. Comparison of juvenile SpA with other RA subtypes showed a broad overlap, with the exception of slightly lower vascularity in juvenile polyarthritis and higher inflammatory cell infiltration in juvenile oligoarthritis. Unsupervised clustering analysis identified a subgroup of samples characterized by high plasma cell infiltration, which corresponded with active, longstanding RA, mostly of the oligoarthritis subtype.Conclusion. Despite some similarities with adult SpA, the findings with regard to lining layer hyperplasia and CD163+ macrophage infiltration are indicative of important differences in the synovial immunopathologic features of juvenile-onset SpA. The partial overlap with other JIA subtypes emphasizes the need for further biologic characterization of JIA in order to define pathophysiologic, rather than phenotypic, subgroups
LEE WS, HAFEEZ A, HASSAN H, RAJA NSet PUTHUCHEARY SD: Focal non-typhoidal Salmonella infections from a single center in Malaysia, Southeast Asian J.Trop.Med.Public Health., Vol. 36(3), 678-682., 2005
Organism:Department of Pediatrics, University of Malaya Medical Center, Kuala Lumpur, Malaysia leews@umedumyFAU - Lee, W S
Abstract:A retrospective review of patients with focal non-typhoidal Salmonella (NTS) infection was performed to determine its features and outcome. All patients with focal NTS infection admitted to the University of Malaya Medical Center, Malaysia, from 1993 to 2002 were studied. More than half (58%) of the 35 cases (54% male, median age 39 years, range 1.5 months to 79 years) were immunocompromized or had chronic medical conditions. One-third of the patients (34%) had superficial infections (lymphadenitis or subcutaneous tissue infection) and all recovered with antimicrobial therapy alone. Deep infections (66%) noted were: meningitis (9%), osteomyelitis or arthritis (26%), abscesses of the gastrointestinal tract or adjacent organs (20%), and others (11%). Deep infections were more likely to occur in the extremes of age (<6 months or >60 years, p< 0.04), associated with adverse outcomes with an overall mortality rate of 9%, or required major surgery (15%)
LORENTZEN AR, SMESTAD C, EKSTROM PO, OTURAI AB, AKESSON E, SAARELA J et MYHR K-M: The role of SH2D2A gene polymorphisms in development of multiple sclerosis, 528-529., 08-06-2001
LOVELL DJ: Update on treatment of arthritis in children - new treatments, new goals, Bull.Hosp.Jt.Dis., Vol. 64(1-2), 72-76., 2006
Organism:Although improved in recent years, the outcome for children who have juvenile idiopathic arthritis (JIA) is still less than ideal. Between 25% and 70% of children with JIA will still have active arthritis 10 years after disease onset, and over 40% will enter adulthood with active arthritis. Based on older publications, the fatality rate for JIA is 4- to 14-fold greater than an age- and sex-matched healthy population, and up to 39% of patients are significantly incapacitated, either wheelchair-bound or bedridden (Steinbrocker Classes III or IV). The hope is that recent changes in treatment approaches will result in marked improvement in long-term functional outcomes, although this has yet to be proven. JIA-associated chronic uveitis has a high frequency of serious complications: 20% develop cataracts, 19% glaucoma, and 16% band keratopathy. The anti-TNF biologics have all been tested in children with polyarticular JIA in blinded, placebo-controlled clinical trials, with over 70% in each trial demonstrating response that has been sustained in longer term follow-up studies. In systemic JIA approximately 50% respond to anti-TNF agents, but in many the response is not sustained. Openlabel studies have shown promising results for biologic therapies that block interleukins 1 and 6 in systemic JIA
MACRAE VE, WONG SC, FARQUHARSON Cet AHMED SF: Cytokine actions in growth disorders associated with pediatric chronic inflammatory diseases (Review), Int.J.Mol.Med., Vol. 18(6), 1011-1018., 2006
Organism:Division of Gene Function and Development, Roslin Institute, Roslin, Midlothian EH25 9PS, UK vickymacrae@bbsrcacukFAU - Macrae, V E
Abstract:Growth disorders are commonly observed in children suffering from chronic inflammatory diseases such as Juvenile Idiopathic Arthritis (JIA) and Inflammatory Bowel Disease (IBD). These disorders range from general growth retardation to local acceleration of growth in the affected limb and are associated with the increased production of pro-inflammatory cytokines. In this article, we review how cytokines influence child growth by exerting a local effect at the level of the growth plate, and through systemic effects throughout the whole body
MIER R: Recent therapeutic advances in the care of children with chronic arthritis|, 2006
Organism:PURPOSE OF REVIEW: This review is timely and relevant because we are in the midst of a revolution in the medical management of childhood and adolescent chronic arthritis. This revolution has been fueled by two things. The first is a philosophical change such that medications higher on the therapeutic pyramid and with greater potential toxicity have been initiated earlier and used more aggressively. The second is the greater availability of therapeutic choices with proven effectiveness, beginning with methotrexate in 1992, and continuing to the present and into the future with an entire new family of therapies, termed the biologics. RECENT FINDINGS: Recent advances in the care of children with arthritis have included randomized, controlled trials demonstrating the effectiveness of etanercept, meloxicam and leflunomide and open label studies suggesting the therapeutic possibilities of several biologics including infliximab, anakinra and tocilizumab. SUMMARY: Recent work in this arena is summarized, including therapeutic advances both medical and surgical, as well as changes in the classification of the chronic pediatric inflammatory arthritides. Improved therapeutic options for children with juvenile rheumatoid arthritis will translate into improved future outcomes as they become increasingly available to physicians and families. (c) 2006 Lippincott Williams & Wilkins, Inc
PAUL-PLETZER K: Tocilizumab: Blockade of interleukin-6 signaling pathway as a therapeutic strategy for inflammatory disorders, Drugs Of Today, Vol. 42, 559-576., 2006
Organism:Prous Sci, Med Informat Dept, Provenca 388, Barcelona 08025, Spain Spain
Abstract:Interleukin (IL)-6 contributes to a myriad of physiologic and pathophysiologic processes. Among its many physiologic functions, IL-6 plays an active role in immunology, inflammatory responses, bone metabolism, arthritis and neoplasia. Overproduction of IL-6 has been implicated in the disease pathology of several inflammatory and autoimmune disorders, including rheumatoid arthritis, Castleman's disease, Crohn's disease and systemic-onset juvenile idiopathic arthritis. Interception of the IL-6 signaling pathway could thus represent a new treatment option for these diseases, given their refractory status to conventional therapy. Clinical studies with tocilizumab, a humanized monoclonal anti-IL-6 receptor antibody, have been undertaken to explore this option. Current short-term results indicate that tocilizumab dramatically improves disease activity and is well tolerated. Further long-term safety and efficacy studies are needed to confirm the therapeutic benefit of this antibody in inflammatory and autoimmune disorders. (c) 2006 Prous Science. All rights reserved
PAY S: The use of anti-TNF drugs in rheumatic disorders: Medical education|, 2006
Organism:TNF-alpha is a proinflammatory cytokine, which plays a crucial role in the pathogenesis of rheumatoid arthritis and a number of other inflammatory diseases. Recently, anti-TNF drugs, which antagonize the biological activity of TNF-alpha have started to be used in the treatment of rheumatological disorders. There are 3 anti-TNF drugs currently used in our country and throughout the world for this purpose. Of those, etanercept (Enbrel(R)) is a dimeric TNF receptor, and infliximab (Remicade(R)) and adalimumab (Humira(R)) are human-murine chimeric (75% human and 25% murine) and human monoclonal antibodies, respectively. In Turkey, infliximab was licensed for the treatment of rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, and Crohn's disease, etanercept for rheumatoid arthritis, ankylosing spondylitis. psoriasis, psoriatic arthritis and juvenile idiopathic arthritis and adalimumab only for rheumatoid arthritis. Although their antiinflammatory activities are dependent on the blockade of TNF-alpha, they have different effects on the immune system and inflammation due to their different structures and physiological features, which determine both their clinical indicatio ns and side effects. As in all other drugs, side effects as well as clinical activity are very crucial issues. Copyright (c) 2006 by Turkiye Klinikleri
PETTIT AR, WALSH NC, MANNING C, GOLDRING SRet GRAVALLESE EM: RANKL protein is expressed at the pannus-bone interface at sites of articular bone erosion in rheumatoid arthritis, Rheumatology (Oxford)., Vol. 45(9), 1068-1076., 2006
Organism:Institute for Molecular Bioscience, University of Queensland, Brisbane, QLD 4072, AustraliaFAU - Pettit, A R
Abstract:OBJECTIVES: Receptor activator of NF-kappaB ligand (RANKL) and osteoprotegerin (OPG) have been demonstrated to be critical regulators of osteoclast generation and activity. In addition, RANKL has been implicated as an important mediator of bone erosion in rheumatoid arthritis (RA). However, the expression of RANKL and OPG at sites of pannus invasion into bone has not been examined. The present study was undertaken to further elucidate the contribution of this cytokine system to osteoclastogenesis and subsequent bone erosion in RA by examining the pattern of protein expression for RANKL, OPG and the receptor activator of NF-kappaB (RANK) in RA at sites of articular bone erosion. METHODS: Tissues from 20 surgical procedures from 17 patients with RA were collected as discarded materials. Six samples contained only synovium or tenosynovium remote from bone, four samples contained pannus-bone interface with adjacent synovium and 10 samples contained both synovium remote from bone and pannus-bone interface with adjacent synovium. Immunohistochemistry was used to characterize the cellular pattern of RANKL, RANK and OPG protein expression immediately adjacent to and remote from sites of bone erosion. RESULTS: Cellular expression of RANKL protein was relatively restricted in the bone microenvironment; staining was focal and confined largely to sites of osteoclast-mediated erosion at the pannus-bone interface and at sites of subchondral bone erosion. RANK-expressing osteoclast precursor cells were also present in these sites. OPG protein expression was observed in numerous cells in synovium remote from bone but was more limited at sites of bone erosion, especially in regions associated with RANKL expression. CONCLUSIONS: The pattern of RANKL and OPG expression and the presence of RANK-expressing osteoclast precursor cells at sites of bone erosion in RA contributes to the generation of a local microenvironment that favours osteoclast differentiation and activity. These data provide further evidence implicating RANKL in the pathogenesis of arthritis-induced joint destruction
POPE E, JANSON A, KHAMBALIA Aet FELDMAN B: Childhood acquired lipodystrophy: a retrospective study, J.Am.Acad.Dermatol., Vol. 55(6), 947-950., 2006
Organism:Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada elenapope@sickkidscaFAU - Pope, Elena
Abstract:OBJECTIVE: We sought to describe the clinical characteristics and complications of children with acquired lipodystrophy (LD). METHODS: We conducted a retrospective chart review at a tertiary, academic children's hospital of children clinically given a diagnosis of acquired LD between January 1997 and December 2004. RESULTS: During the study period, 23 patients were identified. Their mean age at diagnosis was 9.74 +/- 3.98 years. Of patients, 61% were girls. The length of the follow-up was 4.8 +/- 3.5 years from the time of LD diagnosis. Of patients, 30% had evidence of localized disease (7 of 23), 26% (6 of 23) had localized partial disease, and 44% (10 of 23) had generalized LD. The most common underlying diagnosis was dermatomyositis (78%), alone or in association with other autoimmune diseases (juvenile rheumatoid arthritis 17%). Panniculitis with autoimmunity was noted in 17% of the patients. More than half of the patients had at least one complication attributable to LD such as acanthosis nigricans (22%), hyperpigmentation (22%), hepatomegaly (13%), hypertension (13%), protuberant abdomen (9%), and hyperlipidemia (4%). The only predictor for development of complications was the age of diagnosis of LD, with younger age being associated with increased risk (7 vs 12 years, P = .04). LIMITATIONS: Limitations were inherent to the retrospective design. CONCLUSIONS: Childhood acquired LD is seen more frequently in the context of autoimmunity. Affected children should be monitored for the development of complications, particularly if given a diagnosis of LD at a younger age
PRICE JR FWet ZIEMBA SL: Placement of a collagen glaucoma drainage device to control intraocular pressure and chronic iritis secondary to juvenile rheumatoid arthritis|, 2002
Organism:A patient with juvenile rheumatoid arthritis and chronic iritis is reported with intraocular pressure near 30 mm Hg and previous episodes of intraocular pressure as high as 50 mm Hg despite maximally tolerated medical therapy. Because of the potential risk involved with a full-thickness filtration procedure, it was decided that a nonpenetrating deep sclerectomy would be appropriate, followed by placement of a collagen glaucoma drainage device to maintain aqueous outflow. Immediately postoperatively, intraocular pressure was stabilized. At 24 months postoperatively, intraocular pressure was wel l controlled at 15 mm Hg with patient receiving only Lotemax. No significant complications were noted at any point in the postoperative course. Because of the patient's predisposition for serious complications frequently associated with trabeculectomy, nonpenetrating deep sclerectomy with the collagen glaucoma drainage device was an effective alternative for this patient
RAVELLI Aet MARTINI A: Remission in juvenile idiopathic arthritis, Clin.Exp.Rheumatol., Vol. 24(6 Suppl 43), S105-S110, 2006
Organism:Dirigente Medico, Pediatria II, Istituto di Ricovero e Cura a Carattere Scientifico G Gaslini, Genova, ItalyFAU - Ravelli, A
Abstract:Until recently, no uniform and widely accepted criteria for defining remission in juvenile idiopathic arthritis (JIA) were available. In recent years, a set of preliminary criteria for clinical remission in JIA was developed through an international collaborative effort. These criteria enable the classification of patients in the states of inactive disease, clinical remission with medication, and clinical remission without medication. The first phase of the validation process of the criteria, which was accomplished recently, established that they are feasible and have good face, content and construct validity, and strong discriminant properties. A few studies have applied the new remission criteria in series of patients with JIA, with results that concur with those of previous surveys in showing that only a few patients with JIA have a chance of remaining in long-term remission status without medications. These findings highlight the critical need for therapies that have the capacity to induce sustained complete disease control of JIA
SAINT MBet DE BANDT M: Vasculitides induced by TNFalpha antagonists: a study in 39 patients in France, Joint Bone Spine., Vol. ., 2006
Organism:Service de Rhumatologie, Robert Ballanger Hospital, 93600, Aulnay sous Bois, France
Abstract:TNFalpha antagonists are effective in the treatment of chronic inflammatory joint disease. Despite a good overall safety profile, they can induce a number of adverse effects, including autoimmunity and infections. A link between TNFalpha antagonists and vasculitides has been suggested. METHODS: Between December 2004 and January 2005, a nationwide survey was conducted among 1200 hospital-based rheumatologists and internists in France, who were asked to report cases of vasculitis in patients taking TNFalpha antagonists. RESULTS: The survey identified 39 cases (32 women) of vasculitis during TNFalpha antagonist therapy. The joint disease was rheumatoid arthritis (RA) in 34 patients (including four without rheumatoid factor), juvenile idiopathic arthritis in two patients, ankylosing spondylitis in two patients, and psoriatic arthritis in one patient. Mean disease duration was 14.1+/-8.7 years. The TNFalpha antagonist was etanercept in 21 patients, infliximab in 15, adalimumab in 2, and another drug in 1; mean treatment duration was 9.6 months. The manifestations of vasculitis involved the skin (n=32); peripheral nervous system (n=9); kidney (n=7); central nervous system (n=3); pleura (n=2), pericardium (n=2); and the lung, gallbladder, and heart (n=1 each). Antinuclear factor (ANF) was present in 22 patients, hypocomplementemia in 6, and antineutrophil cytoplasmic antibody in 5. Histology (30 biopsies from 27 patients) showed nonnecrotizing vasculitis in 12 patients, necrotizing vasculitis in 7, an inflammatory dermal infiltrate without vasculitis in 3, extravascular necrotic granulomas in 2, chilblain lupus in 1, and cicatricial fibro-inflammatory changes in 1. Renal biopsy in three patients showed extracapillary glomerulonephritis with IgA deposits (n=2) or active floccular necrosis against a background of glomerular sclerosis (n=1). TNFalpha antagonist therapy was stopped in 33 patients, among whom 18 recovered without further treatment and 14 required high-dose glucocorticoids and/or immunosuppressant therapy, which ensured symptom resolution within a few weeks. The remaining patient died with multiple organ failures. DISCUSSION: The relative contributions of TNFalpha antagonist therapy and of the underlying disease to the development of vasculitis cannot be determined. Features that suggest a causal link between TNFalpha antagonists and vasculitis include the short time from TNFalpha antagonist initiation to vasculitis onset; the favorable response to discontinuation of TNFalpha antagonist therapy; and the development of systemic vasculitis in patients with rheumatoid factor-negative RA, in adults with juvenile-onset arthritis, and in patients with spondyloarthropathies
SCHERER HUet BURMESTER GR: [Biologicals in the treatment of rheumatic diseases], Dtsch.Med.Wochenschr., Vol. 131(41), 2279-2285., 2006
Organism:Klinik fur Rheumatologie und klinische Immunologie, Charite-Universitatsmedizin Berlin ulrichscherer@charitedeFAU - Scherer, H U
Abstract:
SHAABAN FA, METWALLY IM, SAMY SM, SALAMA IIet HASSANIN AI: Health related quality of life, disease activity, severity and coping in juvenile rheumatoid arthritis|, 2006
Organism:The aim of the present study was to assess the health related quality of life in children and adolescences suffering from Juvenile Rheumatoid Arthritis. It also aimed at studying its relationship with disease activity, subtypes and important clinical parameters and accumulated damage and the usefulness of this tool to indicate the improvement or worsening of the patient's clinical condition in daily practice and physical capacity. This study is a case control study that comprised 52 children and adolescent suffering from Juvenile Rheumatoid Arthritis (JRA) in the age range 5-18 years classified as polyarticular (46.2%), systemic onset (26.9%) and pauciarticular (26.9 %) attending the rheumatology and rehabilitation outpatient clinic at Cairo University Hospitals and 61 healthy children as a control. All patients underwent clinical rheumatologic and laboratory assessments. A questionnaire including demographic and anthropometric variables (age, sex, weight and height....), clinical variables (onset, age at onset, subtype, course, duration, morning stiffness....) and laboratory variables (ESR, ANA, RF, CRP and HB) was fulfilled. The outcome measure and health assessment were evaluated by two tools: The Childhood Health Assessment Questionnaire (CHAQ) which is a disease specific instrument that measures functional ability in daily living activities in children with juvenile rheumatoid arthritis and The Chi ld Health Questionnaire (CHQ-PF 50) which is a generic health instrument designed to capture the physical and psychosocial well being of children independently from the underlying disease. The disability index (DI), Physical and Psychosocial scores (PhS and PsS) were then drawn out and correlated to the variables reflecting disease activity. There was a significant difference for almost all measures of disease activity being significantly higher in the systemic onset compared to the other two subtypes (p<0.005). The CHAQ significantly discriminated between healthy subjects and JRA patients [Dl= 0.1+/-0.2 vs 1.1:+/-0.8, respectively (p<0.01)]. It also discriminated between the three subtypes of JRA where the DI was significantly higher in the systemic onset type (1.4+/-0.7), followed by the polyarticular (1.2+/-0.7) then the pauciarticular subtype (0.7+/-0.6 and p<0.05). The CHQ discriminated clinically healthy subjects from JRA patients, with the patients having significantly lower physical and psychosocial well-being scores when compared to their healthy peers (PhS = 46.8+/-18.8 vs 78.0+/-14.2), (PsS = 57.3+/-17.2 vs 68.9+/-15.6), respectively and (p = 0.000). The CHQ proved to be less able to discriminate clinically between the different JRA subtypes. The CHAQ and the CHQ proved to be reliable and valid tools for the functional, physical and psychological assessment of children with JRA and this was confirmed by the strong correlation between the DI, PhS and PsS and the variables reflecting disease activity
SIJSSENS KM, SWART JFet DE BOER JH: Two children with arthritis and uveitis: The clinical importance of ophthalmologic screening in children with juvenile arthritis|, 2006
Organism:Two girls, aged 8 and 13 years, with a history of arthritis presented with already advanced uveitis. Neither girl had been screened for uveitis by an ophthalmologist according to the recommendations of the American Academy of Pediatrics. Both patients had extensive posterior synechiae (adhesions between lens and iris) at their first ophthalmologic examination, which is associated with an unfavourable prognosis. At the last follow-up, the first patient had undergone seven ocular operations: five for glaucoma and two cataract surgeries. Uveitis occurs in 20% of children with juvenile idiopathic arthritis. The visual outcome of uveitis is negatively influenced by the pr esence of ocular complications at the first ophthalmologic examination. Ophthalmologic screening of children with juvenile idiopathic arthritis is very important because it is a common cause of uveitis in childhood, the uveitis is usually asymptomatic, and there are sight-threatening complications. Screening is always important, even when the arthritis is in remission
SILVA CAA, SILVA CHM, ROBAZZI TCMV, LOTITO APN, MENDRONI JA, JACOB CMAet KISS MHB: Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis|, 2004
Organism:
SIMSEK B, BAYAZIT AK, ERGIN M, SORAN M, DURSUN Het KILINC Y: Renal amyloidosis in a child with sickle cell anemia, Pediatr.Nephrol., Vol. 21(6), 877-879., 2006
Organism:Department of Pediatric Nephrology, Cukurova University, Balcali, Adana 01120, Turkey drmago@hotmailcomFAU - Simsek, Behcet
Abstract:The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood
SMOLEWSKA E, STANCZYK J, ROBAK Tet SMOLEWSKI P: Inhibited apoptosis of synovial fluid lymphocytes in children with juvenile idiopathic arthritis is associated with increased expression of myeloid cell leukemia 1 and XIAP proteins, Rinsho Ganka, Vol. 33, 1684-1690., 2006
Organism:Med Univ Lodz, Inst Pediat, Dept Pediat Cardiol, Sporna 36-50, PL-91738 Lodz, Poland Poland
Abstract:Objective. Inhibited apoptosis of lymphocytes present in synovial fluid (SFL) and persistently infiltrating synovial tissue may be crucial in the pathogenesis of rheumatoid arthritis (RA). Similarly, this may be the case in juvenile idiopathic arthritis (JIA). Little is known about lymphocyte apoptosis in this disease. Recently, we reported significantly enhanced apoptosis of peripheral blood lymphocytes (JIA-PBL) compared to synovial fluid (JIA-SFL) or healthy lymphocytes, with downregulation of p53 in JIA-SFL. In this study we assessed other possible molecular mechanisms of this phenomenon.M ethods. PBL from 31 children with JIA and 26 healthy children were examined. SFL obtained from 18 patients was also studied. Apoptosis was assessed by TdT-mediated dUTP-biotin nick-end labeling (TUNEL) method. Expression of several apoptosis-regulating proteins was analyzed, including myeloid cell leukemia I (Mcl-1), cross-linked inhibitor of apoptosis (XIAP), FLICE-inhibitory protein (FLIP), or Bcl-xL inhibitors and proapoptotic p53, Bcl-w, Bak, and Bid.Results. We found significant overexpression of Mcl-1 and XIAP in JIA-SFL (p < 0.001 and p < 0.02, respectively). Expression of Mcl-1 and XIAP in SFL correlated inversely with the apoptotic index (p < 0.002 and p < 0 .01, respectively). FLIP expression was also distinctly higher in SFL than in JIA-PBL; however, the difference was not statistically significant (p = 0.061). No statistically significant differences were found in the expression of other proteins between SFL and PBL.Conclusion. This is the first study showing that upregulation of anti-apoptotic Mcl-1 and XIAP proteins, along with downregulation of p53 protein, is correlated with inhibition of JIA-SFL apoptosis
STABILE A, AVALLONE L, COMPAGNONE A, ANSUINI V, BERTONI Bet RIGANTE D: Focus on juvenile idiopathic arthritis according to the 2001 Edmonton revised classification from the International League of Associations for Rheumatology: an Italian experience, Eur.Rev.Med.Pharmacol.Sci., Vol. 10(5), 229-234., 2006
Organism:Department of Pediatric Sciences, Universita Cattolica Sacro Cuore, Rome, Italy astabile@rmunicattitFAU - Stabile, A
Abstract:OBJECTIVE: To classify a cohort of Italian patients categorized as affected with juvenile idiopathic arthritis (JIA) according to the revised 2001 Edmonton International League of Associations for Rheumatology (ILAR) criteria. METHODS: Eighty-five patients with JIA firstly framed depending on traditional criteria during the last ten years were reallocated according to the JIA revised criteria proposed in 2001 by ILAR in Edmonton. RESULTS: The revision consented to define the following distribution of patients: 28.2% systemic, 55.3% oligoarticular and 11.8% polyarticular forms; only one child was defined as having psoriatic arthritis, one child with enthesitis-associated arthritis and two with the undifferentiated form of JIA. DISCUSSION: The 97.6% of the recruited patients were strictly classified according to the Edmonton ILAR criteria, demonstrating a very low number of patients whose arthritis could not be assigned to any JIA category due to unfulfillment of the required criteria
SUPPIAH V, ROONEY Met VANDENBROECK K: Polymorphisms in the interleukin-4 and IL-4 receptor genes modify risk for chronic inflammatory arthropathies in women|, 2006
Organism:Rheumatoid and juvenile idiopathic arthritis (RA, JIA) are chronic inflammatory arthropathies with polygenic autoimmune background. We analysed the IL-4 +33 C/T and IL-4R Q551R single nucleotide polymorphisms (SNPs) in 294 RA, 72 JIA and 165 controls from Northern Ireland. Analysis of the individual phenotypes (RA or JIA) showed that both the IL-4 +33 TT (P = 0.02; OR: 0.25, 95% CI: 0.07-0.87) and the IL-4R Q551R CC genotypes (P = 0.001; OR: 0.19, 95% CI: 0.06-0.56) were exclusively decreased in female RA patients compared to female controls. Similar non-significant trends were observed in female JIA patients (OR: 0.25, 95% CI: 0.03-2.11 and OR: 0.31, 95% CI: 0.07-1.47, respectively). Analysis of the common phenotype (inflammatory arthropathy; i.e. JIA and RA combined) corroborated the unique association of these polymorphisms with female inflammatory arthropathy (P = 0.013 and 0.002, respectively). This is the first demonstration of sex-specific association of the two foremost genes of the IL-4 signalling cascade with chronic inflammatory arthropathies. (c) 2006 Elsevier Inc. All rights reserved
TAKATA Y, MATSUI Y, HAMADA D, GOTO T, KUBO T, EGAWA H, NAKANO S, SHINOMIYA F, INOUE H, ITAKURA Met YASUI N: The alpha 2 type IX collagen gene tryptophan polymorphism is not associated with rheumatoid arthritis in the Japanese population, Clin.Rheumatol., Vol. 25(4), 491-494., 2006
Organism:Department of Orthopedics, Institute of Health Biosciences, The University of Tokushima Graduate School, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, JapanFAU - Takata, Yoichiro
Abstract:The aim of this study was to investigate whether the alpha 2 type IX collagen (COL9A2) polymorphism that introduces tryptophan residue into the collagen triple-helix is a marker of susceptibility to, or severity of, rheumatoid arthritis (RA). The study included 749 Japanese patients with RA. One hundred twenty-four unrelated healthy individuals served as the control subjects. The relationship between the COL9A2 gene polymorphism and clinical manifestations of RA was evaluated. For the number of subjects positive for COL9A2 tryptophan polymorphism, there was no statistically significant difference between RA patients and normal controls. Furthermore, we did not detect any association of COL9A2 tryptophan polymorphism with disease status, least erosive subset, more erosive subset, or mutilating disease. The lack of association of COL9A2 tryptophan polymorphism with RA and the clinical findings in our study implies that the polymorphism may not function as a candidate gene marker for screening RA patients
TSUMI E, LIFSHITZ Tet ABU-SHAKRA M: Eye involvement in rheumatoid arthritis in children and adults|, 2006
Organism:Rheumatoid arthritis (RA) and juvenile rheumatoid arthritis (JRA) are systemic autoimmune diseases characterized by synovitis and a wide range of extraarticular manifestations. Ocular involvement occurs frequently in both diseases and it may affect all layers of the eyes. This review summarizes the clinical manifestations, the diagnostic tools and the therapeutic modalities of the various ocular features of patients with juvenile and adult rheumatoid arthritis
TYC VLet THROCKMORTON-BELZER L: Smoking rates and the state of smoking interventions for children and adolescents with chronic illness, Pediatrics, Vol. 118, E471-E487, 2006
Organism:St Jude Childrens Hosp, Div Behav Med, 332 N Lauderdale St, Memphis, TN 38105 USA USA
Abstract:Engaging in smoking is particularly risky for children and adolescents with chronic illness whose health status is already compromised because of disease- and treatment-related complications. Yet, some of these youngsters smoke at rates at least comparable to those of their healthy peers. To date, few randomized smoking-prevention and cessation trials have been conducted in children with chronic medical problems. In this review we report on the smoking rates among youngsters with chronic illness, identify specific disease- and treatment-related complications that can be exacerbated by smoking, examine risk factors associated with tobacco use among medically compromised youngsters, and review smoking interventions that have been conducted to date with pediatric populations in the health care setting. The following chronic illnesses are included in this review: asthma, cystic fibrosis, cancer, sickle cell disease, juvenile-onset diabetes, and juvenile rheumatoid arthritis. Objectives for a tobacco-control agenda and recommendations for future tobacco studies in chronically ill pediatric populations are provided. Finally, tobacco counseling strategies are suggested for clinicians who treat these youngsters in their practices
WILSON AS, GOODALL JE, AMBROSINI G, CARRUTHERS DM, CHAN H, ONG SG, GORDON Cet YOUNG SP: Development of an interactive learning tool for teaching rheumatology - a simulated clinical case studies program, Rheumatology (Oxford), Vol. 45, 1158-1161., 2006
Organism:Univ Birmingham, Sch Med, Div Immun and Infect, Dept Rheumatol, Birmingham B15 2TT, W Midlands, UK UK
Abstract:Objectives. To promote independent self-study involving problem solving and decision analysis in the undergraduate medical curriculum, we have developed a series of interactive web-based clinical case studies.Methods. An initial needs assessment was performed to determine students' attitudes to e-learning. From these results we designed a series of 30 interactive case studies for delivery from a web-server.Results. A survey of 59 undergraduate students believed that online teaching resources were a useful supplement to existing teaching and they could see a positive use for e-learning. The int eractive case studies program was well received by a broad range of respondents (n=84) of different abilities and backgrounds who felt that the program was realistic and clearly presented in an intuitive manner.Conclusions. The recent increases in numbers of medical undergraduates, the trend towards student-centred learning and the emphasis on patient-related teaching means a great pressure on teachers and resources in medical schools. The case studies program we have developed was effective and well received by both biomedical and medical students. This approach may provide a way to increase the exposure of students to clinical cases involving interactive diagnostic and treatment procedures, that mimic real-world scenarios, but with fewer resource implications
WULFFRAAT NM, DE KLEER IMet PRAKKEN B: Refractory juvenile idiopathic arthritis: using autologous stem cell transplantation as a treatment strategy, Expert.Rev.Mol.Med., Vol. 8(26), 1-11., 2006
Organism:Department of Pediatric Immunology, University Medical Center Utrecht, 'Wilhelmina Children's Hospital', 3508 AB Utrecht, The Netherlands
Abstract:Cellular immune therapy for severe autoimmune diseases can now be considered when such patients are refractory to conventional treatment. The use of autologous stem cell transplantation (ASCT) to treat human autoimmune diseases has been initiated following promising results in a variety of animal models. Anecdotal observations have been made of autoimmune disease remission in patients who have undergone allogeneic bone marrow transplantation as a result of coincidental haematological malignancies. The possibility of inducing immunological self-tolerance by ASCT is particularly attractive as a means for treating juvenile idiopathic arthritis (JIA). In this disease, ASCT restores self-tolerance both through a cell-intrinsic mechanism, involving the reprogramming of autoreactive T cells, and through a cell-extrinsic mechanism, involving a renewal of the immune balance between CD4+CD25+ regulatory T cells and other T cells. This review describes the clinical results of ASCT performed for this disease and the possible underlying immunological mechanisms
YILDIZ Bet KURAL N: IgG1 deficiency and high IgA level with juvenile idiopathic arthritis, Eur.J.Pediatr., Vol. ., 2006
Organism:Department of Paediatrics, Faculty of Medicine, Eskisehir Osmangazi University, 26480, Eskisehir, Turkey, bilalyn@yahoocom
Abstract:
ZHENG WJ, ZHAO Y, TANG FLet DONG Y: [A study of antiendothelial cell antibodies in Behcet's disease], Zhonghua Nei Ke.Za Zhi., Vol. 44(12), 910-913., 2005
Organism:Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, ChinaFAU - Zheng, Wen-jie
Abstract:OBJECTIVE: To investigate the clinical significant of antiendothelial cell antibodies (AECA) in Behcet's disease. METHODS: With human umbilical vein endothelial cell as substrate cell, sera from 59 Behcet's disease patients were detected for the presence of AECA by using fixed cell-ELISA. The associations of AECA to clinical disease activity were analyzed. In addition, Sera from other 70 systemic vasculitis (including 28 Takayasu arteritis, 20 Wegener's granulomatosis, 8 polyarteritis nodosa, 9 microscopic polyangiitis, 5 Churg-Strauss syndrome), 57 systemic lupus erythematosus (SLE), 25 rheumatoid arthritis and 85 healthy donors were screened for AECA. The associations of AECA to clinical disease activity were analyzed. RESULTS: The prevalence of AECA by human umbilical vein endothelial cell cell-ELISA was 47.5% in Behcet's disease. Compared with patients with rheumatoid arthritis (4.0%) and normal group (1.2%), AECA were more frequently found in patients with Behcet's disease, other systemic vasculitis (68.6%) and SLE (45.7%) (P < 0.01). The positive result on pathergy testing was more frequently found in AECA-positive patient with Behcet's disease (P < 0.05). After the treatment, patients with active disease entering remission showed a decrease in both AECA titers and the mean level of ESR (P < 0.05). CONCLUSIONS: AECA could be found more frequently in patients with Behcet's disease, associated closely with disease activity, which suggest that AECA may be used as an index in monitoring disease activity and effect of therapy in Behcet's disease. But AECA could also be found in other systemic vasculitis and SLE