Bibliography May 05
- BOKAREWA M, NAGAEV I, DAHLBERG L, SMITH U, TARKOWSKI A: Resistin, an adipokine with potent proinflammatory properties. J Immunol 174:9 5789-5795, 2005
Organism: Department of Rheumatology and Inflammation Research, The Sahlgrenska Academy at Goteborg University, Sweden mariabokarewa@rheumaguseFAU - Bokarewa, Maria
Abstract: The adipokine resistin is suggested to be an important link between obesity and insulin resistance. In the present study, we assessed the impact of resistin as inflammatogenic cytokine in the setting of arthritis. In vitro experiments on human PBMC were performed to assess cytokine response and transcription pathways of resistin-induced inflammation. Proinflammatory properties of resistin were evaluated in animal model by intra-articular injection of resistin followed by histological evaluation of the joint. Levels of resistin were assessed by ELISA in 74 paired blood and synovial fluid samples of patients with rheumatoid arthritis. Results were compared with the control group comprised blood samples from 34 healthy individuals and 21 synovial fluids from patients with noninflammatory joint diseases. We now show that resistin displays potent proinflammatory properties by 1) strongly up-regulating IL-6 and TNF-alpha, 2) responding to TNF-alpha challenge, 3) enhancing its own activity by a positive feedback, and finally 4) inducing arthritis when injected into healthy mouse joints. Proinflammatory properties of resistin were abrogated by NF-kappaB inhibitor indicating the importance of NF-kappaB signaling pathway for resistin-induced inflammation. Resistin is also shown to specifically accumulate in the inflamed joints of patients with rheumatoid arthritis and its levels correlate with other markers of inflammation. Our results indicate that resistin is a new and important member of the cytokine family with potent regulatory functions. Importantly, the identified properties of resistin make it a novel and interesting therapeutic target in chronic inflammatory diseases such as rheumatoid arthritis - BRIK R, GEPSTEIN V, BERKOVITZ D: Low-dose methotrexate treatment for oligoarticular juvenile idiopathic arthritis nonresponsive to intra-articular corticosteroids. Clin Rheumatol .: 2005
Organism: Pediatric Department and Pediatric Rheumatology Unit, Technion Faculty of Medicine, Meyer Children's Hospital of Haifa, Rambam Medical Center, POB9602, 31096, Haifa, Israel, r_brik@rambamhealthgovil
Abstract: The objective of this study was to evaluate the efficacy of low-dose (0.2 mg/kg) methotrexate (MTX) in the treatment of children with oligoarticular juvenile idiopathic arthritis (JIA) who do not respond to nonsteroidal anti-inflammatory drugs (NSAIDs) and repeated intra-articular corticosteroid (IA) injections. Nineteen consecutive patients (age: 2-14 years, 18 females) with oligoarticular JIA were studied prospectively. Sixteen had a persistent course and three had an extended course of the disease. Patients were defined as nonresponders to IA injections if the duration of improvement following two consecutive injections was less than 4 weeks. These patients were offered low-dose oral MTX, administered once a week for at least 6 months. Of the 19 patients in this series, 2 responded to NSAIDs alone. Forty-eight IA injections were given to 17 patients; 11 (64%) of them did not respond to this treatment. Nine of the nonresponders were treated with low-dose MTX for a median duration of 15+/-3.8 months. Except for one patient with an extended disease course, all responded very well to treatment and went into remission after a median of 6.4+/-2.9 months, and none required additional IA injections after initiation of MTX treatment. Low-dose oral MTX appears to be very effective in the management of children with oligoarticular JIA, who are unresponsive to IA injections - CLARKE MG, THOMAS HG, CHESTER JF: MRSA-infected external iliac artery pseudoaneurysm treated with endovascular stenting. Cardiovasc Intervent Radiol 28:3 364-366, 2005
Organism: Department of Surgery, Taunton & Somerset Hospital, Musgrove Park, Taunton, TA1 5DA, UKFAU - Clarke, M G
Abstract: A 48-year-old woman with severe juvenile-onset rheumatoid arthritis presented with a bleeding cutaneous sinus distal to her right total hip replacement scar. Methicillin resistant Staphylococcus aureus (MRSA) was isolated on culture. She had previously undergone bilateral total hip and knee replacements at aged 23 and six years later had the right knee prosthesis removed for infection, with subsequent osteomyelitis of the femoral shaft and right total hip prosthesis disruption. Peripheral arteriography was performed in view of persistent bleeding from the sinus, which revealed a 6 cm false aneurysm filling from and compressing the right external iliac artery (EIA). A PTFE-covered, balloon expandable JOSTENT was deployed in the right EIA, successfully excluding the false aneurysm and preventing further bleeding from the sinus. No graft infection was reported at 12 months. This case illustrates the potential use of endovascular stent-grafting in the treatment of an infected pseudoaneurysm - DINARELLO CA: Blocking IL-1 in systemic inflammation. J Exp Med 201:9 1355-1359, 2005
Organism: Division of Infectious Diseases, Department of Medicine, University of Colorado Health Sciences Center, Denver, CO 80262, USA cdinare333@aolcomFAU - Dinarello, Charles A
Abstract: A growing number of systemic inflammatory diseases characterized in part by recurrent fevers, leukocytosis, anemia, and elevated acute phase proteins are linked to interleukin (IL)-1 activity since rapid and sustained resolution is observed upon specific blockade of IL-1 receptors. Rapid resolution of systemic and local inflammation is now also reported in systemic onset juvenile idiopathic arthritis (SoJIA). Loss of control of the secretion of IL-1beta might be a common mechanism explaining the aberrant activity of IL-1 in these diseases - EBERHARD BA, ILOWITE NT: Response of systemic onset juvenile rheumatoid arthritis to etanercept: is the glass half full or half empty? J Rheumatol 32:5 763-765, 2005
- HAAPASAARI J, KAUTIAINEN H, ISOMAKI H, HAKALA M: Hydroxychloroquine does not decrease serum methotrexate concentrations in children with juvenile idiopathic arthritis. Arthritis Rheum 52:5 1621-1622, 2005
- JANSE AJ, UITERWAAL CS, GEMKE RJ, KIMPEN JL, SINNEMA G: A difference in perception of quality of life in chronically ill children was found between parents and pediatricians. J Clin Epidemiol 58:5 495-502, 2005
Organism: Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, The NetherlandsFAU - Janse, A J
Abstract: BACKGROUND AND OBJECTIVES: Quality of life measurements can help to estimate the well-being of chronically ill patients, and disclose discrepancies in perception between physicians and patients that might otherwise interfere with the effectiveness of treatment. The objective was to investigate the differences in perception of quality of life between parents of chronically ill children and pediatricians. METHODS: A cross-sectional study was conducted in four tertiary pediatric care centers in The Netherlands. The Health Utilities Index mark 3 (HUI3) was used by 37 pediatricians and 279 parents of patients (children aged 1 to 17 years) with cystic fibrosis admitted either in daycare or for a pneumonia, or patients with newly diagnosed acute lymphoblastic leukemia, juvenile idiopathic arthritis, or asthma. RESULTS: Differences in perception of quality of life between parents and pediatricians appeared to be dependent of the disease. In patients with acute lymphoblastic leukemia (OR 7.4; [95% CI 2.88-18.97], juvenile idiopathic arthritis (4.7; [95% CI 2.00-11.22]), and asthma (2.3; [95% CI 1.13-4.69]) a difference in perception was more likely to occur than in patients with cystic fibrosis admitted in daycare. CONCLUSION: At the onset of a chronic disease, the parents of pediatric patients may be misunderstood by health care professionals, especially in subjective attributes. Assessment of quality of life may contribute to better understanding between pediatricians and parents, and thus may even enhance compliance and treatment effects - JANSE AJ, SINNEMA G, UITERWAAL CS, KIMPEN JL, GEMKE RJ: Quality of life in chronic illness: perceptions of parents and paediatricians. Arch Dis Child 90:5 486-491, 2005
Organism: Department of Paediatrics, Wilhelmina Children's Hospital, University Medical Centre Utrecht, NetherlandsFAU - Janse, A J
Abstract: AIMS: To investigate the differences in perception of quality of life between parents of chronically ill children and paediatricians at diagnosis and follow up. Quality of life was assessed using the (HUI3). METHODS: Longitudinal study (July 1999-January 2002) of 37 paediatricians and 181 parents of patients (children aged 1-17 years) with cystic fibrosis admitted for a pneumonia or patients with newly diagnosed acute lymphatic leukaemia, juvenile idiopathic arthritis, or asthma. Main outcome measure was percentage agreement on the attributes of the HUI3 between parents and paediatricians. RESULTS: Differences in perception of health and wellbeing between paediatricians and parents of children with a chronic disease were found, not only at diagnosis but also after a period of follow up. Differences were particularly clear in the subjective attributes emotion (range of agreement 28-68%) and pain/discomfort (range of agreement 11-33%). In all patient groups, at baseline and follow up, the paediatrician assessed the patient to have less pain/discomfort in comparison to the parents. Despite a prolonged patient- paediatrician relationship, differences at follow up did not decrease compared to baseline. CONCLUSION: At the onset of a chronic disease, but also after a period of follow up, quality of life of paediatric patients may be misunderstood by healthcare professionals, especially in the subjective attributes. Systematic assessment of quality of life may contribute to better understanding between physicians and parents - KAYA A, OZGOCMEN S, KIRIS A, CIFTCI I: Clinical and radiological diagnosis of progressive pseudorheumatoid dysplasia in two sisters with severe polyarthropathy. Clin Rheumatol .: 2005
Organism: Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Firat University, Firat Tip Merkezi, Fiziksel Tip ve Reh ABD, Romatoloji BD, 23119, Elazig, Turkey, sozgocmen@hotmailcom
Abstract: The aim of this case report is to describe unusual cases of progressive pseudorheumatoid dysplasia (PPD) affecting the axial skeleton and peripheral joints and to stress the importance of examining the entire skeleton for definite diagnosis and the importance of rehabilitation interventions. PPD is a rare familial disease characterized by generalized bone-cartilage dysplasia, progressive arthropathy, and platyspondyly. PPD presents as spondyloepiphyseal dysplasia (SED) tarda with progressive arthropathy and progressive pseudorheumatoid arthritis of childhood and is described as a specific autosomal recessive subtype of SED. Two sisters, 18 and 16 years old, with low back pain and polyarthritis are presented. Radiographic and magnetic resonance imaging of the cases revealed typical features characteristic for PPD-like platyspondyly, multiple intravertebral herniations, changes in metaphyses and epiphysis, and mega os trigonum. Consequently, PPD is a rare disease of childhood and should be kept in mind in the differential diagnosis of juvenile idiopathic arthritis to prevent delayed diagnosis and to begin rehabilitation interventions early. It is essential to carefully examine the entire body, particularly the axial skeleton, and to perform radiological evaluation of the spine. These illustrative cases serve to remind physicians to examine the entire skeleton and not to concentrate only on "branches" but also on the "trunk." - KIMURA Y, PINHO P, WALCO G, HIGGINS G, HUMMELL D, SZER I, HENRICKSON M, WATCHER S, REIFF A: Etanercept treatment in patients with refractory systemic onset juvenile rheumatoid arthritis. J Rheumatol 32:5 935-942, 2005
Organism: Joseph M Sanzari Children's Hospital at Hackensack University Medical Center, Hackensack, New Jersey 07601, USA ykimura@humedcomFAU - Kimura, Yukiko
Abstract: OBJECTIVE:. To assess the efficacy and safety of etanercept in a large cohort of children with refractory systemic onset juvenile rheumatoid arthritis (SOJRA). METHODS: Standardized questionnaires were sent to US pediatric rheumatologists about patients with SOJRA treated with etanercept. Data were collected at baseline and at the last visit on etanercept. Response to treatment was assessed and compared to baseline as the mean percentage reduction in the following: acute phase reactants, prednisone dose, active joint count, and physician global assessment of disease activity. Response was defined as poor if the mean reduction was < 30%, fair if 30% to < 50%, good if 50% to < 70%, and excellent if > 70%. RESULTS: We analyzed data obtained by survey of 82 SOJRA patients treated with etanercept for a mean of 25 months. Poor response to treatment was observed in 45% of the children, fair response in 9%, good in 13%, and excellent in 33%. Baseline steroid therapy could be discontinued in 27/59 (46%) patients. One or more disease flares occurred in 45% of all patients. Twenty-nine patients (35%) discontinued therapy, mostly due to lack of response or flare. There were 32 adverse event reports, most not considered serious, except for 2 cases of macrophage activation syndrome. CONCLUSION: In this cohort of children with SOJRA, 46% had a good or excellent response, and most were able to reduce concomitant corticosteroid doses. The response to etanercept was fair or poor in more than half our study population, and disease flares were common. Due to the unique cytokine profile of SOJRA, tumor necrosis factor blockade may not be the optimal therapeutic approach for children with treatment-resistant SOJRA - KOFOD T, CATTANEO PM, MELSEN B: Three-Dimensional Finite Element Analysis of the Mandible and Temporomandibular Joint on Simulated Occlusal Forces before and after Vertical Ramus Elongation by Distraction Osteogenesis. J Craniofac Surg 16:3 421-429, 2005
Organism: From the *Department of Oral and Maxillofacial Surgery, Aarhus University Hospital, Aarhus, Denmark, and the daggerDepartment of Orthodontics, Royal Dental College University of Aarhus, Aarhus, Denmark
Abstract: Distraction osteogenesis has recently become a mainstay for treatment of mandibular hypoplasia. Thorough knowledge about changes in the temporomandibular joint (TMJ) and the surrounding parts of the mandible and the skull after mandibular distraction is still lacking. The purpose of the current study was to investigate the stress distribution in the mandible and the TMJ before and after skeletal correction by intraoral unilateral vertical mandibular ramus distraction, using a finite element (FE) model. The FE models were based on computed tomography scans and magnetic resonance imaging scans of a patient with unilateral hypoplasia of the right mandibular ramus caused by juvenile idiopathic arthritis. The character of stress distribution in the mandible and TMJ before and after skeletal correction by 15 mm of vertical distraction of the mandibular ramus was analyzed quantitatively and compared during centric occlusion. Before the distraction osteogenesis treatment, the condyles, articular discs, and glenoid fossa regions are loaded with a different stress pattern. The affected right condyle, disc, and fossa are loaded diffusely and externally in comparison with the anterior and with centralized loading on the normal left side. After unilateral mandibular distraction osteogenesis, the load became more centric and symmetrical. The results suggest that correction of the mandibular deformity by distraction osteogenesis tends to normalize the stress patterns in the TMJ - KUMP LI, CERVANTES-CASTANEDA RA, ANDROUDI SN, FOSTER CS: Analysis of Pediatric Uveitis Cases at a Tertiary Referral Center. Ophthalmology .: 2005
Organism: Ocular Immunology and Uveitis Foundation, Boston, Massachusetts, USA; Massachusetts Eye Research and Surgery Institute, Boston, Massachusetts, USA
Abstract: OBJECTIVE: To analyze demographics, anatomic data, diagnoses, systemic associations, and visual outcomes of pediatric patients in a large tertiary eye center. DESIGN: Retrospective cohort study. METHODS: The records of 1242 patients with uveitis referred to the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary (MEEI) from 1985 to 2003 were reviewed retrospectively. Two hundred sixty-nine patients 16 years and younger were identified. RESULTS: Among 269 children with uveitis, 53.5% were girls, 82% were Caucasian, and 82% were born in the United States. Mean age was 8 years (standard deviation, 3.8; range, 1-16). Anterior uveitis represented 56.9% of cases; intermediate, 20.8%; panuveitis, 16%; and posterior, 6.3%. Nongranulomatous (77.6%) and noninfectious (85.7%) were the most frequent types of inflammation. The process was bilateral in 74.4% of patients. Mean follow-up was 22 months, with mean age of 8 years at diagnosis. Mean duration of uveitis at the time of presentation at the MEEI was 2 years. The range of time between the diagnosis of uveitis and referral was 1 day to 5.6 years. The length of time between diagnosis of uveitis and the referral to the tertiary center strongly correlated with the complication rate and degree of visual impairment in our study. The longer the time before the patients were seen by the uveitis expert, the worse the visual outcomes. No systemic associations were found in 58% of patients, juvenile idiopathic arthritis was responsible for 33% of cases, 8% of patients had other systemic associations, and 1% had tubulointerstitial nephritis uveitis syndrome. CONCLUSIONS: Uveitis remains a serious cause of morbidity and visual loss in children. Timely referral to uveitis specialists in the tertiary referral centers may lead to improved visual outcomes in children with chronic uveitis - LIDAR M, KEDEM R, MOR A, LEVARTOVSKY D, LANGEVITZ P, LIVNEH A: Arthritis as the sole episodic manifestation of familial Mediterranean fever. J Rheumatol 32:5 859-862, 2005
Organism: Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, IsraelFAU - Lidar, Merav
Abstract: OBJECTIVE: To clinically and genetically characterize patients with familial Mediterranean fever (FMF) in whom arthritis constitutes the only manifestation, and to establish the most important features distinguishing FMF arthritis in such a setting from other forms of mono/oligo arthritides. METHODS: The study population comprised 14 patients with episodes of arthritis as the only manifestation of FMF who nevertheless fulfilled the diagnostic criteria for FMF. The control group consisted of 28 patients with episodic mono/oligo arthritis of different disease entities (palindromic, reactive, inflammatory bowel disease, Reiter's, seronegative spondyloarthropathy, chronic juvenile, Behcet's, and gouty arthritis) who presented to the rheumatology clinic during the study period. Patients in both groups underwent clinical evaluation and donated blood for FMF gene analysis. RESULTS: The study and control groups shared similar age and sex distribution and experienced the monoarthritic attacks at similar sites, usually the knee and ankle joint. The 2 groups differed significantly in features of arthritis (which were febrile and of short duration in FMF), family history of FMF, mutation analysis, and response to colchicine. These differences allowed the defining of a rule, which readily distinguishes FMF arthritis from other forms of episodic mono/oligo arthritis. CONCLUSION: The clinical, ethnic, and genetic features of recurrent monoarthritis of FMF are specific and may separate FMF from other entities with mono/oligo arthritis - LOVELL DJ, RUTH NM: Pediatric clinical research. Curr Opin Rheumatol 17:3 265-270, 2005
Organism: Cincinnati Children's Hospital, Cincinnati, Ohio 45229, USA Daniellovell@cchmcorgFAU - Lovell, Daniel J
Abstract: PURPOSE OF REVIEW: This review will focus on childhood-onset systemic lupus erythematosus, juvenile idiopathic arthritis, and juvenile dermatomyositis, with special interest on strategies to improve the health-related quality of life in these conditions. RECENT FINDINGS: The contribution of plasma insulin levels, lipoproteins, markers of oxidized state (including nitric oxide metabolites, isoprostanes) and autoantibodies to oxidized low-density lipoprotein to risk for atherosclerosis has been studied in childhood-onset systemic lupus erythematosus. Elevated serum levels of myeloid-related protein-8 (also called S100A8) and myeloid-related protein-14 (S100A9) in children with juvenile idiopathic arthritis can indicate clinically occult disease activity. Serum levels of S100A12 correlate with disease activity in juvenile idiopathic arthritis. Magnetic resonance imaging T2 relaxation times in weight-bearing cartilage in patients with juvenile idiopathic arthritis may help with early detection of cartilage changes. Quantitative computed tomography commonly shows decreased muscle mass and abnormal bone geometry in juvenile idiopathic arthritis patients. In patients with juvenile idiopathic arthritis who do not respond to oral methotrexate, subcutaneous methotrexate dosing was frequently successful. Duration of inactive disease while a patient is receiving methotrexate does not decrease the frequency of flaring of disease once methotrexate is discontinued. Residual synovial inflammation seems to be a stronger influence on the rate of relapse. In juvenile dermatomyositis, the quantitative magnetic resonance imaging T2 relaxation time and overexpression of Class I major histocompatibility complex in early juvenile dermatomyositis are reported. Intravenous cyclophosphamide in refractory juvenile dermatomyositis and tacrolimus ointment for the dermatologic manifestations of juvenile dermatomyositis seem promising. SUMMARY: Progress has been made in the diagnosis and treatment of childhood-onset systemic lupus erythematosus, juvenile idiopathic arthritis, and juvenile dermatomyositis - MARGETIC B, AUKST-MARGETIC B, BILIC E, JELUSIC M, TAMBIC BL: Depression, anxiety and pain in children with juvenile idiopathic arthritis (JIA). Eur Psychiatry 20:3 274-276, 2005
Organism: Neuropsychiatric Hospital "Dr Ivan Barbot", 44317 Popovaca, CroatiaFAU - Margetic, Branimir
Abstract: The aim of this study was to assess relations among depression, anxiety and pain in children with juvenile idiopathic arthritis (JIA). Pain was measured with the visual analogue scale (VAS), and depression and anxiety with depression and anxiety subscales from the Trauma Symptom Checklist for Children (TSC-C). Pain perception was significantly correlated with depression scores - MIHARA M, NISHIMOTO N, OHSUGI Y: The therapy of autoimmune diseases by anti-interleukin-6 receptor antibody. Expert Opin Biol Ther 5:5 683-690, 2005
Organism: Fuji-Gotemba Research Laboratories, Chugai Pharmaceutical Co Ltd, 135, Komakado 1-chome, Shizuoka, 412-8513 Japan miharamsh@chugai-pharmcojpFAU - Mihara, Masahiko
Abstract: Interleukin (IL)-6 plays essential roles not only in the immune response, but also in haematopoiesis and the central nervous system. Deregulated production of IL-6 has been found in chronic inflammatory autoimmune diseases, such as rheumatoid arthritis (RA), systemic onset juvenile idiopathic arthritis (soJIA), Crohn's disease (CD) and systemic lupus erythematosus (SLE). Furthermore, IL-6 activities can explain many symptoms of these diseases. More importantly, serum levels of IL-6 are correlated with disease activity. Based on these facts, the authors planned to develop a humanized anti-IL-6 receptor antibody, tocilizumab (previously known as MRA), as a therapeutic agent for these inflammatory autoimmune diseases. Tocilizumab is a neutralising antibody to suppress IL-6 signalling mediated by both membranous and soluble IL-6R. Clinical efficacy of tocilizumab in RA, soJIA, adult-onset Still's disease or CD patients has been discussed in this review. In all of these diseases, tocilizumab has improved the disease activity, suggesting that IL-6 plays an essential role in the pathogenesis of these diseases - MINGELS A, HUDDE T, HEINZ C, HEILIGENHAUS A: [Vision-threatening complications in childhood uveitis]. Ophthalmologe 102:5 477-484, 2005
Organism: Augenabteilung, St Franziskus Hospital, Munster Mingels@gmxdeFAU - Mingels, A
Abstract: PURPOSE: We analyzed the epidemiologic data and vision-threatening complications in different forms of childhood uveitis.METHODS: This retrospective study included 187 consecutive patients with onset of uveitis before the age of 16 years classified as anterior (AU), intermediate (IU), posterior (PU), and panuveitis (PanU). We analyzed the epidemiologic data as well as visual acuity, uveitic complications and the conservative and surgical therapy.RESULTS: Associated disease was observed in 85 of 187 patients. The most common complications in AU patients were cataract, posterior synechiae, band keratopathy and CME. IU was accompanied by dense vitreous opacities, cataract and CME. Macular scars were the most frequent cause for visual loss in PU. PanU was complicated by dense vitreous opacities, cataract, retinal detachment, CME and phthisis bulbi.CONCLUSIONS: Childhood uveitis is frequently associated with systemic immune-mediated diseases. The diverse uveitis types have different but typical complications - OEN K, MALLESON PN, CABRAL DA, ROSENBERG AM, PETTY RE, NICKERSON P, REED M: Cytokine genotypes correlate with pain and radiologically defined joint damage in patients with juvenile rheumatoid arthritis. Rheumatology (Oxford) .: 2005
Organism: Departments of Paediatrics, University of Manitoba, Winnipeg, Manitoba, Canada
Abstract: Objectives. Single nucleotide polymorphisms (SNPs) in cytokine genes have been associated with risk of a number of autoimmune diseases. Moreover, some SNPs are associated with variations in rates of in vitro gene expression, and it is therefore possible that these functional polymorphisms may differentially affect inflammatory processes and disease outcome. This project's objective was to determine whether cytokine genotypes correlate with disease outcomes in patients with juvenile rheumatoid arthritis (JRA). Methods. Genotypes of SNPs of pro-inflammatory cytokines, tumour necrosis factor-alpha -308G -->A, interleukin-6 (IL-6) -174G -->C and interferon-gamma +874G -->A, and anti-inflammatory, immunosuppressive cytokines, interleukin-10 -1082G -->A, -819C -->T and -592A -->C and transforming growth factor-beta1 (TGF-beta1) codon 10T -->C and codon 25G -->C, were determined for patients with JRA who previously participated in a long-term outcome study. Cytokine genotypes and clinical variables showing significant correlations with clinical outcomes at the alpha = 0.100 level in univariate analyses were entered in multivariate tests. Results. In multivariate tests, the IL-6 genotype -174G/G was positively correlated with pain [regression coefficient B = 0.899, 95% confidence intervals (CI) 0.185, 1.612, P = 0.014]. The homozygous TGF-beta1 codon 25G/G genotype showed a protective effect against joint space narrowing on radiographs taken within 2 yr of disease onset, but confidence intervals were wide [odds ratio (OR) 0.176, 95% CI 0.037, 0.837 P = 0.029]. Conclusions. The correlation of IL-6 genotype with pain and the possible association of the TGF-beta1 codon 25 genotype with short-term radiographic damage (G/C with greater risk and G/G with decreased risk) suggests that both these polymorphisms may be useful early prognostic indicators. Further studies of the relation between cytokine genotypes and outcomes in patients with all forms of juvenile idiopathic arthritis (JIA) are warranted - PLEYER U, KEITZER R: [Uveitis in childhood. Small patients--big problems]. Ophthalmologe 102:5 461-462, 2005
Organism: Augenklinik, Universitatsmedizin Berlin - Charite, Campus Virchow-Klinikum, 13353 Berlin uwepleyer@charitedeFAU - Pleyer, U - POWELL M, SEID M, SZER IS: Efficacy of custom foot orthotics in improving pain and functional status in children with juvenile idiopathic arthritis: a randomized trial. J Rheumatol 32:5 943-950, 2005
Organism: Department of Physical Therapy, Children's Hospital and Health Center, San Diego, California 92123, USA mpowell@chsdorgFAU - Powell, Mary
Abstract: OBJECTIVE:. To compare the clinical efficacy of custom foot orthotics, prefabricated "off-the-shelf" shoe inserts, and supportive athletic shoes worn alone, on reducing pain and improving function for children with juvenile idiopathic arthritis (JIA). METHODS: Children with JIA and foot pain (n = 40) were randomized to one of 3 groups receiving: (1) custom-made semirigid foot orthotics with shock absorbing posts (n = 15), (2) off-the-shelf flat neoprene shoe inserts (n = 12), or (3) supportive athletic shoes with a medial longitudinal arch support and shock absorbing soles worn alone (n = 13). Foot pain and functional limitations were measured using the Pediatric Pain Questionnaire-visual analog scale (VAS), Timed Walking, Foot Function Index (FFI), and the Physical Functioning Subscale of the Pediatric Quality of Life Inventory (PedsQL). Measures were administered by personnel blinded to group status at baseline (before wearing the assigned intervention) and at 3 months' followup. RESULTS: Children in the orthotics group showed significantly greater improvements in overall pain (p = 0.009), speed of ambulation (p = 0.013), activity limitations (p = 0.002), foot pain (p = 0.019), and level of disability (p = 0.024) when compared with the other 2 groups. Both children and parents in the orthotics group reported clinically meaningful improvement in child health-related quality of life, although the group by time interaction did not show statistical significance. Except for a reduction in pain for supportive athletic shoes (paired t test, p = 0.011), neither the off-the-shelf shoe inserts nor the supportive athletic shoes worn alone showed significant effect on any of the evaluation measures. CONCLUSION: In children with JIA, custom-made semirigid foot orthotics with shock-absorbing posts significantly improve pain, speed of ambulation, and self-rated activity and functional ability levels compared with prefabricated off-the-shelf shoe inserts or supportive athletic shoes worn alone - RAVELLI A, MAGNI-MANZONI S, PISTORIO A, BESANA C, FOTI T, RUPERTO N, VIOLA S, MARTINI A: Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 146:5 598-604, 2005
Organism: Department of Pediatrics, Division of Pediatrics II, University of Genoa, Istituto G Gaslini, Largo G Gaslini 5, 16147 Genoa, Italy angeloravelli@ospedale-gaslinigeitFAU - Ravelli, Angelo
Abstract: OBJECTIVE: To develop diagnostic guidelines for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (S-JIA). STUDY DESIGN: We followed the classification criteria approach that is based on the comparison of patients with the index disease with patients with a "confusable" disease. The former group included 74 patients with S-JIA-associated MAS reported in the literature or seen by the authors; the latter group included 37 patients with S-JIA who had 51 instances of "high disease activity" seen by the authors. The relative power of clinical, laboratory, and histopathologic variables in discriminating patients with MAS from patients with high disease activity was evaluated by calculating the sensitivity rate, specificity rate, area under the receiver operating characteristic curve, and diagnostic odds ratio (DOR). The combinations of variables that led to best separation between patients and control subjects were identified through "the number of criteria present" method. RESULTS: The strongest clinical discriminators were hemorrhages (DOR = 67) and central nervous system dysfunction (DOR = 63); the strongest laboratory discriminators were decreased platelet count (DOR = 1092), increased aspartate aminotransferase (DOR = 247), leukopenia (DOR = 70), and hypofibrinogenemia (DOR = 165). The best separation between patients and control subjects occurred when any 2 or more laboratory criteria (DOR = 1309) were simultaneously present; the second best performance was provided by the presence of any 2, 3, or more clinical and/or laboratory criteria (DOR = 765 and 743, respectively). CONCLUSION: We identified preliminary diagnostic guidelines for MAS complicating S-JIA. These guidelines deserve prospective validation - RIDDLE R, RYSER CN, MORTON AA, SAMPSON JD, BROWNE RH, PUNARO MG, GATCHEL RJ: The Impact on Health-Related Quality of Life from Non-Steroidal Anti-Inflammatory Drugs, Methotrexate, or Steroids in Treatment for Juvenile Idiopathic Arthritis. J Pediatr Psychol .: 2005
Organism: Texas Scottish Rite Hospital for Children
Abstract: Objective To assess and compare the impact of medication treatments on health-related quality of life (HRQOL), family function, and medical status in children with juvenile idiopathic arthritis (JIA). Methods Fifty-seven children diagnosed with JIA were assessed by a pediatric rheumatologist and placed into one of three treatment groups: (1) non-steroidal anti-inflammatory; (2) methotrexate; or (3) steroids via IV methylprednisolone. Questionnaires were administered at baseline and 4-month follow-up. The attending pediatric rheumatologist provided additional medical information. Results Data document the impact of JIA on HRQOL, particularly on physical and pain domains. Steroid patients experienced improved HRQOL at follow-up relative to other groups, despite reporting more problems with side effects. Conclusion These results demonstrate positive benefits of steroids in treating JIA children, despite the greatest incidence of adverse side effects - SENGLER C, KEITZER R, PLEYER U: [Uveitis in conjunction with rheumatological diseases in childhood]. Ophthalmologe 102:5 463-476, 2005
Organism: Klinik fur Padiatrie mit Schwerpunkt Pneumologie und Immunologie, Campus Virchow-Klinikum der Charite-Universitatsmedizin, BerlinFAU - Sengler, C
Abstract: Children with juvenile chronic arthritis are at risk to develop intraocular inflammation depending on the type of arthritis. The pathogenic mechanisms are unclear; however, an association with antinuclear antibodies is well known. In particular young girls with oligoarticular onset of arthritis are affected most often. Regular ophthalmologic examinations should allow early diagnosis and effective therapy. Complications such as synechiae, cataract, or macula edema are seen especially in uveitis patients with late diagnosis and insufficient anti-inflammatory therapy. Better therapeutic regimens have led to a better overall prognosis of intraocular inflammation in recent years - THOMAS S, PRICE AJ, SANKEY RA, THOMAS M: Shoulder hemiarthroplasty in patients with juvenile idiopathic arthritis. J Bone Joint Surg Br 87:5 672-676, 2005
Organism: Heatherwood and Wexham Park Hospitals Trust, Wexham Street, Wexham, Berkshire SL2 4HL, UK mikethomas@ukdoctorcomFAU - Thomas, S
Abstract: Replacement of the shoulder in juvenile idiopathic arthritis is not often performed and there have been no published series to date. We present nine glenohumeral hemiarthroplasties in eight patients with systemic or polyarticular juvenile idiopathic arthritis. The mean follow-up was six years (59 to 89 months). The mean age at the time of surgery was 32 years. Surgery took place at a mean of 27 years after diagnosis. The results indicated excellent relief from pain. There was restoration of useful function which deteriorated with time, in part because of progression of the systemic disease in this severely affected group. No patient has required revision to date and there has been no radiological evidence of loosening or osteolysis around the implants. We discuss the pathoanatomical challenges unique to this group. There was very little space for a prosthetic joint and, in some cases, bony deformity and the small size necessitated the use of custom-made implants - TOPALOGLU R, OZALTIN F, YILMAZ E, OZEN S, BALCI B, BESBAS N, BAKKALOGLU A: E148Q is a disease-causing MEFV mutation: a phenotypic evaluation in patients with familial Mediterranean fever. Ann Rheum Dis 64:5 750-752, 2005
Organism: Hacettepe University Faculty of Medicine, Department of Paediatric Nephrology and Rheumatology, 06100 Ankara, Turkey rtopalog@hacettepeedutrFAU - Topaloglu, R
Abstract: BACKGROUND: Familial Mediterranean fever (FMF) is one of the periodic fever syndromes. It is common among Turks, Jews, Arabs, and Armenians. Several mutations in the MEFV gene, including E148Q, have been identified as causing this disease. It has been suggested that the E148Q mutation is the mildest mutation and some reports have questioned its disease association. OBJECTIVE: To evaluate the phenotypic features of the patients with E148Q mutation. SUBJECTS: 26 patients homozygous for E148Q, 10 compound heterozygous for E148Q, and eight complex cases were assessed. RESULTS: Although four of the 26 patients with E148Q/E148Q were asymptomatic at the time of evaluation, abdominal pain was seen in 77% of the patients, fever in 66%, arthralgia in 50%, arthritis in 15.4%, and vomiting in 23.8%. Compound heterozygotes and complex cases had a higher frequency of abdominal pain, fever, arthralgia, arthritis, myalgia, and chest pain than subjects who were homozygous for E148Q, but none of these symptoms reached statistical significance. None of our patients had amyloidosis but two with E148Q/E148Q had a family history of amyloidosis and one had rapidly progressive glomerulonephritis secondary to vasculitis, which progressed to chronic renal failure. CONCLUSIONS: Patients homozygous for E148Q have a heterogeneous clinical presentation. Most are symptomatic and colchicine treatment is required in these patients - VIKEN MK, AMUNDSEN SS, KVIEN TK, BOBERG KM, GILBOE IM, LILLEBY V, SOLLID LM, FORRE OT, THORSBY E, SMERDEL A, LIE BA: Association analysis of the 1858C>T polymorphism in the PTPN22 gene in juvenile idiopathic arthritis and other autoimmune diseases. Genes Immun 6:3 271-273, 2005
Organism: Institute of Immunology, Rikshospitalet University Hospital and University of Oslo, Rikshospitalet, Norway mkviken@medisinuionoFAU - Viken, M K
Abstract: A functional single nucleotide polymorphism, 1858C>T, in the PTPN22 gene, encoding a tyrosine phosphatase, has been reported to be associated with type I diabetes and some other autoimmune diseases. To further investigate whether this polymorphism may be a general susceptibility factor for autoimmunity, we performed an association study in five different autoimmune diseases, three previously not tested. We found an association with juvenile idiopathic arthritis (OR=1.41; P=0.04), not previously reported, and a tendency for an association with coeliac disease (OR=1.35; P=0.08). In primary sclerosing cholangitis, no association was observed (OR=0.95; P=0.8). Furthermore, we confirmed the increased risk in rheumatoid arthritis (OR=1.58; P=0.001), but could not find support for an association with systemic lupus erythematosus (OR=0.94; P=0.8). Altogether, we have provided further evidence of an association between autoimmune diseases and the 1858C>T polymorphism in PTPN22 - YOKOTA S: [Juvenile rheumatoid arthritis]. Nippon Rinsho 63 Suppl 5:274-80.: 274-280, 2005
Organism: Department of Pediatrics, Yokohama City University School of MedicineFAU - Yokota, Shumpei - ZHOU H: Clinical pharmacokinetics of etanercept: a fully humanized soluble recombinant tumor necrosis factor receptor fusion protein. J Clin Pharmacol 45:5 490-497, 2005
Organism: Clinical Pharmacology, Wyeth Pharmaceuticals, Collegeville, PA 19426, USAFAU - Zhou, Honghui
Abstract: Etanercept, a fully humanized soluble recombinant tumor necrosis factor receptor fusion protein, is an approved treatment for rheumatoid arthritis, juvenile rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and psoriasis. Etanercept is absorbed slowly from the site of subcutaneous injection, with time to peak concentration at approximately 48 to 60 hours, and is cleared slowly from the body with a t(1/2) of 70 to 100 hours. The absolute bioavailability of etanercept was 58% in healthy subjects following subcutaneous administration. The 25-mg twice-weekly dosage regimen generates systemic exposures comparable to 50 mg once weekly, as predicted by pharmacokinetic modeling and simulation and later confirmed by clinical studies. The pharmacokinetics of etanercept in patients with rheumatoid arthritis are comparable to those in healthy individuals and patients with ankylosing spondylitis, congestive heart failure, and psoriasis. In children with polyarticular-course juvenile rheumatoid arthritis, after subcutaneous doses of 0.4 mg/kg twice weekly, the clearance of etanercept may be slightly reduced in children aged 4 to 8 years. Pharmacokinetic simulation predicts that a dose of 0.8 mg/kg once weekly generates comparable systemic exposure as 0.4 mg/kg twice weekly. No requirement for etanercept dosage adjustment is needed when etanercept is coadministered with warfarin, digoxin, or methotrexate