Bibliography May 2003
- AGGARWAL A, MISRA R: Methotrexate inhibits interleukin-6 production in patients with juvenile rheumatoid arthritis. Rheumatol Int 23:3 134-137, 2003
Organism: Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India 226 014 amita@sgpgiacinFAU - Aggarwal, Amita
Abstract: OBJECTIVES: Methotrexate (MTX) is one of the most widely used disease-modifying antirheumatoid drugs in the treatment of juvenile rheumatoid arthritis (JRA). We studied its effect on the production of two proinflammatory cytokines, interleukin-6 (IL-6) and tumor necrosis factor alpha (TNFalpha), by peripheral blood cells in patients with JRA. METHODS: Interleukin-6 and TNFalpha levels were measured at 0 and 4 weeks in whole blood cultures with and without lipopolysaccharide (LPS) stimulation in 19 children treated with MTX (10 mg/m(2 )per week) or placebo. Ten healthy individuals were included as healthy controls. RESULTS: Spontaneous production of IL-6 and TNFalpha by peripheral blood cells of patients with JRA was higher than in healthy controls ( P<0.01). However, IL-6 and TNFalpha production after LPS stimulation was similar in healthy controls and patients. The two groups of patients, i.e., those treated with placebo and those treated with MTX, had similar spontaneous and induced IL-6 and TNFalpha production. At 4 weeks, the drop in spontaneous IL-6 and TNFalpha production was no different in the two groups, but LPS-stimulated IL-6 production was significantly lower in the MTX-treated group than the placebo group ( P<0.05). CONCLUSION: Methotrexate reduces the production of IL-6 by activated cells, and this may be responsible for its anti-inflammatory property - AUTRET-LECA E: A general overview of the use of ibuprofen in paediatrics. Int J Clin Pract Suppl135 9-12, 2003
Organism: Tours University Hospital, FranceFAU - Autret-Leca, Elisabeth
Abstract: Ibuprofen is prescribed for children for the treatment of acute pain and fever, and for juvenile idiopathic arthritis. The pharmacokinetic characteristics of ibuprofen in children are similar to those in adults and the relationship between dose and response is linear over the range 5-10 mg/kg. Clinical trials of ibuprofen have shown the effective dose range to be 7.5-10 mg/kg. The maximum reduction in temperature occurs 3-4 hours after administration. In comparative clinical trials, ibuprofen has been shown to be equally as effective as or more effective than paracetamol as an analgesic and antipyretic and to have a longer duration of action; it is also as effective as aspirin. The adverse effects of ibuprofen are similar to those of other non-steroidal anti-inflammatory drugs but clinical experience suggests that ibuprofen is better tolerated by children than adults and it is safer in overdose than paracetamol and aspirin - BALLMANN M, JUNGE S, VON DER HH: Low-dose methotrexate for advanced pulmonary disease in patients with cystic fibrosis. Respir Med 97:5 498-500, 2003
Organism: Paediatric Department, Medical School Hannover, Hannover, Germany BallmannManfred@MH-HannoverdeFAU - Ballmann, M
Abstract: Inflammation is a hallmark in the pathogenesis of pulmonary destruction in cystic fibrosis (CF). There is no proven effective systemic anti-inflammatory treatment for CF patients with advanced pulmonary disease. Methotrexate (MTX) is known as an effective anti-inflammatory treatment in asthma and in juvenile rheumatoid arthritis. The question was: Is an improvement in pulmonary function achievable with low-dose MTX in patients with cystic fibrosis and advanced pulmonary disease.? METHODS: We treated five CF patients with advanced pulmonary disease, who deteriorated in spite of intensive conventional therapy on an individual basis with low-dose MTX. FEV1% and immunoglobulin G (IgG) serum levels were followed from the year before to the year after starting with MTX. RESULTS: In the year before starting with MTX, FEV1% decreased (median: 10% FEV1; range 9-15% FEV1; P<0.005) after starting with MTX, FEV1% increased (median: 9% FEV1; range: 2-15% FEV1; P<0.05). IgG changed (median: -2 g/l; range: 0.2 to -7.3 g/l) in the first year with MTX. CONCLUSION: These preliminary data suggest a beneficial effect of MTX even in advanced pulmonary disease in CF patients and supports the need for a controlled prospective study - BEGIC Z, MESIHOVIC H, DINAREVIC S, TERZIC R: [Morbidity trends in the cardio-rheumatology department of the Pediatric Clinic in Sarajevo during the pre-war, war and post-war periods]. Med Arh 56:3 Suppl 1 21-22, 2002
Organism: Pedijatrijska klinika, KCU SarajevoFAU - Begic, Zijo
Abstract: In this work is presented the structure of the morbidity of the patients treated at the Cardiohematology department of the Pediatric Clinic in Sarajevo. It were followed four the most frequent groups of the diseases. The results show the trend of the increasing of the number of the hospitalized children with the congenital anomalies of the heart and juvenile chronic arthritis, with the decrease of the number of the ill become by streptococcal infections of the upper respiratory tract and the rheumatic illness. It is increased the number of the hospitalized children is the result of the beginning of the cardiosurgical way of the taking care of these patients. The decreased of the number of the ill become is the consequence of the improvement of the healthcare protection at the primary level with the continuity of the further trend of the decrease of the number of the patients ill become from acute rheumatic disease - CELIKER R, BAL S, BAKKALOGLU A, OZAYDIN E, COSKUN T, CETIN A, DINCER F: Factors playing a role in the development of decreased bone mineral density in juvenile chronic arthritis. Rheumatol Int 23:3 127-129, 2003
Organism: Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Hacettepe University, 06100 Sihhiye, Ankara, Turkey celiker@trnetFAU - Celiker, Reyhan
Abstract: OBJECTIVE: The aims of this study were to evaluate bone mineral density (BMD) in patients with juvenile chronic arthritis (JCA), compare them with healthy controls, and assess the effects of disease activity and corticosteroid treatment on BMD. METHODS: Twenty-eight patients diagnosed with JCA and 45 healthy controls were included in this study. Disease activity was determined by clinical and laboratory evaluation, Articular Disease Severity Score (ADSS), and the Juvenile Arthritis Functional Assessment Report (JAFAR). Bone mineral density of the lumbar spine was measured by dual energy X-ray absorptiometry (DEXA). RESULTS: Patients with JCA showed significant decreases in BMD compared with healthy controls. The JCA patients treated with corticosteroids showed significantly lower BMDs than the healthy control group. Age of the patients and age of onset were found to correlate with BMD. CONCLUSION: Our study showed that glucocorticoids were involved in the development of osteoporosis in JCA, with many other factors affecting bone mineralization. We could not demonstrate any relationship between BMD and disease activity, but the study data suggest that early onset disease is also an important factor in the development of osteoporosis in JCA - CHIEN YH, YANG YH, HWU WL, CHOU CC, CHIANG BL: Common variable immunodeficiency with hypoglycemia, Kikuchi lymphadenitis, and hemiparesis in two siblings. J Microbiol Immunol Infect 36:1 65-68, 2003
Organism: Department of Pediatric, National Taiwan University Hospital, Taipei, Taiwan, ROCFAU - Chien, Yin-Hsiu
Abstract: Common variable immunodeficiency is a heterogeneous group of disorders with arthritis and/or arthralgia as its most commonly associated autoimmune manifestation. We report 2 cases of common variable immunodeficiency in siblings who also had other unusual signs and symptoms. A 10-year-old boy suffered from bradycardia, hypothermia, hypoglycemia, and chronic eczema. His 13-year-old sister suffered from Kikuchi lymphadenitis, hypoperfusion and atrophy of the left cerebral hemisphere, and hemiparesis. They both showed classical laboratory findings of common variable immunodeficiency and the boy's associated symptoms responded to intravenous immunoglobulin therapy. The findings from these cases suggest that a defect in the neuro-endocrine-immune axis may be one of the genetic bases of common variable immunodeficiency - DE BENEDETTI F, MEAZZA C, VIVARELLI M, ROSSI F, PISTORIO A, LAMB R, LUNT M, THOMSON W, RAVELLI A, DONN R, MARTINI A: Functional and prognostic relevance of the -173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis. Arthritis Rheum 48:5 1398-1407, 2003
Organism: IRCCS Ospedale Pediatrico Bambino Gesu, Rome, Italy debenedetti@opbgnetFAU - De Benedetti, Fabrizio
Abstract: OBJECTIVE: To address the functional and prognostic relevance of the -173 single-nucleotide G-to-C polymorphism of the macrophage migration inhibitory factor (MIF) gene in patients with systemic-onset juvenile idiopathic arthritis (systemic-onset JIA) by evaluating its association with serum and synovial fluid levels of MIF, with glucocorticoid requirement, and with the outcome of the disease. METHODS: A total of 136 patients with systemic-onset JIA were studied, including 98 patients from the British Paediatric Rheumatology Study Group's National Repository for JIA and 38 patients who were followed up at the IRCCS Policlinico San Matteo (Pavia, Italy) and the IRCCS G. Gaslini (Genoa, Italy). The MIF-173 polymorphism was genotyped using SnaPshot ddNTP primer extension and capillary electrophoresis. MIF levels were measured by enzyme-linked immunosorbent assay. The evaluation of the association of the MIF-173 polymorphism with outcome was performed only in Italian patients who were followed up for >5 years, by analyzing retrospectively 1) the number of joints with active arthritis and the number of joints with limited range of motion; 2) the score, at the last visit, on the Italian version of the Childhood Health Assessment Questionnaire (C-HAQ); and 3) data concerning the treatment regimens during the disease course. RESULTS: Systemic-onset JIA patients carrying a MIF-173*C allele had serum and synovial fluid levels of MIF significantly higher than those in patients with the GG genotype. The duration of glucocorticoid treatment on a daily regimen was significantly longer in patients carrying a MIF-173*C allele than in MIF-173 GG homozygous patients. Moreover, the duration of clinical response to intraarticular injection of triamcinolone hexacetonide was significantly shorter in patients carrying a MIF-173*C allele. At the last visit, the numbers of joints with active arthritis, the C-HAQ scores, and the numbers of joints with limited range of motion were significantly higher in patients carrying the MIF-173*C allele. CONCLUSION: Our study shows the functional relevance of the MIF-173 polymorphism and suggests that the MIF-173*C allele is a predictor of poor outcome in systemic-onset JIA - GERHARDT CA, VANNATTA K, MCKELLOP JM, TAYLOR J, PASSO M, REITER-PURTILL J, ZELLER M, NOLL RB: Brief report: child-rearing practices of caregivers with and without a child with juvenile rheumatoid arthritis: perspectives of caregivers and professionals. J Pediatr Psychol 28:4 275-279, 2003
Organism: Cincinnati Children's Hospital Medical Center and University of Cincinnati, Cincinnati, Ohio 45229-3039, USA cindygerhardt@chmccorgFAU - Gerhardt, Cynthia A
Abstract: OBJECTIVE: To evaluate predictions from professionals in pediatric rheumatology regarding the child-rearing practices of caregivers of children with juvenile rheumatoid arthritis (JRA) and healthy classmates. METHODS: Sixteen professionals identified items from the Child-Rearing Practices Report (CRPR) that were expected to differentiate between caregivers of children with JRA (64 mothers, 45 fathers) and caregivers of healthy classmates (64 mothers, 40 fathers). Families were interviewed, and physician ratings of disease severity were obtained. RESULTS: Experts predicted difficulties in protectiveness, discipline, and worry. Ratings from parents of children with JRA showed modest agreement with the professionals, surprising similarity to controls, and a limited association with disease factors. CONCLUSIONS: Contrary to expert opinion, JRA has only a modest influence on some child-rearing practices. Educating health care providers may minimize misperceptions about caring for children with JRA, and screening parents of children with more severe disease may assist in allocating education and services for families - GRATEAU G: [What has become of inflammatory amyloidosis?]. Rev Prat 53:5 516-519, 2003
Organism: Service de medecine interne L'Hotel-Dieu 75181 Paris gillesgrateau@htdap-hop-parisfrFAU - Grateau, Gilles
Abstract: Inflammation associated amyloidosis (AA amyloid) remains a severe complication of chronic inflammatory diseases, namely of rheumatoid arthritis and of juvenile arthritis. Infectious causes of AA amyloid have become uncommon. In the course of chronic inflammatory diseases, amyloid should be regularly screened by proteinuria. The proof of amyloidosis requires tissue analysis examplifying specific apple-green birefringence after staining with Congo red. When amyloidosis is progressive in the course of one chronic disease, powerful anti-inflammatory and immunosuppressive drugs can be used - HUEMER M, FODINGER M, HUEMER C, SAILER-HOCK M, FALGER J, RETTENBACHER A, BERNECKER M, ARTACKER G, KENZIAN H, LANG T, STOCKLER-IPSIROGLU S: Hyperhomocysteinemia in children with juvenile idiopathic arthritis is not influenced by methotrexate treatment and folic acid supplementation: a pilot study. Clin Exp Rheumatol 21:2 249-255, 2003
Organism: Department of Pediatrics, University of Vienna, Vienna, Austria m333huemer@aonatFAU - Huemer, M
Abstract: OBJECTIVE: Our first objective was to compare plasma total homocysteine (tHcy) concentrations in juvenile idiopathic arthritis (JIA) patients requiring methotrexate (MTX) treatment and healthy children. Our second aim was to evaluate the influence of low-dose (10-15 mg/m2/week) MTX treatment combined with folic acid supplementation (1 mg/d) or placebo on tHcy concentrations in JIA patients. METHODS: In 17 JIA patients and 17 age- and sex-matched healthy children, baseline tHcy concentrations were measured. When MTX treatment was initiated, JIA patients were randomly assigned to folic acid 1 mg/d/p.o. followed by placebo (8 weeks each) or vice versa. Blood samples for measurement of tHcy, vitamin B6, B12 and folate were taken after 4 weeks, 12 weeks and 20 weeks of treatment. RESULTS: 1) In the healthy children the mean tHcy concentration was 6.3 +/- 1.68 mumol/l as compared to 9.99 +/- 5.17 mumol/l in JIA patients (p < 0.04). At baseline, 5/17 JIA patients had tHcy concentrations > 10.5 mumol/l, the 99th percentile for teenagers. 3/5 patients even exceeded the upper normal level for adults (tHcy > or = 15 mumol/l). MTX treatment did not result in a significant increase of tHcy and folic acid supplementation had no significant impact on tHcy levels. CONCLUSION: This pilot study shows that patients with JIA requiring MTX treatment have significantly elevated baseline plasma tHcy concentrations compared to age- and sex-matched healthy controls. No significant impact of MTX and folate supplementation on tHcy concentration was found - IHLE J, FLECKENSTEIN B, TERREAUX C, BECK H, ALBERT ED, DANNECKER GE: Differential peptide binding motif for three juvenile arthritis associated HLA-DQ molecules. Clin Exp Rheumatol 21:2 257-262, 2003
Organism: University Children's Hospital, Eberhard Karls University, Tubingen, Germany johannesihle@webdeFAU - Ihle, J
Abstract: OBJECTIVE: In the oligoarticular subgroup of juvenile idiopathic arthritis, a strong association has been found with the expression of human leukocyte antigen class II molecules HLA-DQA1 *0401-DQB1*0402 and DQA1*0501-DQB1*0301, whereas DQA1*0501-DQB1*0201 is neutral and DQA1 *0201-DQB1*0201 protective. A presentation of different peptides by these DQ alleles would support their role in the disease process. METHODS: Using a synthetic nonapeptide library, a peptide binding motif was determined for the associated DQA1*0501-DQB1*0301 molecule and compared to the neutral and the protective DQ molecules. RESULTS: A differential motif for the three molecules could be deduced, suggesting that peptides preferentially binding to the associated vs. the neutral/protective DQ-molecules are mutually exclusive. CONCLUSION: These results imply a role for differential peptide presentation in the pathogenesis of oligoarthritic JIA. The search for peptides initiating the disease process might be facilitated which could then lead to therapeutical interventions - KNIPP S, FEYEN O, NDAGIJIMANA J, NIEHUES T: Ex vivo apoptosis, CD95 and CD28 expression in T cells of children with juvenile idiopathic arthritis. Rheumatol Int 23:3 112-115, 2003
Organism: Department of Pediatrics, Pediatric Immunology and Rheumatology, Heinrich Heine University of Dusseldorf, 40225, Dusseldorf, GermanyFAU - Knipp, Sabine
Abstract: We hypothesise that T-cell apoptosis and the percentage of T cells expressing molecules involved in apoptosis modulation (CD95, CD28) are altered at the inflammation site and in peripheral blood (PB) of children with juvenile idiopathic arthritis (JIA). Paired JIA samples of synovial fluid (SF) and PB ( n=7) and PB samples from age-matched normal children ( n=23) were analysed immediately ex vivo. Apoptosis was measured by detection of phophatidylserine (PS) externalisation on T cells. CD95 or CD28 was detected by FACS, and soluble CD95 and CD95 ligand levels were detected by enzyme-linked immunosorbent assay (ELISA). In SF, the mean percentage of apoptotic T cells was somewhat higher than in PB. However, the percentages of T cells expressing CD95 and soluble CD95 levels were markedly higher in SF (CD4 cells 96+/-2%, CD8 91+/-6%, soluble CD95 6,420+/-2,571 pg/ml) than in PB (CD4 32+/-10%, CD8 36+/-9%, soluble CD95 4,296+/-2,142 pg/ml). Peripheral blood T-cell apoptosis in JIA (CD4 20+/-8%, CD8 42+/-19%) was higher than in the control group (CD4 5+/-2%, CD8 9+/-6%). Interestingly, the percentage of PB CD4 cells expressing CD28 was lower in JIA than controls. In conclusion, systemic T-cell apoptosis was higher in JIA while a substantial number of SF T cells survived locally, despite the fact that almost all cells express CD95 - LAINE M, VAINIONPAA R, UKSILA J, OKSI J, NISSILA M, KAARELA K, LUUKKAINEN R, TOIVANEN A: Prevalence of Sindbis-related (Pogosta) virus infections in patients with arthritis. Clin Exp Rheumatol 21:2 213-216, 2003
Organism: Department of Medicine, Medical Microbiology, Turku University, Turku, FinlandFAU - Laine, M
Abstract: OBJECTIVE: To determine the role of Pogosta virus as a triggering infection in non-specific arthritis. METHODS: Serum samples of 142 patients with acute arthritis were screened for the evidence of Pogosta virus infection. Serological tests for Chlamydia trachomatis, salmonella, parvovirus B19, and Borrelia burgdorferi were also carried out. As verified later, 78 of the patients had rheumatoid arthritis and 63 seronegative poly- or oligoarthritis, while one had systemic lupus erythematosus. RESULTS: In the early stage of the joint symptoms 4 patients with rheumatoid arthritis, 1 with seronegative polyarthritis and 1 with systemic lupus erythematosus had recent Pogosta virus infection. Four of them had probably had Pogosta disease at the time of the onset of arthritis. In 11 patients with a diagnosis of seronegative arthritis, serological evidence of preceding infection due to salmonella or Chlamydia trachomatis was found, strongly suggesting classical reactive arthritis in these cases. CONCLUSIONS: Our study suggests that also a Sindbis virus infection may be associated both to an acute joint inflammation as a part of Pogosta disease or chronic arthritis. At present, this possibility still needs further research - LEPORE L, MARCHETTI F, FACCHINI S, LEONE V, VENTURA A: Drug-induced systemic lupus erythematosus associated with etanercept therapy in a child with juvenile idiopathic arthritis. Clin Exp Rheumatol 21:2 276-277, 2003
- MCMINN JR, JR., COHEN S, MOORE J, LILLY S, PARKHURST J, TARANTINO MD, TERRELL DR, GEORGE JN: Complete recovery from refractory immune thrombocytopenic purpura in three patients treated with etanercept. Am J Hematol 73:2 135-140, 2003
Organism: Hematology-Oncology Section, Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City 73190, USAFAU - McMinn, J R Jr
Abstract: Management of patients with immune thrombocytopenic purpura (ITP) who have persistent, severe, and symptomatic thrombocytopenia following splenectomy is difficult and empirical. No single agent or regimen provides long-term success for most patients, and for most treatments it is difficult to assess whether benefits outweigh risks. We report three consecutive patients with critical chronic refractory ITP, who responded promptly and completely following treatment with etanercept, an inhibitor of tumor necrosis factor-alpha. These patients had failed 6-11 previous treatments. In the first patient, etanercept was given for its approved indication: a flare of co-existing rheumatoid arthritis. The next two patients were treated with etanercept because of successful outcomes in the previous patients. Although etanercept appeared to be effective treatment for ITP in these 3 patients, the experimental nature of this treatment and the potential risks must be emphasized. On the basis of these case reports, a clinical trial has been initiated to systematically evaluate the efficacy and risks of etanercept in the management of children and adults with chronic ITP - MERA VA, BLANCO RJ, CAAMANO FM: [Approximation to the cost of pharmacological treatment of rheumatoid arthritis in Spain]. An Med Interna 20:3 114-121, 2003
Organism: Servicio de Reumatologia, Complejo Hospitalario Universitario, Facultad de Medicina, Universidad de Santiago, Santiago de Compostela mereuma@uscesFAU - Mera Varela, A
Abstract: OBJECTIVE: To study the direct cost derived of utilization of pharmaceutical compounds in the treatment of rheumatoid arthritis(RA). MATERIAL AND METHODS: A prospective study with 150 patients (125 women/25 men) suffered of RA was carry out in Galicia-Spain public hospital. The mean age was 60.2 years, with a mean lengthy of disease of 11.2 years (1-53); the 64.4% come from rural areas. A personal interview was made with a complete registry of all data: demographic, activity score (ESR, Swollen joints), radiological status, functional class (ACR), extrarticular manifestations and co-morbid diseases. Such data was accomplished with all the medications employees with a calculation of monthly or annual cost for the different therapeutic groups and a final total cost. The statistical study was made with Excel's (Microsoft) and the Analysis Tool Pack. RESULTS: The 53% of patients was in functional class I and 52% in radiological stage I or II (Steinbrocker) whereas 16 patients was considered in remission. Non-steroideal ant-inflammatory drugs (NSAID) were used in the 82.7% of patients with a monthly cost of 12.71 [symbol: see text] (1.10-80). Corticosteroids at low doses were used in 90.7% with a monthly cost of 5.17 [symbol: see text] (1.24-33.7). The Disease Modifying Anti-rheumatic Drugs (DMARD) was used in 94% of cases, the most common methotrexate and association of two or more in 21%. The mean monthly cost for a single DMARD was 3.63 [symbol: see text] and for two, 13.75 [symbol: see text]. Gastroprotection and therapy for co-morbid diseases was employee in 80% and 95% of cases, with a monthly cost of 36.9 [symbol: see text] and year cost of 568.6 [symbol: see text], respectively. The study included 23 patients under treatment with anti-TNF therapy with a monthly mean cost of 933.8 [symbol: see text]. For pharmaceuticals exclusively for RA, annual cost was 342.8 [symbol: see text] excluding anti-TNF therapy, but with wide variation (6.4-2.910 [symbol: see text]). If we include all patients with anti-TNF therapy, gastro-protection and co-morbid situations in a calculation, the mean cost was 2.587 [symbol: see text] year. The most important cost was found in patients with 50-70 years-old and existing a good correlation between the final burden and use of medications for co-morbid conditions, gastro-protection, use of anti-TNF, age, lengthy of disease between 5-10 years and number of swollen joints, but not radiological stage. CONCLUSIONS: The economic burden for pharmaceuticals used in RA is very variable depending of some variables, including the proper disease and other related conditions. The most important cost occur in case of use of anti-TNF therapy. In the most frequent conditions, gastro-protection and therapy for co-morbid diseases lead the 62% of total annual burden, followed by the use of DMARD and in a minor load, the NSAIDs and corticosteroids - MERT A, OZARAS R, TABAK F, BILIR M, OZTURK R, OZDOGAN H, AKTUGLU Y: Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. Clin Rheumatol 22:2 89-93, 2003
Organism: Cerrahpasa Medical Faculty, Department of Infectious Disease and Clinical Microbiology, University of Istanbul 34303, Aksaray, Istanbul, Turkey doktoralimert@yahoocomFAU - Mert, A
Abstract: In this study we aimed to investigate the findings in patients with adult-onset Still's disease (AOSD) admitted with fever of unknown origin (FUO) during the last 18 years in our unit, in order to discover the ratio of such patients to all patients with FUO during the same period, and to determine the clinical features of AOSD in FUO. The number and the aetiologies of the patients with FUO diagnosed between 1984 and 2001, and the clinical features of those with AOSD, were taken from the patient files. The diagnosis of AOSD was reanalysed according to the diagnostic criteria of Cush et al. [11]. The presumed diagnoses before a diagnosis of AOSD was established were also noted. The chi(2) and Fisher's exact tests were used for statistical analysis. We studied 130 patients with a diagnosis of FUO, 36 (28%) of whom had collagen vascular diseases. Of these 36 patients, 20 (56%, 12 female, 8 male, mean age 34 years, range 16-65) had AOSD. Clinical and laboratory findings were as follows: fever (100%), arthralgia (90%), rash (85%), sore throat (75%), arthritis (65%), myalgia (60%), splenomegaly (40%), hepatomegaly (25%), lymphadenopathy (15%), anaemia (65%), neutrophilic leukocytosis (90%), increased erythrocyte sedimentation rate (100%), elevated transaminase levels (65%), a negative RF (100%), and a negative FANA (80%). Antibiotics had been prescribed in 18 (90%) of cases. The presumed infectious diagnoses were streptococcal tonsillitis/pharyngitis (50%), infective endocarditis (four patients), sepsis (two patients) and acute bacterial meningitis (two patients). The presumed non-infectious diagnoses were acute rheumatic fever (three patients), seronegative rheumatoid arthritis (two patients) and polymyositis (two patients). Sixteen patients were followed for a mean duration of 30 months (range 2-59). A remission was obtained with indomethacin in three cases (19%), and with prednisolone in the remainder. Relapse was detected in three cases (19%). AOSD is one of the most frequent aetiologies of FUO. During the diagnostic course of a patient with FUO, a maculopapular rash and/or arthralgia and/or sore throat should raise the suspicion of AOSD. Because the disease has heterogeneous clinical findings, certain bacterial infections (e.g. streptococcal pharyngitis and sepsis) are generally considered and the prescribing of antibiotics is common - NORRBY U, NORDHOLM L, FASTH A: Reliability and validity of the swedish version of child health questionnaire. Scand J Rheumatol 32:2 101-107, 2003
Organism: Department of Pediatrics, the Queen Silvia Children's Hospital, Goteborg University, Sweden ullanorrby@brackeorgFAU - Norrby, Ulla
Abstract: Child Health Questionnaire (CHQ) is a North American self-administered instrument to assess physical and psychosocial health status for children (CHQ-CF) and their parents (CHQ-PF). We have determined reliability and validity of the CHQ on 45 children with JCA, who were treated as outpatients. Reliability (Cronbach's alpha) ranged between .86 and .94 for the dimensions of the CHQ-CF and between .69 and .94 for the CHQ-PF. Significant correlations (p<.01) were obtained between 7 of the CHQ dimensions and the Child Health Assessment Questionnaire (CHAQ) categories, and were particularly strong in the physical domain. Evidence of validity was found through significant correlations (p<0.01) between the CHQ and disease variables. Correlations between parents' and children's responses ranged between .366 and .786. CHQ was found to be valid and reliable for children with JCA and their parents. It seems particularly important to recommend that the children themselves are asked about their perceptions of health status and quality of life - OZCAKAR L, DINCER F, OZCAKAR ZB: Juvenile chronic arthritis in a monozygotic twin couple. Rheumatol Int 23:3 149-150, 2003
- RADSTAKE TR, PETIT E, PIERLOT C, VAN DE PUTTE LB, CORNELIS F, BARRERA P: Role of Fcgamma receptors IIA, IIIA, and IIIB in susceptibility to rheumatoid arthritis. J Rheumatol 30:5 926-933, 2003
Organism: Department of Rheumatology, University Medical Centre St Radboud, Nijmegen, The Netherlands tradtake@reumaaznnlFAU - Radstake, Timothy R D J
Abstract: OBJECTIVE: To investigate the role of Fcg receptor (FcgR) genes in susceptibility to rheumatoid arthritis (RA) using family based studies, to examine possible interactions between FcgR genotypes and the shared epitope (SE), and to assess linkage disequilibrium between FcgR loci. METHODS: Association studies were performed in 95 Caucasian, single-case, nuclear Caucasian families with both parents alive using haplotype based haplotype relative risk (HHRR) and transmission disequilibrium test (TDT) statistics. Three FcgR polymorphisms (FcgRIIA-131H/R, FcgRIIIA-158V/F, and FcgRIIIB-NA1/NA2) were genotyped using polymerase chain reaction methods. Linkage analysis was performed using 3 microsatellite markers (D1S498, D1S2844, D1S2762) flanking the FcgR region in an independent set of 90 Caucasian, multiple-case families. Potential effects of disease heterogeneity, including sex and the presence of rheumatoid factor, SE, and erosive or nodular disease, were taken into account in the analysis. Logistic regression analysis was performed to determine whether FcgR alleles are independent risk factors for the susceptibility to and/or severity of RA. Linkage disequilibrium was calculated using pairwise linkage disequilibrium statistics. RESULTS: HHRR and TDT analysis showed no evidence of preferential transmission of any FcgR alleles studied, and there were no important associations with any given disease phenotype. Moreover, neither linkage to microsatellite markers close to the FcgR genes on chromosome 1 nor linkage disequilibrium between FcgR loci was present in our population. The distribution of inherited genotypes provided evidence for an interaction between the SE and the FcgRIIIA-158V allele and between the SE and the FcgRIIIA-158V-FcgRIIA-131H 2-locus haplotype since the combined presence of these factors increased the susceptibility to RA (OR 4.13, 95% CI 1.6-10.62 and OR 2.83, 95% CI 1.25-6.38, respectively). However, regression analysis showed that neither the 158V allele nor the 158V-131H haplotype contributed as independent factors to susceptibility or severity of RA. CONCLUSION: Isolated FcgR genes do not play a major independent role in susceptibility to RA. To a limited extent, the presence of high-binding alleles at the FcgRIIIA locus or at the FcgRIIIA-FcgRIIA haplotype might predispose to RA in SE positive individuals - ROGOZ S, AVRAMESCU C, SILOSI I, DRACKOULOGONA O, BADEA P, STATE A: [Immunopathologic mechanism in some ocular diseases]. Oftalmologia 54:3 51-55, 2002
Organism: UMF CraiovaFAU - Rogoz, Suzana
Abstract: OBJECTIVES: The goal of the present study is to elucidate the pathogenic mechanisms that determine the ocular involvement in some systemic diseases. MATERIAL AND METHODS: We have studied 45 patients with ocular diseases and concomitant systemic diseases (systemic lupus erythematosus-SLE, ankylosing spondylitis, Reiter syndrome, juvenile rheumatoid arthritis, Sjogren syndrome). The patients studied have been compared with a control group represented by 20 healthy individuals. In each case we have determined the serum levels of the following humoral markers: the C3 complement fraction, circulating immune complexes, autoantibodies (antinuclear autoantibodies, anti-double stranded DNA autoantibodies, anti-smooth muscle autoantibodies, anti-neutrophilic cytoplasm autoantibodies, anti-cardiolipin autoantibodies, anti-SSA, anti-SSB, rheumatoid factor). RESULTS: The autoantibodies detected in certain cases of uveitis suggest the existence of an autoimmune etiology; the antibody titers have been high in some severe cases of rheumatic uveitis, especially in patients with ankylosing spondylitis. We have found that there is a correlation between the presence and the titer of certain autoantibodies, and the onset and evolution of the disease. Specifically, the antinuclear autoantibodies may be considered a predictive factor for the development of chorio-retinopathy of SLE. Uveitis mediated by immune complexes has been seen in rheumatoid arthritis, ankylosing spondylitis, and SLE, these being the same systemic diseases where there is an important pathogenic role of immune complexes. CONCLUSIONS: The abnormal immunologic function, that was observed in the ocular inflammatory diseases studied, may be synthesized by the association between two immunopathologic phenomena; both of them are finalized by the non-self transformation of the antigens linked to various eye structures. These are represented by autoimmunity (of humoral, cellular, or mixed type), and the immediate type III hypersensitivity reaction. This study provides important data regarding the involvement of autoimmune diseases in the etiology of ocular diseases - RUDLER M, POUCHOT J, PAYCHA F, GENTELLE S, GRASLAND A, VINCENEUX P: Low dose methotrexate osteopathy in a patient with polyarticular juvenile idiopathic arthritis. Ann Rheum Dis 62:6 588-589, 2003
- RUSCONI R, CORONA F, GRASSI A, CARNELLI V: Age at menarche in juvenile rheumatoid arthritis. J Pediatr Endocrinol Metab 16 Suppl 2:285-8.: 285-288, 2003
Organism: Unit of Endocrinology, University of Milan, Italy dnakr@tinitFAU - Rusconi, Roberto
Abstract: OBJECTIVE: To study the effect on the timing of menarche of the different subtypes of juvenile rheumatoid arthritis (JCA) and their therapies. METHODS: Auxological data (including the age at menarche of themselves and their mothers) were collected from 83 females regularly attending our rheumatological unit. Malnourished patients were excluded from the study. The patients were divided into groups on the basis of disease subtype and glucocorticoid (GC) therapy. RESULTS: The timing of menarche was later in all the patients in comparison with their mothers and normal Italian girls, and those who had taken GC had a later menarche and less pubertal growth than those who had not. The girls with systemic JCA had a later menarche than those with the polyarticular or pauciarticular subtypes. CONCLUSIONS: Our results show that pubertal development is compromised in girls with JCA, particularly if they have received GC therapy - SAILA H, SAVOLAINEN A, KAUPPI M, ALAKULPPI N, TUOMILEHTO-WOLF E, TUOMILEHTO J, LEIRISALO-REPO M, AHO K: Occurrence of chronic inflammatory rheumatic diseases among parents of multiple offspring affected by juvenile idiopathic arthritis. Clin Exp Rheumatol 21:2 263-265, 2003
Organism: Rheumatism Foundation Hospital, Fin-18120, Heinola, Finland HannaSaila@reumafiFAU - Saila, H
Abstract: OBJECTIVE: The rarity of reports on extended multiplex families points out that the genetic component in juvenile idiopathic arthritis (JIA) might not be particularly strong. Our objective was to determine the frequency of chronic inflammatory rheumatic diseases among the parents who had two or more offspring affected by JIA. METHODS: During the last 17 years patients with JIA treated at the Rheumatism Foundation Hospital in Heinola and their parents have been systematically asked about the familial occurrence of rheumatic diseases. A total of 45 families with more than one sibling affected by JIA were found among about 2,300 JIA cases. In these "multicase families", 9 parents from 8 families also had a diagnosis of chronic inflammatory rheumatic disease. Their case histories were studied. RESULTS: Four of the parents had had JIA (one subsequently developed ankylosing spondylitis), and 4 had rheumatoid factor-negative chronic arthritis (one had also had chronic iritis since the age of 10, resembling that seen in JIA). Three of them had features of JIA and only one met the classification criteria for rheumatoid arthritis. One had ankylosing spondylitis. CONCLUSIONS: Since the expected number of JIA cases among the 90 parents was about 0.2, there was drastic increase in JIA frequency among the parents in families with multiple offspring also affected by JIA. These results suggest that JIA susceptibility genes may likely be clustered in these families - SCHANBERG LE, ANTHONY KK, GIL KM, MAURIN EC: Daily pain and symptoms in children with polyarticular arthritis. Arthritis Rheum 48:5 1390-1397, 2003
Organism: Division of Rheumatology, Duke University Medical Center, Durham, North Carolina 27710, USA schan001@mcdukeeduFAU - Schanberg, Laura E
Abstract: OBJECTIVE: To analyze patterns of daily pain, stiffness, and fatigue related to juvenile arthritis; to examine the relationships of demographics, disease severity, and psychological adjustment to daily disease symptoms; and to examine daily disease symptoms as predictors of reduced participation in school and social activity. METHODS: For a 2-month period, 41 children with polyarticular juvenile arthritis completed daily diaries that included measures of symptoms and function. Children also underwent an initial evaluation and 4 followup evaluations that included a joint count, laboratory testing, and completion of questionnaires assessing physical and psychosocial functioning. RESULTS: Children reported having pain an average of 73% of days, with the majority of children (76%) reporting pain on >60% of all days. On average, children described the intensity of their daily pain as being in the mild to moderate range; however, a significant subgroup (31%) reported pain in the severe range. Higher physician global assessment ratings, increased functional disability, and increased anxiety were significantly associated with increased daily pain and other daily symptoms. Multilevel random-effects analyses indicated that increased daily symptoms of pain, stiffness, and fatigue were significant predictors of reduced participation in school and social activities. CONCLUSION: Physicians should consider treating pain more aggressively in children with arthritis, in order to preserve function in school and social domains, as well as physical function. Moreover, optimal pain management in children with arthritis should include therapeutic regimens addressing anxiety as well as standard pharmacologic interventions - SHEN Y, QU Q, WANG D: Clinical observation on treatment of rheumatoid arthritis with fengshi xiandan in 53 cases. J Tradit Chin Med 23:1 21-26, 2003
Organism: Danjiangkou Municipal First Hospital, Hubei Province 442700FAU - Shen, Yujie
Abstract: We have successfully developed Fengshi Xiandan ([symbol: see text]) and used it to treat 53 cases of rheumatoid arthritis (RA), with indomethacin used to treat another 53 cases of RA as the controls. The results showed that the rate of cure and obvious effect was 54.7% and the total effective rate was 92.4% in the treatment group, as compared to 26.4% and 66.0% respectively in the control group, with an obvious difference in curative effect between the two groups (P < 0.01). The improvement of all the main clinical symptoms and signs except arthralgia in the treatment group was superior to that in the control group (P < 0.05 or P < 0.01). The improvement of erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), C-reaction protein (CRP), immunoglobulin (Ig), T-lymphocyte subgroups, micro-circulation in nail fold, and red blood cell (RBC) and hemoglobin (Hb) of anemia patients in the treatment group was superior to that in the control group (P < 0.05 or P < 0.01). There was an obvious difference (P < 0.05 or P < 0.01) in superoxide dismutase (SOD) of RBC and lipid peroxide (LPO) of plasma before treatment in the two groups as compared to normal group (P < 0.05 or P < 0.01). The level of SOD and LPO after treatment in the treatment group obviously tended to normal as compared to that before treatment (P < 0.05 or P < 0.01). The reduction of LPO in the treatment group was obviously superior to that in the control group (P < 0.01). The result shows that Fengshi Xiandan ([symbol: see text]) with an obvious curative effect on RA can not only control the symptoms of RA but also improve its pathologic damage and prevent its development - SIDDIQUE MS, RAO MC, DEEHAN DJ, PINDER IM: Role of abrasion of the femoral component in revision knee arthroplasty. J Bone Joint Surg Br 85:3 393-398, 2003
Organism: Department of Orthopaedics, Freeman Hospital, Newcastle upon Tyne, England, UKFAU - Siddique, M S
Abstract: We carried out 60 revision procedures for failed porous coated anatomic total knee replacements in 54 patients, which were divided into two groups. The 14 knees in group I had a well-fixed femoral component at surgery which was retained, and in the 46 knees in group II both tibial and femoral components were loose and were revised using a variety of implants. Our review comprised clinical and radiological assessment. A total of 13 knees required a second revision. Six (42%) in group I failed very early (mean 2.1 years) when compared with seven (15%) in group II (mean 6.8 years). Failure was due to wear of the polyethylene insert by the abraded, retained femoral component (crude odds ratio 4.07; 95% CI 1.07 to 15.5). We recommend a complete change of primary bearing surfaces at the time of revision of an uncemented total knee replacement in order to prevent early wear of polyethylene - SILLS JA: Consistency for childhood arthritis. J Rheumatol 30:5 1123, 2003
- SILVERIO AMANCIO OM, ALVES CHAUD DM, YANAGUIBASHI G, ESTEVES HILARIO MO: Copper and zinc intake and serum levels in patients with juvenile rheumatoid arthritis. Eur J Clin Nutr 57:5 706-712, 2003
Organism: Department of Pediatrics, Universidade Federal de Sao Paulo/Escola Paulista de Medicina, Sao Paulo, BrazilFAU - Silverio Amancio, O M
Abstract: OBJECTIVE: To evaluate the copper and zinc intake and serum levels in patients with juvenile rheumatoid arthritis (JRA), considering the pauci and polyarticular types, the disease activity and duration, the number of inflamed joints and the use of corticosteroids therapy. DESIGN: Cross-sectional study with control group. SETTING: Outpatients of the pediatric rheumatology public health clinic, of the Universidade Federal de Sao Paulo/Escola Paulista de Medicina, Brazil. SUBJECTS: Forty-one patients with JRA were evaluated and 23 patients' brothers, as a control group. INTERVENTIONS: Copper and zinc intake evaluation by Food Register method. Copper and zinc serum levels by atomic absorption spectrophotometry. RESULTS: The disease activity did not determine difference in copper (P=0.624) and zinc (P=0.705) intake, being predominantly below the Recommended Dietary Allowances. The serum copper in relation to control was statistically greater (P=0.018), showing that the number of inflamed joints is statistically significantly related with its variation (P=0.001). The serum zinc was not different either in relation to control (P=0.940) or to the disease characteristics. CONCLUSIONS: The evaluation of copper intake seems to be of fundamental importance. It may influence the efficiency of the organic serum response. More research is needed to indicate, with security, adequate zinc intake - SKYTTA E, SAVOLAINEN A, KAUTIAINEN H, LEHTINEN J, BELT EA: Treatment of leg length discrepancy with temporary epiphyseal stapling in children with juvenile idiopathic arthritis during 1957-99. J Pediatr Orthop 23:3 378-380, 2003
Organism: Orthopaedic Department, Rheumatism Foundation Hospital, Heinola, Finland eerikskytta@phksfiFAU - Skytta, Eerik
Abstract: Temporary epiphyseal stapling is a flexible method for correction of leg length discrepancy. Due to a high risk of premature epiphyseal plate closure and other complications reported in previous studies, the technique has not been in wide use. In this study a retrospective analysis of 71 knees in patients with juvenile idiopathic arthritis was performed to determine the safety and effectiveness of the method. Only seven minor reversible complications were encountered, and the authors found the method suitable even for immunologically compromised patients - THOMAS E, SYMMONS DP, BREWSTER DH, BLACK RJ, MACFARLANE GJ: National study of cause-specific mortality in rheumatoid arthritis, juvenile chronic arthritis, and other rheumatic conditions: a 20 year followup study. J Rheumatol 30:5 958-965, 2003
Organism: Primary Care Sciences Research Centre, Keele University, Keele, EnglandFAU - Thomas, Elaine
Abstract: OBJECTIVE: To quantify risks for cause-specific mortality among hospitalized patients with rheumatoid arthritis (RA), juvenile chronic arthritis (JCA), and 4 other rheumatic conditions in a nationwide, population based cohort over a 20 year period. METHODS: All subjects were identified from Scottish hospital inpatient records from 1981 to 2000 and were followed up by computer linkage to the national registry of deaths. Expected mortality was calculated from national mortality rates and was related to the observed incidence by the standardized mortality ratio (SMR) and the corresponding 95% confidence interval (95% CI). RESULTS: Overall mortality was elevated in each of the 6 rheumatic conditions examined, most notably in JCA (males: SMR 3.4, 95% CI 2.0,5.5; females: SMR 5.1, 95% CI 3.2,7.8). Among patients with RA, there was an increased risk for death in all International Classification of Disease chapters other than those relating to mental disorders. Specific causes of death with an increased risk for subjects with RA included lung cancer [males: 1.4 (1.2,1.5); females: 1.6 (1.5,1.8)], hematopoietic malignancies [M: 1.8 (1.4,2.3); F: 2.0 (1.7,2.3)], coronary artery disease (CAD) [M: 1.6 (1.5,1.7); F: 1.95 (1.9,2.0)], respiratory infections [M: 1.9 (1.7,2.2); F: 2.4 (2.3,2.6)], chronic obstructive pulmonary disease [M: 1.8 (1.6,2.0); F: 2.1 (1.9,2.3)], and renal failure [M: 3.1 (2.5,3.9); F: 3.5 (3.0,4.0)]. Conversely, RA subjects were less likely to die from gastrointestinal tract malignancies [M: 0.82 (0.7,1.0); F: 0.8 (0.7,0.9)]. CONCLUSION: Population studies for primary data collection are required to extend our knowledge about the underlying mechanisms of early mortality in patients with rheumatic conditions - ZHANG M, CHEN JQ, LIU ZG, LOU LH, XIAO QG, YAO Y, CHEN LS, ZHANG ZH: [Clinical characteristics of patients with dry eye syndrome]. Zhonghua Yan Ke Za Zhi 39:1 5-9, 2003
Organism: Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou 510060, ChinaFAU - Zhang, Mei
Abstract: OBJECTIVE: To learn the clinical characteristics of patients with dry eye syndrome. METHODS: The following items were recorded in 115 patients (229 eyes) with dry eye, including symptoms, causation, systematic diseases, slit-lamp examination, tear break-up time, basal and reflex Schirmer's test, vital staining (fluorescent and rose bangle) and meibomian gland dysfunction examination. Rheumatoid factor and auto-antibody detection were performed in Sjogren's syndrome suspected patients. RESULTS: Aqueous tear deficiency (ATD, 48.7%) ranked the most common type, followed by over-evaporation dry eye (34.8%), mixed type (13.9%) and conjunctivochalasis (3.5%). In all the causes of the dry eye, about 11.3% had Sjogren syndrome (SS). Females suffering from dry eye were more than males, especially SS. Dryness was the most common symptom (84.0%), especially in ATD patients, then followed by ocular fatigue (72.0%), foreign body sensation (64.0%) and impairment of vision (56.0%). The ocular irritation was more severe in meibomain gland dysfunction (MGD) patients than in ATD patients. Among the results of tear break-up time (BUT), rose bangle (Rb) staining and fluorescent (Fl) staining in all types of dry eye, significant relationship was found among them, especially between Rb and Fl score (r = 0.612, P = 0.000). SS patients had much more severe abnormality in all the four signs than non-SS aqueous tear deficiency (NSTD) and MGD patients. However, in the comparisons of BUT, Rb and Fl between NSTD and MGD patients, there were no significant differences. CONCLUSION: Symptoms combined with examinations of BUT, Schirmer's test, Fl and Rb staining and meibomian gland function are the necessary means to diagnose most of the dry eye patients