Bibliography August 04- AHMED N, BLOCH-ZUPAN A, MURRAY KJ, CALVERT M, ROBERTS GJ, LUCAS VS: Oral health of children with juvenile idiopathic arthritis. J Rheumatol 31:8 1639-1643, 2004
Organism: Department of Rheumatology, Great Ormond Street Hospital for Children, London, United KingdomFAU - Ahmed, Nabila
Abstract: OBJECTIVE:s. To estimate dental disease indices and temporomandibular joint (TMJ) dysfunction in children with juvenile idiopathic arthritis (JIA). METHODS: Indices were recorded for dental caries, bacterial dental plaque, gingival inflammation, and TMJ dysfunction in children with JIA and matched controls. RESULTS: There was no significant difference in dental caries experience or the mean plaque score between children with JIA and controls. The mean gingivitis score for the permanent teeth only was significantly greater in the JIA children compared with the controls (p = 0.02). There was a significantly greater proportion of children with JIA with signs of both left and right TMJ dysfunction (p = 0.05, p = 0.02) and symptoms (p = 0.0001, p = 0.0001) compared with controls. CONCLUSION: The low caries rate was attributed to the fact that children with JIA had received preventive dental care from an early age combined with sugar free medication - BROSTROM E, NORDLUND MM, CRESSWELL AG: Plantar- and dorsiflexor strength in prepubertal girls with juvenile idiopathic arthritis. Arch Phys Med Rehabil 85:8 1224-1230, 2004
Organism: Woman and Child Health and Department of Neuroscience, Karolinska Institutet, Stockholm, SwedenFAU - Brostrom, Eva
Abstract: OBJECTIVE: To compare lower-leg strength of young girls with polyarticular juvenile idiopathic arthritis (JIA) with that of healthy, age-matched controls. DESIGN: Isometric and isokinetic strength tests of the plantar- and dorsiflexors. All strength measures were made at an ankle angle of 90 degrees. Isokinetic plantar- and dorsiflexor measures were made at 15 degrees/s during shortening (concentric) and lengthening (eccentric) actions. SETTING: Strength testing laboratory. PARTICIPANTS: Ten prepubertal girls diagnosed with JIA and 10 healthy girls. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Isometric and isokinetic plantar- and dorsiflexor strength. RESULTS: Isometric plantar- and dorsiflexion torques were significantly lower (48% and 38% respectively; P<.05) for the children with JIA than for the controls. The JIA group also produced lower shortening plantarflexion torques (52%, P<.05). Lengthening plantarflexor torques did not differ significantly between the 2 groups (P<.05). Controls were stronger than the JIA group for both shortening and lengthening maximal dorsiflexor actions (P<.05). All children were 4 to 5 times stronger in plantarflexion than in dorsiflexion. CONCLUSIONS: Girls with JIA had significantly less plantar- and dorsiflexor strength than age-matched, healthy peers. The reduced strength of children with JIA is likely to affect function in daily activities and probably contributes to reduced levels of physical activity - BURGOS-VARGAS R, FOELDVARI I, THON A, LINKE R, TUERCK D: Pharmacokinetics of meloxicam in patients with juvenile rheumatoid arthritis. J Clin Pharmacol 44:8 866-872, 2004
Organism: Department of Rheumatology, Hospital General de Mexico, MexicoFAU - Burgos-Vargas, Ruben
Abstract: The pharmacokinetics of a meloxicam suspension were studied in 18 children with juvenile rheumatoid arthritis. Children received a single 0.25-mg/kg dose up to a maximum of 15 mg. Pharmacokinetic parameters after the first dose were calculated by noncompartmental methods. Geometric mean (percent coefficient of variation for geometric mean [gCV]) C(max), AUC(0- infinity ), apparent clearance, apparent volume of distribution, and elimination half-life values were 1.24 microg/mL (47% gCV), 25.6 microg x h/mL (81% gCV), 0.17 mL/min/kg (83% gCV), 0.19 L/kg (63% gCV), and 13.4 hours (54% gCV) in the younger group and 1.89 microg/mL (25% gCV), 35.8 microg x h/mL (21% gCV), 0.12 mL/min/kg (23% gCV), 0.13 L/kg (22% gCV), and 12.7 hours (21% gCV) for the older group, respectively. Area under the curve, volume of distribution, and clearance tended to be higher in the younger group, whereas elimination half-lives were similar. A post hoc comparison to pharmacokinetic data in adults revealed no relevant differences. Thus, a common body weight-normalized dose is considered appropriate for children older than 2 years - CORONA F, SCARAZATTI M, DELL'ERA L, BELTRAMELLI M, CARNELLI V, BARDARE M: Active refractory juvenile idiopathic arthritis: Treatment with infliximab. Efficacy and safety. Italian Journal of Pediatrics (Italy ) 30:3 165-168, 2004
Abstract: Objectives. To evaluate efficacy and safety of infliximab (Remicade) in patients affected by severe Juvenile Idiopathic Arthritis (JIA) unresponsive to traditional DMARDs. Methods. Nine patients were treated with infliximab (3 mg/kg ev) on days 0, 15, 45 and then every two months. Patients who had a 30% improvement in at least three of the six variables evaluated, with no more than one indicator worsening by more than 30%, were considered responders. Results. Six out of nine patients satisfied the criteria for improvement. Two patients experienced adverse events that required withdrawal from the study. Conclusions. These data confirm infliximab as an effective treatment for refractory JIA - COUZIN J: Basic and clinical immunology meeting. And action! Dendritic cells go live. Science 305:5685 772-773, 2004
- EL GAMAL YM, HESHMAT NM, EL KERDANY TH, FAWZY AF: Serum thrombomodulin in systemic lupus erythematosus and juvenile idiopathic arthritis. Pediatric Allergy and Immunology (Denmark ) 15:3 270-277, 2004
Abstract: Thrombomodulin is a thrombin receptor on the vascular endothelial cell surface which is likely released upon endothelial cell damage. Serum soluble thrombomodulin (sTM) was assessed and investigated as a parameter of disease activity in children and adolescents with systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA). Patients included in this study were regularly attending the Allergy and Immunology Clinic, Children's Hospital, Ain Shams University. They were 38 (76%) females and 12 (24%) males, their ages ranged between 5 and 18 years with a mean of 14.3 +/- 4.84 years and median of 13 years. They were divided into two groups: SLE group which included 20 patients and JIA group which included 30 patients; and the control group which included 30 healthy age and sex-matched individuals for comparison. Disease activity in SLE patients was evaluated by systemic lupus erythematosus disease activity index (SLEDAI) score, while in JIA patients disease activity was determined by number of joints with active arthritis, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Serum levels of sTM were determined by enzyme-linked immunosorbent (ELISA) assay. Serum levels of sTM were significantly higher in SLE and JIA patients in comparison with the control group; there was no significant difference between SLE and JIA patients. In SLE patients, a highly significant correlation was found between sTM and SLEDAI score (r = 0.99, p < 0.001). In JIA patients, a highly significant correlation was found between sTM and number of joints with active arthritis as well as ESR (r = 0.85, p < 0.001; r = 0.93, p < 0.001, respectively). Levels of sTM were significantly higher in CRP-positive than CRP-negative JIA patients. Serum sTM is a useful serologic marker of disease activity in SLE and JIA. It may prove to be a potential indicator for early and more aggressive treatment. Furthermore, sTM may prove to be an important marker for vasculitis in general - EL SAYED ZA, SALEH MT, AL WAKKAD AS, SHERIEF LS, NASR EL-DIN AM: Cartilage proteoglycan aggrecan as a predictor of joint damage in juvenile rheumatoid arthritis. East Mediterr Health J 7:6 992-1003, 2001
Organism: Department of Paediatrics, Faculty of Medicine, Ain Shams University, Cairo, EgyptFAU - El-Sayed, Z A
Abstract: Aggrecan was measured in the sera of 31 children with juvenile rheumatoid arthrits and in the synovial fluid of 10 of them. Patients were evaluated at baseline and 3 months later. Radiographs were repeated also after 1 year. As comparison, 15 apparently healthy children with no disease and 10 children with arthritis due to other collagen vascular diseases were studied. Baseline serum aggrecan was significantly higher in juvenile rheumatoid arthritis patients compared to controls and other patients. On re-evaluation, a significant drop in serum aggrecan from baseline values coincided with a significant drop in clinical and laboratory indices of active inflammation. Serum aggrecan can help to assess the extent of cartilage destruction and is useful as a prognostic tool to predict joint damage in patients with juvenile rheumatoid arthritis - EMERY H: Pediatric rheumatology: what does the future hold? Arch Phys Med Rehabil 85:8 1382-1384, 2004
Organism: Department of Pediatric Rheumatology, University of Washington/Children's Hospital, Seattle, WA 98105, USAFAU - Emery, Helen
Abstract: Effectiveness of the traditional rehabilitation approaches used in pediatric rheumatology has been difficult to prove and, in times of cost containment, this lack of evidence may lead to undertreatment with physical and occupational therapies. Quantitative methods such as those described in this issue by Brostrom and colleagues can be used to validate those approaches and to reinforce the need for careful attention to the effects of even minor loss of range and strength in children with juvenile arthritis. Historically, up to half of the children affected by polyarticular juvenile arthritis became disabled. Some factors that have led to improved outcomes for childhood rheumatic diseases are discussed, including medications (use of weekly low-dose methotrexate, intra-articular steroid injections, new biologic agents that specifically block mediators of inflammation, for example, tumor necrosis factor and interleukin-1), surgery (joint replacements), and psychosocial interventions (with schools and families). The importance of maintaining range of movement, strength, weight bearing, and ambulation, in an effort to prevent sequelae such as osteoporosis and wheelchair dependence, is emphasized. Early identification of children with rheumatic diseases and aggressive intervention, with a combined medical, rehabilitation, psychosocial, and, rarely, surgical approach, should now allow most affected children to reach adulthood with little or no disability - FABER MR, VERLAAK R, FISELIER TJ, HAMEL BC, FRANSSEN MJ, GERRITS GP: Inherited multicentric osteolysis with carpal-tarsal localisation mimicking juvenile idiopathic arthritis. Eur J Pediatr .: 2004
Organism: Department of Paediatrics, University Medical Centre Nijmegen, Nijmegen, The Netherlands
Abstract: Five patients with multicentric carpal-tarsal osteolysis are presented: a mother and her three children with an autosomal dominant mode of inheritance and one of the children with nephropathy, the fifth a sporadic case also with renal involvement. The main findings common to these five patients are symptoms and signs simulating arthritis of the wrists and/or ankles starting at a young age and mimicking juvenile idiopathic arthritis. Early signs of osteolysis and shortening of the carpus or tarsus are radiological characteristic. The disease may be associated with a peculiar face, but most importantly with nephropathy. The pathogenesis is still unknown. Conclusion: recognition of this disease and differentiation from juvenile idiopathic arthritis is important to avoid unnecessary investigations and treatment. Follow-up of renal function is indicated - FELDMAN DE, DUFFY C, DE CIVITA M, MALLESON P, PHILIBERT L, GIBBON M, ORTIZ-ALVAREZ O, DOBKIN PL: Factors associated with the use of complementary and alternative medicine in juvenile idiopathic arthritis. Arthritis Rheum 51:4 527-532, 2004
Organism: Universite de Montreal, Montreal, Quebec, Canada debbiefeldman@umontrealcaFAU - Feldman, Debbie Ehrmann
Abstract: OBJECTIVE: To describe the frequency of complementary and alternative medicine (CAM) use in patients with juvenile idiopathic arthritis (JIA) and to explore whether CAM was associated with patient-specific characteristics, parent-specific characteristics, and medical management factors. METHODS: Parents of children with JIA completed questionnaires that addressed the use of CAM, adherence and perceived problems, parental distress, and coping with childhood illness. Clinical variables were abstracted from the patients' medical files. RESULTS: One hundred-eighteen children with JIA, mean +/- SD age of 10.4 +/- 3.9 years and mean +/- SD disease duration of 4.5 +/- 3.5 years, participated in the study. Ever use of CAM was 33.9% and was higher in patients whose parents used CAM (odds ratio [OR] 5.1, 95% confidence interval [95% CI] 1.4-19.5) and among those who considered themselves as "Canadian" as opposed to belonging to a specific ethnic group (OR 10. 7, 95% CI 1.2-99.8). Adherence to conventional treatment was high for both users and nonusers of CAM. CONCLUSION: Use of CAM is common among patients with JIA. CAM use is not related to any decrease in adherence to conventional medical treatment - GOSSEC L, BETTEMBOURG-BRAULT I, PHAM T, DOUGADOS M: HLA DRB1*01 and DRB1*04 phenotyping does not predict the need for joint surgery in rheumatoid arthritis. A retrospective quantitative evaluation of 300 French patients. Clin Exp Rheumatol 22:4 462-464, 2004
Organism: Service de Rhumatologie B, Universite Rene Descartes, Centre Hospitalier Universitaire Cochin, Paris, France lauregossec@cchap-hop-parisfrFAU - Gossec, L
Abstract: OBJECTIVE: To determine if the presence of HLA-DR disease-associated epitopes predicts the need for total joint arthroplasty or joint fusion in rheumatoid arthritis (RA). METHODS: Tertiary-referral, monocenter study. Three hundred RA patients (1987 ACR criteria) were retrospectively evaluated; outcome measure was recourse to total joint arthroplasty, joint fusion or bone resection. HLA-DR1 and DR4 were considered as the disease-associated epitopes (subtypes were not available for analysis). Analysis was performed using the lifetable method (Kaplan-Meyer technique). RESULTS: Of the 300 patients included, 78% were women, mean age: 56+/-14 years, mean RA follow-up: 12+/-9 years. Phenotyping: 73% of patients carried one (52%) or two (21%) disease-associated epitopes. Surgery was performed on 24% of the patients during follow-up. The most frequent surgery was total hip arthroplasty (13% of patients). According to lifetable analysis, 13% of patients had surgery (total joint arthroplasty or joint fusion) after 10 years of follow-up, 34% after 20 years. years of follow-up, There was no statistically significant difference in recourse to surgery according to absence or presence (single or double-dose) of disease-associated epitopes. Similar results were observed if the event was the second surgical procedure on a given patient. CONCLUSION: This study failed to demonstrate a relation between HLA phenotyping and the severity of RA defined by the requirement for surgery - HAFNER R, BEISKEN C: Clinical symptoms in rheumatoid pain - Physiotherapeutic intervention
KLINISCHE SYMPTOMATIC BEI ENTZUNDLICH-RHEUMATISCHEM SCHMERZ - PHYSIOTHERAPEUTISCHE INTERVENTION. Aktuelle Rheumatologie (Germany ) 29:3 133-136, 2004
Abstract: Pain in children with rheumatoid disease shows manifold expressions. Acute pain is expressed by crying, moaning and groaning. It is seen in highly active arthritis. Chronic pain is more difficult to recognize. It is not expressed directly but nonverbally. The children have behavioral disturbances, are restless during their sleep and assume pain relieving malpositions. Besides analgesic medication, the pain therapy consists of pain relieving procedures, such as partial loading of affected jounts and careful moving of the joints as well as several physical measures. These therapies are part of the Garmisch Treatment Concept, which offers an effective therapy even for very painful joints and severe malpositions - HENDRICKX G, DE BOECK H, GOOSSENS A, DEMANET C, VANDENPLAS Y: Persistent synovitis in children with Lyme arthritis: two unusual cases. An immunogenetic approach. Eur J Pediatr .: 2004
Organism: Paediatric Rheumatology Unit, Department of Paediatrics, Free University of Brussels, Laarbeeklaan 101, 1090, Jette, Belgium
Abstract: We report on two patients with a persistent Lyme arthritis. In addition both had a peculiar disease history. The first patient had oligoarticular juvenile idiopathic arthritis in remission. Five months after an infected tick bite, she developed a relapse of arthritis in the same knee. We considered Lyme borreliosis as the possible trigger for this reactivation. The disease history of the second patient was that of a classical non-responder. After extensive antibiotic treatment osteolytic lesions became visible. MRI images suggested an erosive arthropathy and arthroscopy was used to investigate possible erosive arthritis. Studies on collected material made us consider the following hypothesis. Despite demonstration of a spirochete fragment in a synovial biopsy, the patient recovered without additional antibiotic treatment. Conclusion: delay of antibiotic treatment after appearance of erythema migrans may cause systemic spread of the antigen and predispose to Lyme arthritis. If intra-articular steroids are considered when spontaneous resolution of Lyme arthritis does not occur, magnetic resonance imaging of the affected joint, prior to administration, may provide additional information. The success of synovectomy may be related to removal of undegraded antigenic material which may prolong the inflammation - HUANG C, YANG Y, CHIANG B: Different familial association patterns of autoimmune diseases between juvenile-onset systemic lupus erythematosus and juvenile rheumatoid arthritis. Journal of Microbiology Immunology and Infection 37:2 88-94, 2004
Abstract: The aim of this study was to determine if the prevalence of autoimmune disorders in the relatives of patients with systemic lupus erythematosus (SLE) is greater than that of relatives of patients with juvenile rheumatoid arthritis (JRA). Interviews were used to obtain histories of the following autoimmune disorders among living or deceased first-, second-, and third-degree relatives of 91 SLE and 110 JRA families: ankylosing spondylitis, SLE, rheumatoid arthritis (RA), JRA, multiple sclerosis, juvenile dermatomyositis, Sjogren's syndrome, myasthenia gravis, psoriasis, and thyroid diseases. There were statistically significant differences between the SLE and JRA probands in mean age and gender ratio (19.1 +/- 4.8 vs 14.0 +/- 5.5 years; M (male)/F (female): 17/74 vs 62/48, p0.005). The prevalence rate of autoimmune diseases in relatives of SLE families (20.9%) was greater than in JRA families (11.8%), but not statistically significantly so. The mean age (18.0 +/- 5.3 vs 14.0 +/- 4.3 years), mean age at diagnosis (13.4 +/- 4.3 vs 7.9 +/- 3.9 years) and gender ratio (F/M, 16/3 vs 5/8) of the patients with affected relatives between these 2 groups all had statistically significant differences. A higher prevalence of SLE in relatives was found in SLE families than in JRA cases. Furthermore, this study revealed a higher incidence of autoimmune disorders among second- and third-degree relatives of SLE or JRA probands versus first-degree ones, especially sisters (including 1 pair of twins) and the maternal aunt in SLE families. These data demonstrate that the prevalence of autoimmune disorders in the relatives of patients with SLE is greater than those of relatives of patients with JRA. This suggests that clinically different autoimmune phenotypes may share common susceptibility genes, which may act as risk factors for autoimmunity - HUANG J-L, YEH C-C, SHAW C-K, YAO T-C, CHEN L-C, LEE T-D, KUO M-L: HLA-DRB1 genotyping in patients with juvenile idiopathic arthritis in Taiwan. European Journal of Immunogenetics (United Kingdom ) 31:4 185-188, 2004
Abstract: The object of this study was to investigate whether there is an association between HLA-DRB1 alleles and the development of juvenile idiopathic arthritis (JIA) in Taiwan. HLA-DRB1 alleles were studied in 60 patients with JIA and 200 healthy controls using polymerase chain reaction (PCR)/sequence-specific oligonucleotide probes (SSO). The frequency of HLA-DRB1*0405 in patients with JIA was found to be significantly higher than that in healthy controls [odds ratio (OR) 2.64, 95% confidence interval (CI) 1.01-6.91]. The DRB1*0405 allele was significantly associated with the development of both polyarthritis (OR 4.30, 95% CI 1.34-13.80) and oligoarthritis (OR 3.27, 95% CI 1.01-10.58). The frequency of HLA-DRB1*1502 was higher in Taiwanese JIA patients with systemic arthritis than in controls (OR 18.09, 95% CI 2.25-145.73). We conclude that, in Taiwan, HLA-DRB1*0405 is associated with the development of polyarthritis and oligoarthritis in children, and HLA-DRB1*1502 is associated with the development of systemic arthritis - INGEGNOLI F, DEL PAPA N, COMINA DP, MAGLIONE W, LUPI E, GERLONI V, FANTINI F: Autoantibodies to chromatin: prevalence and clinical significance in juvenile rheumatoid arthritis. Clin Exp Rheumatol 22:4 499-501, 2004
Organism: Department of Rheumatology, University of Milan, Istituto Ortopedico Gaetano Pini, Milan, Italy francescaingegnoli@yahoocomFAU - Ingegnoli, F
Abstract: OBJECTIVE: To determine the prevalence of anti-chromatin antibodies (Abs) in juvenile rheumatoid arthritis (JRA) and to assess any association between the presence of anti-chromatin Abs and clinical subsets of the disease. METHODS: IgG anti-chromatin Abs and anti-extractable nuclear antigens (ENA) Abs were detected by an enzyme-linked immunosorbent assay (ELISA), and antinuclear Abs (ANA) by indirect immunofluorescence in sera of 89 children with JRA. Ten children with systemic, 32 with polyarticular and 47 with pauciarticular disease onset (uveitis occurred in 17/47 children) were studied. As a control group, 12 sera of patients suffering from idiopathic uveitis and 31 age- and-sex-matched healthy children (HC) were examined. RESULTS: Abs to chromatin were detected in 14/47 (29.8%) of children suffering from pauciarticular onset JRA and in this group the higher prevalence of anti-chromatin Abs has been found in children with chronic uveitis (p = 0.002). Anti-chromatin positivity was observed in 2/10 (20%) of systemic and in 3/32 (9.3%) of polyarticular onset JRA. Furthermore, none of the patients with idiopathic uveitis and HC had Abs to chromatin. anti-chromatin Abs titers remained relatively stable over a 6-month control period. CONCLUSION: Our results confirm previous data about the presence of circulating anti-chromatin Abs in juvenile arthritis. Interestingly, anti-chromatin Abs were significantly higher in the group of patients with pauciarticular onset with past or present history of uveitis, than in patients without ocular involvement. A long-term follow-up study could be useful to demonstrate the potential utility of these autoantibodies in diagnosing, classifying and treating children affected - JANSEN LM, VAN DER HORST-BRUINSMA IE, VAN SCHAARDENBURG D, LARD LR, HAZES JM, HUIZINGA TW, DIJKMANS BA: Comparison of the baseline disease activity of early oligo- and polyarthritis in sequential years. Clin Exp Rheumatol 22:4 447-452, 2004
Organism: Jan van Breemen Instituut, Amsterdam, The NetherlandsFAU - Jansen, L M A
Abstract: OBJECTIVE: Many early arthritis clinics (EACs) have been started in the last decade in order to detect and treat rheumatoid arthritis early. The present study evaluates whether the disease activity at admission of patients with early oligo- and polyarthritis changed during the period 1993--1998 in two EACs in the Netherlands. METHODS: Patients were selected who were diagnosed after one year as having rheumatoid arthritis (RA) or oligo- or polyarthritis (UPA), had a symptom duration of less than 2 years, and were referred from two Dutch EACs between 1993 and 1998. The data from the two clinics were combined and stratified by referral year. Differences in baseline disease characteristics as well as changes in radiological and functional scores after two years of follow-up between referral years were analysed by ANOVA using Bonferroni corrected p levels. RESULTS: A total of 405 patients (66% females; median age 57 yrs (18-93): 80% diagnosed as RA, the remainder as UPA) were included in the study. The year-groups did not differ significantly in demographic characteristics or in the duration of complaints (median 6 months). The number of patients with a diagnosis of RA declined over the years, as did the mean baseline erythrocyte sedimentation rate (ESR), in RA and UPA patients. The functional status (Health Assessment Questionnaire: HAQ) was enhanced in 1998 compared with the previous years (p < 0.001). Radiographic progression (Sharp/van der Heijde score) after the 2-year follow-up decreased (p < 0.001) in the later referral years compared to the referral group of 1994. Disease modifying anti-rheumnatic drugs (DMARDs) were started in an earlier stage and the prescription rate of sulfasalazine and methotrexate increased over the years, whereas the number of patients not treated with DMARDs declined. CONCLUSION: The pattern of patient referral changed over 6 years towards fewer patients who fulfilled the RA diagnosis and a lower ESR (among UPA as well as RA patients), whereas the number of swollen joints and the duration of complaints remained the same. The radiological progression declined over time, probably due to less inflammation at the first visit and the increased use of DMARDs - KASAPCOPUR O, CULLU F, KAMBUROGLU-GOKSEL A, CAM H, AKDENIZLI E, CALYKAN S, SEVER L, ARYSOY N: Hepatitis B vaccination in children with juvenile idiopathic arthritis. Ann Rheum Dis 63:9 1128-1130, 2004
Organism: Department of Pediatrics, Cerrahpaa Medical Faculty, Istanbul University, Istanbul, Turkey ozgurcopur@e-kolaynetFAU - Kasapcopur, O
Abstract: OBJECTIVES: To evaluate the responsiveness of children with juvenile idiopathic arthritis (JIA) to hepatitis B vaccination and to determine the most useful vaccination schedule. METHODS: 39 children with JIA were enrolled in the study; all were in remission and negative to serological testing for hepatitis B surface antigen (HbsAg). The control group consisted of 41 healthy children. There were two different vaccination schedules: group I was vaccinated at 0, 1, and 3 months; group II was vaccinated at 0, 1, and 6 months. Positive responsiveness to the vaccine was defined as an anti-hepatitis B antibody titre above 10 mIU/ml. RESULTS: All the children except one with systemic JIA developed an antibody response. None of the JIA patients experienced a flare up or clinical deterioration related to the vaccination. The antibody levels in children with JIA were significantly lower than in the healthy controls. Comparison of the antibody levels between the two vaccination schedules showed no statistical difference in the controls; in the JIA subjects the group II schedule resulted in a trend to a greater response than the group I schedule (p<0.07). Vaccine responsiveness was not influenced by either methotrexate or prednisolone treatment. CONCLUSIONS: Children with JIA had an adequate response to hepatitis B vaccination and the response was not affected by immunosuppressive treatment. A vaccination schedule at 0, 1, and 6 months seems to be preferable to 0, 1, and 3 months - LESCAUT W, BROCQ O, ALBERT C, PLUBEL Y, FLORY P, EULLER-ZIEGLER L: Juvenile idiopathic polyarthritis in monozygotic twins with congenital CSUB4 deficiency [2]
POLYARTHRITE IDIOPATHIQUE JUVENILE CHEZ DES JUMEAUX MONOZYGOTES ASSOCIEE A UN DEFICIT CONGENITAL PARTIEL EN C4. Revue de Medecine Interne (France ) 25:8 604-606, 2004 - LIN L, WU F-Q, ZHANG X-G, LIU Y-Q, WANG J: Differential diagnosis of juvenile ankylosing spondylitis and juvenile rheumatoid arthritis by subtypes of HLA-B27 alleles. Chinese Journal of Clinical Rehabilitation (China ) 8:9 1780-1782, 2004
Abstract: Background: The relationship of juvenile ankylosing spondylitis(JAS) and juvenile rheumatoid arthritis(JRA) with HLA-B27 alleles has been studied for about 25 years. Some researchers abroad reported that the relationship between ankylosing spondylitis and HLA-B27 allele subtypes is different from that between arthritis and HLA-B27 allele subtypes. Objective: To investigate the relationship of JAS and JRA with HLA-B27 allele subtypes and its role in the pathogenesis so as to find out the testing methods for earlier diagnosis of JAS or JRA. Design: Non-random control laboratory study. Setting, participants and interventions: A total of 60 patients diagnosed with JAS or JRA in Beijing Children's Hospital were recruited, including 5 fathers or mothers from 5 patients' families, compared with 9 normal controls (from 200 donors for bone marrow transplantation). We studied 74 cases of HLA-B27 allele subtypes and analyzed their correlation by using PCR/SSP. Main outcome measures: Correlation of JAS and JRA with HLA-B27 allele subtypes; difference of HLA-B*2704 between JAS and JRA; data of patients' families. Haplotypes analysis and homozygote analysis on patients' families showed that there existed a close link between HLA-B* 2704 and C*1202, but not with HLA-A, and that JAS had a remarkable tendency of family inheritance. Results: HLA-B*2704, *2705, *2702 and *2707 were found in the cases. In JAS group, allelic frequency of HLA-B27 was B*2704 56. 25%, B*2705 40.63% and B*2702 3.13% , respectively. However, in JRA group, it was B*2705 60.7%, B* 2704 28.57%, B*2702 3.57% and B* 2707 7.14%, respectively. Comparison between JAS and JRA showed that the allelic frequency of HLA-B*2704 in JAS patients was higher than that in JAS patients (RR = 3.21, P < 0.05). Conclusion: The susceptibility of JAS is positively associated with subtypes HLA-B*2704. Testing HLA-B27 subtypes may be a valuable marker in differential diagnosis of AS and JRA - LYBACK CC, LYBACK CO, KYRO A, KAUTIAINEN HJ, BELT EA: Survival of Bi-Metric femoral stems in 77 total hip arthroplasties for juvenile chronic arthritis. Int Orthop .: 2004
Organism: Faculty of Medicine, University of Helsinki, Toolontullinkatu 8, 00014, Helsinki, Finland
Abstract: The survival of 77 cementless total hip arthroplasties using a Bi-Metric femoral stem and two types of acetabular components was analysed in 55 patients with juvenile chronic arthritis. The patients were treated between 1986 and 1996. Their mean age was 8.0 years at the onset of the disease and 28.1 years at the time of surgery. The mean follow-up period was 9.6 years. Follow-up evaluations were conducted 3 months and 1, 4, 8, 12 and 16 years post-operatively. The endpoints of survival analysis were revision surgery, death of the patient or the end of the year 2002. The 10-year survival was 77.6% for the Romanus cup and 49.1% for the TTAP-ST cup. In contrast to these inadequate outcomes, the cementless Bi-Metric stem yielded excellent results with a survival rate of 100% for aseptic loosening during a mean follow-up period of 10 years - MAULDIN J, CAMERON HD, JEANOTTE D, SOLOMON G, JARVIS JN: Chronic arthritis in children and adolescents in two Indian health service user populations. BMC Musculoskelet Disord 5:1 30, 2004
Organism: Dept, of Pediatrics, University of Oklahoma College of Medicine, BSEB #235A, Oklahoma City, OK, 73104 USA jmauldin@ihsgovFAU - Mauldin, Joyce
Abstract: BACKGROUND: High prevalence rates for rheumatoid arthritis, spondyloarthopathies, and systemic lupus erythematosus have been described in American Indian and Alaskan Native adults. The impact of these diseases on American Indian children has not been investigated. METHODS: We used International Classification of Diseases-9 (ICD-9) codes to search two Indian Health Service (IHS) patient registration databases over the years 1998-2000, searching for individuals 19 years of age or younger with specific ICD-9-specified diagnoses. Crude estimates for disease prevalence were made based on the number of individuals identified with these diagnoses within the database. RESULTS: Rheumatoid arthritis (RA) / juvenile rheumatoid arthritis (JRA) was the most frequent diagnosis given. The prevalence rate for JRA in the Oklahoma City Area was estimated as 53 per 100,000 individuals at risk, while in the Billings Area, the estimated prevalence was nearly twice that, at 115 per 100,000. These rates are considerably higher than those reported in the most recent European studies. CONCLUSION: Chronic arthritis in childhood represents an important, though unrecognized, chronic health challenge within the American Indian population living in the United States - MICHELS H: Pain in pediatric and juvenile rheumatology
SCHMERZEN IN DER KIND. Aktuelle Rheumatologie (Germany ) 29:3 125-127, 2004 - POUCHOT J, ECOSSE E, COSTE J, GUILLEMIN F: Validity of the childhood health assessment questionnaire is independent of age in juvenile idiopathic arthritis. Arthritis Rheum 51:4 519-526, 2004
Organism: Hopital Louis Mourier, Colombes, France jacquespouchot@lmrap-hop-parisfrFAU - Pouchot, Jacques
Abstract: OBJECTIVE: To determine whether the Childhood Health Assessment Questionnaire (CHAQ) is valid for the comparison of different age subgroups and for longitudinal studies in juvenile idiopathic arthritis (JIA). METHODS: A CHAQ was administered to 306 children with JIA. Rasch analyses were used to compare the difficulty of each of the 30 items of the questionnaire for children of 2 age groups (> or =10 years old and <10 years old). RESULTS: Independent of the physical disability level assessed by the Rasch model, 8 of the 30 items (27%) of the CHAQ were rated significantly different in the 2 age groups. Despite this age-related variation in item difficulty, the impact on the CHAQ disability index using its original scoring system remained low (about 0.25 points on a scale of 0-3). CONCLUSION: The difficulty of 8 of 30 items of the CHAQ depends on the respondent's age. Nevertheless, the design of the CHAQ and its scoring system remove most of the expected physical development bias - PRAHALAD S: Genetics of juvenile idiopathic arthritis: an update. Curr Opin Rheumatol 16:5 588-594, 2004
Organism: Department of Pediatrics, Division of Immunology and Rheumatology, University of Utah School of Medicine, Salt Lake City, Utah 84132-2206, USA sampathprahalad@hscutaheduFAU - Prahalad, Sampath
Abstract: PURPOSE OF REVIEW: Juvenile idiopathic arthritis (JIA) refers to a collection of chronic arthritides in children, and the major subtypes of JIA are similar to the subtypes of juvenile rheumatoid arthritis (JRA). Several genetic variants influencing susceptibility to JIA have been identified, including genes encoding the HLA molecules, cytokines, and other modulators of immune responses. This review outlines the principles behind genetic studies and summarizes recent studies on the genetics of JIA. RECENT FINDINGS: Recent studies confirm the association/linkage between JIA and the HLA region and provide evidence for additional loci involved in susceptibility to JIA. Several studies suggest that polymorphisms in other candidate genes also influence susceptibility to JIA. In addition, some genetic variants seem to influence the phenotype of JIA. A genome-wide scan for JRA in 121 affected sibling pair families confirms that gene(s) in the HLA region influence susceptibility to JRA and identifies other chromosomal regions that possibly influence susceptibility to JRA or subtypes of JRA. Functional studies suggest that biologic markers could be useful in defining the phenotype of individuals with JIA. Familial studies and gene expression profiling are useful tools in the dissection of the genetic basis of JIA. SUMMARY: Although there are challenges to the identification of genetic factors underlying complex diseases such as JIA, considerable progress has been made in JIA genetics. Candidate gene studies remain important to identify genetic variants with small to moderate effects on the JIA phenotype - RUPERTO N, MARTINI A: International research networks in pediatric rheumatology: the PRINTO perspective. Curr Opin Rheumatol 16:5 566-570, 2004
Organism: IRCCS G Gaslini, Universita di Genova, Pediatria II-Reumatologia, Genova, Italy nicolaruperto@ospedale-gaslinigeitFAU - Ruperto, Nicolino
Abstract: PURPOSE OF REVIEW: The purpose of this review is to highlight the problems and possible solutions for the conduct of international collaborative research for pediatric rheumatic diseases. RECENT FINDINGS: Pediatric rheumatic diseases are rare conditions associated with important sequelae on the quality of life and long-term outcome. The research aimed at studying new therapeutic approaches is difficult because of logistic, methodological, and ethical problems. To face these problems, two international networks have been founded: the Pediatric Rheumatology Collaborative Study Group (or PRCSG) and the Paediatric Rheumatology international Trials Organization (or PRINTO). The two networks have the goal to promote, facilitate, and conduct high-quality research into pediatric rheumatic diseases. In particular they have been able to standardize the evaluation of response to therapy in juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, and juvenile dermatomyositis; to draft clinical remission criteria in juvenile idiopathic arthritis; and to provide cross-cultural adapted and validated quality-of-life instruments like the Childhood Health Assessment Questionnaire and the Child Health Questionnaire in 32 different languages. SUMMARY: The creation of large international trial networks such as PRINTO and PRCSG, the definition of internationally recognized and standardized outcome measures and definitions of improvement, the validation of quality-of-life instruments, and the adoption of adequate legislative measures (pediatric rule) have created the basic premises for the best future assessment of pediatric rheumatic diseases. This progress now affords children with pediatric rheumatic diseases the same opportunities as adults to be treated with drugs whose safety and efficacy have been assessed through legitimate scientifically valid investigations - SAVIOLI C, SILVA CA, LIN HC, CAMPOS LM, PRADO EF, SIQUEIRA JT: Dental and facial characteristics of patients with juvenile idiopathic arthritis. Rev Hosp Clin Fac Med Sao Paulo 59:3 93-98, 2004
Organism: Dentistry Division, Hospital das Clinicas, Faculty of Medicine, University of Sao PauloFAU - Savioli, Cynthia
Abstract: OBJECTIVE: It has been shown that the temporomandibular joint is frequently affected by juvenile idiopathic arthritis, and this degenerative disease, which may occur during facial growth, results in severe mandibular dysfunction. However, there are no studies that correlate oral health (tooth decay and gingival diseases) and temporomandibular joint dysfunction in patients with juvenile idiopathic arthritis. The aim of this study is to evaluate the oral and facial characteristics of the patients with juvenile idiopathic arthritis treated in a large teaching hospital. METHOD: Thirty-six patients with juvenile idiopathic arthritis (26 female and 10 male) underwent a systematic clinical evaluation of their dental, oral, and facial structures (DMFT index, plaque and gingival bleeding index, dental relationship, facial profile, and Helkimo's index). The control group was composed of 13 healthy children. RESULTS: The mean age of the patients with juvenile idiopathic arthritis was 10.8 years; convex facial profile was present in 12 juvenile idiopathic arthritis patients, and class II molar relation was present in 12 (P =.032). The indexes of plaque and gingival bleeding were significant in juvenile idiopathic arthritis patients with a higher number of superior limbs joints involved (P =.055). Anterior open bite (5) and temporomandibular joint noise (8) were present in the juvenile idiopathic arthritis group. Of the group in this sample, 94% (P =.017) had temporomandibular joint dysfunction, 80% had decreased mandibular opening (P = 0.0002), and mandibular mobility was severely impaired in 33% (P =.015). CONCLUSION: This study confirms that patients with juvenile idiopathic arthritis a) have a high incidence of mandibular dysfunction that can be attributed to the direct effect of the disease in the temporomandibular joint and b) have a higher incidence of gingival disease that can be considered a secondary effect of juvenile idiopathic arthritis on oral health - SCHEPP CP, DANNECKER L, HAUG M, KUMMERLE-DESCHNER J, BECK H, KOTTER I, HOLZER U, DANNECKER GE: Autoantibodies in juvenile idiopathic arthritis: glucose-6-phosphate isomerase is not a specific target. J Rheumatol 31:8 1630-1638, 2004
Organism: University Children's Hospital, Eberhard Karls University, Tuebingen, GermanyFAU - Schepp, Carsten P
Abstract: OBJECTIVE: Antibodies recognizing the ubiquitous cytosolic enzyme glucose-6-phosphate isomerase (GPI) cause arthritis in the K/BxN mouse model. Studies have shown that these antibodies are not specific for rheumatoid arthritis (RA) in humans. We evaluated GPI as a target of autoantibodies in juvenile idiopathic arthritis (JIA). METHODS: We studied 324 serum and 48 synovial fluid (SF) samples from 103 patients with JIA, 36 with RA, and 8 with arthralgia and 11 controls. Anti-GPI antibodies were assessed by densitometrically evaluating immunoblots and ELISA using native and recombinant GPI. We determined the GPI activity of the soluble antigen in serum and SF. RESULTS: Although several samples contained anti-GPI-IgG antibodies, this was not specific for JIA or its subgroups, or for RA. Other proteins in the GPI preparation were also frequently recognized by antibodies. Additionally, we observed increased GPI activity in patients with the systemic manifestation of JIA, but not in other patients. Neither anti-GPI concentrations nor GPI activity were associated with disease activity. CONCLUSION: In addition to the findings in RA, our results indicate that GPI is not a general target of autoantibodies in JIA - SINGER NG, SCALZI LV: Remittive agents in pediatric rheumatology. Curr Opin Rheumatol 16:5 571-576, 2004
Organism: Department of Pediatrics and Internal Medicine, CASE School of Medicine and Rainbow Babies and Children's Hospital/University Hospitals, Cleveland, Ohio 44106, USA ngs@cwrueduFAU - Singer, Nora G
Abstract: PURPOSE OF REVIEW: Children with rheumatic diseases frequently require therapy with disease-modifying antirheumatic drugs and/or biologic agents. Therapies that have been prospectively tested in adults are often used in children before full evaluation of their safety and efficacy. Published experience that may report "off-label" usage can be helpful in decision making, although such reports do not reduce the need for prospective clinical trials in children. The purpose of this review is to summarize the recent published evidence regarding efficacy (and safety, when available) of standard and novel agents used in pediatric rheumatic disease. RECENT FINDINGS: Etanercept, one of three currently available tumor necrosis factor-alpha inhibitors has a juvenile idiopathic arthritis indication. Novel "off-label" uses in children for interleukin-1 receptor agonist (Anakinra), antiinterleukin-6 receptor antibody (MRA), and rituximab (anti-CD20 monoclonal antibody) are discussed. SUMMARY: This review summarizes the published evidence that supports the use of selected disease-modifying antirheumatic drugs and novel biologic agents in children with rheumatic diseases - SMERDEL A, DAI K-Z, LORENTZEN AR, FLATO B, MASLINSKI S, THORSBY E, FORRE O, SPURKLAND A: Genetic assocaition between juvenile rheumatoid arthritis and polymorphism in the SH2D2A gene. Genes and Immunity (United Kingdom ) 5:4 310-312, 2004
Abstract: T-cell-specific adapter protein (TSAd) involved in the negative control of T-cell activation is encoded by the SH2D2A gene. Our recent studies indicate that homozygosity for short (ie GASUB13 and GASUB16) alleles of the SH2D2A gene promoter is associated with development of multiple sclerosis. To study whether the same SH2D2A promoter polymorphism also contributes to the genetic susceptibility to develop juvenile rheumatoid arthritis (JRA), we examined 210 JRA patients and 558 healthy unrelated controls from Norway. The frequency of the short allele GASUB13 was increased among the JRA patients compared to control (0.098 vs 0.05; PSUBn=8=0.042). There was a significant increased frequency of HLA-DRB1*08-positive patients carrying two copies of 'short' alleles GASUB13 and/or GASUB16 compared to healthy controls (16% vs 6%; PSUBn=4=0.016). Our data indicate that the 'short' alleles of the SH2D2A promoter could contribute to the genetic susceptibility to JRA. (c) 2004 Nature Publishing Group. All rights reserved - SORNAY-SOARES C, JOB-DESLANDRE C, KAHAN A: Joint lavage for treating recurrent knee involvement in patients with juvenile idiopathic arthritis. Joint Bone Spine 71:4 296-299, 2004
Organism: Rheumatology A Department, Cochin Teaching Hospital, Paris V University, Paris, FranceFAU - Sornay-Soares, Christine
Abstract: OBJECTIVE: To retrospectively evaluate the benefits of knee joint lavage with intraarticular glucocorticoid injection in patients who have juvenile idiopathic arthritis with knee involvement unresponsive to repeated intraarticular glucocorticoid injections. PATIENTS: Seventeen knees in 10 children (eight girls and two boys) were treated from 1997 to 2000. Mean age was 14 years 9 months and mean disease duration was 7.2 years. The diagnoses were juvenile oligoarthritis (n = 6, including two with extended disease), systemic arthritis (n = 2), juvenile spondyloarthropathy (n = 1), and juvenile dermatomyositis (n = 1). Repeated intraarticular triamcinolone hexacetonide injections had been performed in all the patients, the mean number of injections being 2.2 per patient within the last 30 months. Plain radiographs were normal in six of the eight patients. Mean erythrocyte sedimentation rate was 21.7 mm/h and mean C-reactive protein level was 20.6 mg/l. Joint fluid was obtained from 10 knees and had a mean cell count of 12?660 mm(-3). Second-line therapy was with methotrexate alone or combined with cyclosporine or azathioprine. Oral glucocorticoids and/or nonsteroidal antiinflammatory drugs were used for symptom relief. TREATMENT PROCEDURE: Lavage was performed under strict aseptic conditions with simple analgesia, on a day-hospital basis. After aspiration of the joint, lavage was performed with saline, and a delayed-action glucocorticoid was injected. The knee joint was immobilized in the extended position for 48 h. Efficacy criteria were presence of effusion, presence of pain, and presence of a systemic treatment-sparing effect. RESULTS: Freedom from effusion and pain was noted in all 17 knees after 1 month, in eight (47%) knees after 6 months, and in seven (41%) knees after 12 months. The patients with the longest lasting improvements had systemic polyarthritis. After joint lavage, second-line treatment was reduced in two patients and oral glucocorticoid therapy was stopped in two others. None of the variables studied (age, sex, disease duration, inflammatory syndrome, or joint fluid cytology) predicted a good response. No adverse effects were recorded. CONCLUSION: These preliminary results show that joint lavage with glucocorticoid injection is safe in children. The improvements were modest, but the patients had a history of arthritis refractory to multiple triamcinolone hexacetonide injections. Thus, joint lavage may have a place in the treatment pyramid just before synovectomy - TEN CATE R, NIBBERING PH, BREDIUS RGM: Therapy-refractory systemic juvenile idiopathic arthritis successfully treated with statins [2]. Rheumatology (United Kingdom ) 43:7 934-935, 2004
- UKARAPOL N, LERTPRASERTSUK N, PONGPROT Y, SITTIWANGKUL R, WONGSAWASDI L: Case of eosinophilic granulomatous enterocolitis caused by Strongyloides stercoralis infection with marked hypoalbuminemia and ascites. Digestive Endoscopy (Japan ) 16:3 241-243, 2004
Abstract: We report a 10-year-old boy presenting with generalized pitting edema, ascites, abdominal pain, and chronic mucous diarrhea for 4 weeks. He had underlying diseases of hemoglobin E and juvenile rheumatoid arthritis and had been treated with immunosuppressive agents for a long period of time, including prednisolone and methotrexate. After extensive investigations, Strongyloides stercoralis infection, leading to protein-losing enteropathy and eosinophilic granulomatous enterocolitis, was diagnosed. In the present report, we demonstrate early colonoscopic findings, revealing patchy erythema and small raised mucosal nodules with erosions at the cecum. Histopathological study showed open ulceration with cryptitis, intense infiltration of eosinophils and histiocytes with granuloma formation, in which Strongyloides stercoralis larvae were noted - WILAND P, WIELA-HOJENSKA A, GLOWSKA A, CHLEBICKI A, HURKACZ M, ORZECHOWSKA-JUZWENKO K, SZECHINSKI J: Renal function in rheumatoid arthritis patients treated with methotrexate and infliximab. Clin Exp Rheumatol 22:4 469-472, 2004
Organism: Unit of Internal Diseases and Rheumatology, Regional Railway Hospital of Wroclaw, Wroclaw, Poland pwiland@providerplFAU - Wiland, P
Abstract: OBJECTIVE: This study was aimed at monitoring the early and late effects of infliximab on renal proximal function in RA patients treated with methotrexate. N-acetyl-3-D-glucosaminidase (NAG) activity in urine served as an indicator of proximal tubular damage METHODS: NAG activity was estimated in 21 patients during the course of treatment with infliximab and methotrexate. In every patient NAG-enzymuria was estimated directly before and 60 min after infliximab infusions and 62 weeks after starting the therapy. RESULTS: The total of mean NAG activities observed before each infusion of infliximab was significantly lower (p < 0.02) than NAG-enzymuria before the start of infliximab treatment (7.4 UI/g vs 11.8 UI/g). The proportion of patients in whom NAG activity rose by more than 50% during treatment ranged from 5.3% to 25%. Administration of infliximab did not significantly change the mean serum creatinine levels or creatinine clearance. No significant differences were observed in the mean values of NAG values before and 60 min after infliximab infusion. Patients who demonstrated elevated NAG activities during the course of the whole treatment demonstrated significantly more pronounced NAG enzymuria before treatment and one hour after the first infusion (p < 0.0005), as well as higher RA activity (p < 0.05). There was no observed influence of NSAIDs or prednisone on the frequency of elevated NAG activities. Raised creatinine concentrations (> 1.3 mg/dL) were noted before and during the course of infliximab treatment in 3 patients. In 16 patients abdominal fat aspiration biopsy was performed and in 3 the presence of amyloid deposits was demonstrated. In these patients NAG activity exceeded twice the upper normal limit. CONCLUSION: The introduction of infliximab during methotrexate therapy demonstrated no early or delayed nephrotoxicity of the drug in patients with rheumatoid arthritis - WULFFRAAT NM, DE KLEER IM, PRAKKEN BJ, KUIS W: Stem cell transplantation for autoimmune disorders. Refractory juvenile idiopathic arthritis. Best Pract Res Clin Haematol 17:2 277-289, 2004
Organism: Department of Paediatric Immunology and Rheumatology, Wilhelmina Children's Hospital, University Medical Center Utrecht, PO Box 85090, Utrecht 3508 AB, The Netherlands nwulffraat@wkzazunlFAU - Wulffraat, Nico M
Abstract: Since 1997, haematopoietic stem cell transplantation (HSCT) has been applied as an experimental procedure in more than 50 children with refractory juvenile idiopathic arthritis. We describe here follow-up data on 34 children with juvenile idiopathic arthritis, treated with HSCT in order to evaluate its feasibility, safety and efficacy. Data were collected on immunological reconstitution, complications and key rheumatological parameters. The clinical follow-up of the children ranged from 12 to 48 months. Eighteen of the 34 patients achieved a drug-free complete remission. Seven of these patients had previously failed treatment with anti-tumour necrosis factor-alpha. Six of the 34 patients showed a partial response (ranging from 30 to 70%), and 7 of the 34 patients showed a complete relapse of disease. Infectious complications were frequently seen. There were three cases of transplant-related mortality and two cases of disease-related mortality. It is still unclear why especially patients with systemic juvenile idiopathic arthritis are at risk of episodes of reactive haemophagocytosis - ZANNIN ME, MARTINI G, PINELLO L, ZACCHELLO F, ZULIAN F: Uveitis associated with juvenile idiopathic arthritis
L'UVEITE NELL'ARTRITE IDIOPATICA GIOVANILE. Medico e Bambino (Italy ) 23:6 383-388, 2004
Abstract: Uveitis is a frequent complication of rheumatic diseases, and represents one of the main causes of visual loss in the pediatric age. In 80% of the cases anterior uveitis is associated with juvenile idiopathic arthritis (JIA) and is chronic and asymptomatic, with severe complications if undiagnosed. The most severe forms can be complicated by synechiae, glaucoma, cataract, band keratopathy, cystoid macular edema and ultimately loss of vision in a proportion of patients ranging from 15% to 40%. Early diagnosis and management of intraocular inflammation can reduce the incidence of complications and improve the visual outcome. The characterization of early predictors of uveitis is crucial in order to identify, at the onset of JIA, patients at high risk for developing severe uveitis and who need early initiation of immunosuppressive treatment. The role of the pediatrician in the early diagnosis of JIA and in its management through close collaboration with rheumatologists and ophthalmologists is essential to optimize treatment and improve prognosis