Bibliography October 2002

1. AGANNA E, MARTINON F, HAWKINS PN, ROSS JB, SWAN DC, BOOTH DR, LACHMANN HJ, GAUDET R, WOO P, FEIGHERY C, COTTER FE, THOME M, HITMAN GA, TSCHOPP J, MCDERMOTT MF: Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosis. Arthritis And Rheumatism 2002, 46:2445-2452.
   Organism:M.F. McDermott, Alexandra Wing, Royal London Hospital, Whitechapel, London E1 1BB
   Abstract: Objective. Familial cold urticaria (FCU) and Muckle-Wells syndrome (MWS) are dominantly inherited autoinflammatory disorders that cause rashes, fever, arthralgia, and in some subjects, AA amyloidosis, and have been mapped to chromosome 1q44. Sensorineural deafness in MWS, and provocation of symptoms by cold in FCU, are distinctive features. This study was undertaken to characterize the genetic basis of FCU, MWS, and an overlapping disorder in French Canadian, British, and Indian families, respectively. Methods. Mutations in the candidate gene NALP3, which has also been named CIAS1 and PYPAF1, were sought in the study families, in a British/Spanish patient with apparent sporadic MWS, and in matched population controls. Identified variants were sought in 50 European subjects with uncharacterized, apparently sporadic periodic fever syndromes, 48 subjects with rheumatoid arthritis (RA), and 19 subjects with juvenile idiopathic arthritis (JIA). Results. Point mutations, encoding putative protein variants R262W and L307P, were present in all affected members of the Indian and French Canadian families, respectively, but not in controls. The R262W variant was also present in the subject with sporadic MWS. The V200M variant was present in all affected members of the British family with MWS, in 2 of the 50 subjects with uncharacterized periodic fevers, and in 1 of 130 Caucasian and 2 of 48 Indian healthy controls. No mutations were identified among the subjects with RA or JIA. Conclusion. These findings confirm that mutations in the NALP3/CIAS1/PYPAF1 gene are associated with FCU and MWS, and that disease severity and clinical features may differ substantially within and between families. Analysis of this gene will improve classification of patients with inherited or apparently sporadic periodic fever syndromes
   Internet : M.F.McDermott@qmul.ac.uk

2. AKIKUSA JD, ALLEN RC: Reducing the impact of rheumatic diseases in childhood. Best Pract.Res.Clin.Rheumatol. 2002, 16:333-345.
   Organism:Rheumatology and General Practice, Royal Children's Hospital, Flemington Rd, Parkville, Melbourne 3052, Australia
   Abstract: Reducing the impact of rheumatic diseases in childhood is the fundamental objective of every member of the multi-disciplinary team involved in the care of affected children and families. The means by which this objective may be achieved are broad and include the implementation of a range of non-pharmacological therapies to address the effects of rheumatic diseases on the physical development of the child. In addition, the treating team must be aware of the psychosocial impact that these diseases may have and the ways in which this may be minimized. This chapter is devoted to an examination of some of the non-pharmacological issues that arise in the management of the commonest rheumatic disease found in children, juvenile idiopathic arthritis (JIA). Aspects of physical rehabilitation, schooling, medication compliance, pain management and family dynamics are discussed, as are interventions to reduce the impact of this disease and its sequelae, utilizing, where possible, evidence-based principles from the literature. Although specific issues applicable to children with arthritis will be discussed, the broad principles of much of what follows applies to all of the rheumatic diseases in childhood
   Internet : PM:12387803

3. AL MATAR MJ, PETTY RE, TUCKER LB, MALLESON PN, SCHROEDER ML, CABRAL DA: The early pattern of joint involvement predicts disease progression in children with oligoarticular (pauciarticular) juvenile rheumatoid arthritis. Arthritis Rheum. 2002, 46:2708-2715.
   Organism:University of British Columbia, Vancouver, British Columbia, Canada
   Abstract: OBJECTIVE: To evaluate features during the first 6 months of disease that may be associated with a poor outcome as measured principally by extension to a polyarticular disease course in patients with oligoarticular-onset juvenile rheumatoid arthritis (oligo-JRA). METHODS: This study was a retrospective review of patients who fulfilled the American College of Rheumatology criteria for oligo-JRA, were followed up for at least 5 years, and did not have juvenile psoriatic arthritis, spondylarthropathy-like disease, or rheumatoid factor positivity. Data from the first 6 months of disease were collected. Continuous variables were dichotomized and then screened by univariate analysis for association with poor outcome at the last followup visit, as measured by extension of involvement (>4 accumulated involved joints) and by "clinically meaningful" extension (> or =10 accumulated joints). Variables significantly associated with this latter outcome, with the addition of disease duration as a confounding independent variable, were included in a multiple logistic regression analysis. The same variables were then examined in separate multiple logistic regression models to look at other measures of outcome, including use of disease-modifying antirheumatic drugs (DMARDs) at any time, erosive disease on radiographs, any remission of disease ever occurring, physician's global assessment of disease activity at the last visit, and disability as measured by the Childhood Health Assessment Questionnaire (C-HAQ)/HAQ. RESULTS: Of the 205 patients (160 of whom were female) studied for a median of 10.8 years (range 5-26.6 years), 39.5% developed extension to >4 joints and 17.6% developed arthritis in > or =10 joints. Using the logistic regression model, symmetric disease was predictive of all measures of poor outcome: extension to > or =10 joints (odds ratio [OR] 19.2), the need to use DMARDs (OR 11.5), radiographic demonstration of erosive disease (OR 4.73), inflammatory activity at last followup visit (OR 3.23), no remission of disease (OR 4.73), and disability as measured by a C-HAQ score >0.12 (OR 2.95). Ankle and/or wrist disease was predictive of extension (OR 6.61) and erosions (OR 3.59). Wrist disease alone was predictive of the need to use DMARDs (OR 5.87) and of inflammatory disease activity at the last followup visit (OR 4.01). An elevated erythrocyte sedimentation rate (ESR) was predictive of extension (OR 3.76), the need to use DMARDs (OR 6.47), and no remission of disease (OR 2.30). Disease duration was a confounding variable for extension (OR 1.18) and erosive disease (OR 1.19). CONCLUSION: The early presence of ankle and/or wrist disease, symmetric joint involvement, and an elevated ESR in a child with oligo-JRA indicates the likelihood of disease progression
   Internet : PM:12384930

4. ALDERMAN AK, CHUNG KC, DEMONNER S, SPILSON SV, HAYWARD RA: The rheumatoid hand: a predictable disease with unpredictable surgical practice patterns. Arthritis Rheum. 2002, 47:537-542.
   Organism:The University of Michigan and the University of Michigan Medical Center, Ann Arbor, Michigan 48109, USA
   Abstract: OBJECTIVES: To evaluate variation in fusion, arthroplasty, and tenosynovectomy rates among rheumatoid arthritis (RA) patients across states; to evaluate associations between surgery rates and the density of hand surgeons; and to evaluate differences in treatment by sex of the patient. METHODS: Data were obtained from the 1996 and 1997 Healthcare Cost and Utilization Project database. The procedure codes for fusion, arthroplasty, and tenosynovectomy were matched to patients with the diagnostic code of RA, which provided the total number of procedures performed in each state. The smoothed estimates of the RA population for each state were derived from age/sex strata in the 1995 US census using age/sex-adjusted RA prevalence data from the Third National Health and Nutrition Examination Survey. The number of hand surgeons was from the 1996 American Society for Surgery of the Hand. RESULTS: Procedure rates across states varied from 9-fold to 12-fold for all 3 procedures. The rates of the reconstructive procedures-fusion and arthroplasty-were highly correlated in each state, but these 2 procedures were only moderately correlated with tenosynovectomy. Surgeon density and procedure rates were minimally correlated. Procedure rates differed by patient sex, with significantly more arthroplasty and fusion procedures performed in women. More tenosynovectomy procedures were performed in men, and they were also performed at a younger age in men. CONCLUSIONS: Significant large area variations are present in the surgical management of the rheumatoid hand, but the correlations between reconstructive and early intervention procedures are modest. These rate differences are not explained by the number of hand surgeons, disease prevalence, or demographic composition of the states. However, men are more likely to receive more aggressive early surgical interventions, and women are more likely to receive end-stage reconstructive surgery
   Internet : PM:12382304

5. AUSAYAKHUN S, LOUTHRENOO W, AUPAPONG S: Ocular diseases in patients with rheumatic diseases. J.Med.Assoc.Thai. 2002, 85:855-862.
   Organism:Department of Ophthalmology, Faculty of Medicine, Chiang Mai University, Thailand
   Abstract: To study the distribution of ocular involvement among persons with rheumatic disease, a cross-sectional survey was performed in 224 patients attending the Division of Rheumatology, Department of Medicine, Maharaj Nakorn Chiang Mai Hospital. Of these patients, 102 presented with rheumatoid arthritis, 74 systemic lupus erythematosus, 39 systemic sclerosis, 6 mixed connective tissue disease, 2 polymyositis and 1 juvenile rheumatoid arthritis. It was found that the ocular involvement probably related to diseases including dry eye (19.9%) and uveitis (0.4%). The ocular involvement was presumably related to treatment including retinopathy (7.6%), cataract (6.3%), and glaucoma (0.9%). Rapid recognition of these complications would lead to early and appropriate management, which would prevent their sequelae
   Internet : PM:12403205

6. BARASH J, DUSHNITZKY D, STHOEGER D, BARDENSTEIN R, BARAK Y: Human parvovirus B19 infection in children: uncommon clinical presentations. Isr.Med.Assoc.J. 2002, 4:763-765.
   Organism:Division of Pediatrics, Kaplan Medical Center, Rehovot, Israel barashj@internetil
   Abstract: BACKGROUND: Human parvovirus B19 is responsible for a variety of clinical syndromes, such as erythema infectiosum, non-immune hydrops fetalis, transient aplastic anemia, and arthropathies. HPV is also suspected of playing a role in the pathogenesis of various chronic inflammatory and autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, Kawasaki disease and multiple sclerosis. OBJECTIVES: To study the age distribution and clinical presentation of patients hospitalized for human parvovirus B19 infection. METHOD: We reviewed the case records of all pediatric patients with serologic evidence of HPV infection who were admitted during a 20 month period to a major community hospital. RESULTS: Of 128 children tested for HPV, 48 had evidence of acute infection based on the presence of immunoglobulin M antibodies; 8 patients who also had positive IgM for other viruses were excluded, thus 40 case records were studied. The mean age of the patients was 5.21 years, but 22 patients were under 4. The clinical presentations included 25 patients with fever, either recurrent or prolonged, accompanied in some by enlarged spleen, liver and lymph nodes, skin rash and arthropathy; the remaining patients were investigated for anemia, skin rash, joint complaints and hepatitis. In addition, HPV infection was documented in several well-defined clinical conditions, such as SLE, vasculitic skin lesions, acute lymphoblastic leukemia, pure red cell aplasia, and optic neuritis. CONCLUSIONS: In a group of 40 pediatric patients exhibiting anti-HPV IgM antibodies, a younger age and less common clinical presentations were observed, furthermore 5 patients had clinical syndromes in which the causative role of HPV infection was not clear
   Internet : PM:12389336

7. BOUAYED K, ALYANAKIAN MA, CORDONNIER C, CAILLAT-ZUCMAN S, TESTELIN S, LAMBREY G, PRIEUR AM: Sjogren syndrome: an unexpected disease occurring fourteen years after oligoarticular onset juvenile idiopathic arthritis. Rheumatology (Oxford) 2002, 41:1190-1191.
   Internet : PM:12364642

8. BRITTON CA, MOORE A: Views from the inside, Part 2: What the children with arthritis said, and the experiences of siblings, mothers, fathers and grandparents. British Journal of Occupational Therapy 2002, 65:413-419.
   Organism:Dr. C.A. Britton, PO Box 58, Hove BN3 5WN
   Abstract: This second article in a series of three presents some of the findings from a study involving 46 families of children with juvenile idiopathic arthritis (JIA). The aim of the study overall was to describe and explore what life was like for families that included a child with JIA in the United Kingdom today. The study deployed a grounded theory approach, collecting different sorts of data over time in order to build up a picture of how these families lived with such a changeable long-term condition. In the first stage, data were collected using a self-completion questionnaire from 46 families, whose children attended a regular outpatient appointment at the paediatric rheumatology clinic. These data formed the contextual landscape to further in-depth data collected in stage two, from a cohesive group of nine families of girls selected from those who had already taken part in stage one. In stage two, the families were interviewed in their own homes, the children wrote diaries and the families recorded a video diary. All the data related to the respondents' recollections of events and their thoughts, feelings and perspectives: they formed the insiders' view of the experiences. This article presents the findings about the common themes that emerged from the data about children with JIA and their siblings, mothers, fathers and grandparents. It also discusses the key elements of the families' experiences and concludes with recommendations for health care practitioners. A distillation of the views of the majority of parents is provided in these two statements by different mothers: 'Immobility and pain makes the joy of play, just armchair viewing' and 'It's a stolen childhood, really, isn't it?'
   Internet : info@kidswitharthritis.org

9. CHOY EH, HAZLEMAN B, SMITH M, MOSS K, LISI L, SCOTT DG, PATEL J, SOPWITH M, ISENBERG DA: Efficacy of a novel PEGylated humanized anti-TNF fragment (CDP870) in patients with rheumatoid arthritis: a phase II double-blinded, randomized, dose-escalating trial. Rheumatology (Oxford) 2002, 41:1133-1137.
   Organism:Academic Department of Rheumatology, GKT School of Medicine, King's College London, King's College Hospital, London, UK
   Abstract: OBJECTIVE: Biological products that neutralize tumour necrosis factor alpha (TNF-alpha) are beneficial in rheumatoid arthritis (RA). We studied the effects of CDP870, a novel anti-TNF-alpha antibody fragment modified to obtain a prolonged plasma half-life ( approximately 14 days). METHODS: Thirty-six patients were randomized in a double-blind, ascending-dose group study to a single intravenous infusion of placebo (n = 12) or 1, 5 or 20 mg/kg CDP870 (each n = 8). The patients were predominantly female (30/36), had a mean age of 56 yr and a mean duration of RA of 13 years. They had received a mean of five DMARDs or experimental therapies (with 1 month washout before the study started) and had active disease. Continuation of NSAIDs and up to 7.5 mg prednisolone daily was allowed. Following the blinded dosing period, 32 patients received a single open-label infusion of either 5 or 20 mg/kg CDP870. RESULTS: In the blinded dosing period, 6/12 placebo patients withdrew from the study (for deteriorating RA < or =4 weeks after dosing). Two of 24 CDP870-treated patients withdrew, both in the 1 mg/kg group (for deteriorating RA or lost to follow up >4 weeks after dosing). The proportion of patients with ACR20 improvement for the per-protocol population with the last observation carried forward was 16.7, 50, 87.5 and 62.5% after 0, 1, 5 and 20 mg/kg CDP870 respectively (combined treatment effect, P = 0.012, primary analysis) at 4 weeks and 16.7, 25, 75 and 75% (P = 0.032) at 8 weeks. The proportion of patients with ACR50 improvement for the per-protocol population with the last observation carried forward was 0, 12.5, 12.5 and 50% after 0, 1, 5 and 20 mg/kg CDP870 respectively (combined treatment effect, P = 0.079) at 4 weeks and 0, 12.5, 12.5 and 50% (P = 0.079) at 8 weeks. Following the open-label dose of CDP870, similar beneficial effects were achieved. CONCLUSION: CDP870 is effective, was very well tolerated in this small study, and has an extended duration of action following one or more intravenous doses
   Internet : PM:12364632

10. CUSTERS JW, VAN DER NET JV, HOIJTINK H, WASSENBERG-SEVERIJNEN JE, VERMEER A, HELDERS PJ: Discriminative validity of the Dutch pediatric evaluation of disability inventory. Archives Of Physical Medicine And Rehabilitation 2002, 83:1437-1441.
    Organism:Dr. J.W. Custers, Dept. of Pediatric Physical Therapy, University Medical Centre, Children's Hospital, PO Box 85090, 3508 AB Utrecht
    Abstract: Objective: To examine the discriminative validity of the Dutch Pediatric Evaluation of Disability Inventory (PEDI) to differentiate functional status between children with and without disabilities. Design: Cross-sectional study. Setting: A university children's hospital in the Netherlands. Participants: A clinical sample comprising 197 children with disabilities (infantile encephalopathy, n=40; juvenile idiopathic arthritis, n=20; neurometabolic conditions, n=36; neuromuscular disorders, n=9; skeletal disorders, n=28; spina bifida, n=41; traumatic injury, n=23), and 62 children without disabilities. Interventions: Not applicable. Main Outcome Measure: Functional status was measured by using a Dutch version of the PEDI. Results: Discriminant analysis established the sensitivity and specificity of the PEDI. Correct predictions of group membership (disabled vs nondisabled) were found in both children without disabilities (93.5% correctly predicted) and children with disabling conditions (91.6% correctly predicted). Conclusion: The discriminative validity of the Dutch PEDI between children with and without disabilities was excellent. (c) 2002 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation
    Internet : j.custers@wkz.azu.nl

11. DE BENEDETTI F, MEAZZA C, MARTINI A: Role of interleukin-6 in growth failure: an animal model. Horm.Res. 2002, 58 Suppl 1:24-27.
    Organism:Pediatria Generale e Reumatologia, IRCCS Policlinico San Matteo, Pavia, Italia fdebenedetti@smatteopvit
    Abstract: Indirect evidence suggests a link between factors produced during the inflammatory response and stunted growth. The demonstration of this link was provided by the observation that mice transgenic for the inflammatory cytokine interleukin-6 (IL-6), expressing high circulating levels of IL-6 since birth, show a marked decrease in growth rate leading to adult mice 50-70% the size of wild-type littermates. The growth defect is completely abolished by neutralization of IL-6. In these mice the production of GH is normal, while circulating levels of IGF-I are markedly decreased. Administration of IL-6 to wild-type mice results in a marked decrease in IGF-I levels. These observations show that in vivo high levels of IL-6 are associated with low levels of IGF-I. However, IL-6 does not directly affect IGF-I production both in vitro and in vivo. In contrast, markedly decreased levels of IGFBP-3 are present in the IL-6 transgenic mice and administration of IL-6 to wild-type mice results in a marked decrease in IGFBP-3 levels. In these mice the decrease in IGFBP-3 levels is associated with impaired formation of the 150 kD ternary complex, even in the presence of normally functional ALS. As a consequence, IL-6 transgenic mice show increased clearance of circulating IGF-I, suggesting that IL-6 decreases IGF-I levels by increased clearance. Proteolytic degradation of IGFBP-3 occurs in the IL-6 transgenic mice, suggesting that the decrease in IGFBP-3 could be at least in part due to proteolysis. The abnormalities of the IGF-I system observed in the IL-6 transgenic mice are similar to those found in patients with systemic juvenile idiopathic arthritis, one of the chronic inflammatory diseases characterized by stunted growth and prominent production of IL-6. The IL-6 transgenic mice represent a faithful animal model of the growth impairment associated with chronic inflammation and may therefore provide information relevant to the understanding and treatment of this complication of inflammatory diseases
    Internet : PM:12373010

12. DONN R, ALOURFI Z, DE BENEDETTI F, MEAZZA C, ZEGGINI E, LUNT M, STEVENS A, SHELLEY E, LAMB R, ABINUN M, BECKER M, BELL A, CRAFT A, CRAWLEY E, DAVID J, FOSTER H, GARDENER-MEDWIN J, GRIFFIN J, HALL A, HALL M, HERRICK A, HOLLINGWORTH P, HOLT L, JONES S, POUNTAIN G, RYDER C, SOUTHWOOD T, STEWART I, VENNING H, WEDDERBURN L, WOO P, WYATT S, OLLIER WER, THOMSON W, RAY D: Mutation screening of the macrophage migration inhibitory factor gene: Positive association of a functional polymorphism of macrophage migration inhibitory factor with juvenile idiopathic arthritis. Arthritis And Rheumatism 2002, 46:2402-2409.
    Organism:Dr. R. Donn, Epidemiology Unit, Arthritis Research Campaign, University of Manchester, Oxford Road, Manchester M13 9PT
    Abstract: Objective. To determine if polymorphisms of the macrophage migration inhibitory factor (MIF) gene are associated with juvenile idiopathic arthritis (JIA). Methods. Denaturing high-performance liquid chromatography was used to screen the MIF gene in 32 UK Caucasian controls and 88 UK Caucasian JIA patients. Ninety-two healthy UK Caucasian controls were then genotyped for each of the polymorphic positions identified. A panel of 526 UK Caucasian JIA patients and 259 UK Caucasian controls were subsequently genotyped for a single-nucleotide polymorphism (SNP) identified in the 5prime-flanking region of the gene, using SNaPshot ddNTP primer extension and capillary electrophoresis. The functional significance of this polymorphism was also studied using luciferase-based reporter gene assays in human T lymphoblast and epithelial cell lines. Results. A tetranucleotide repeat CATTSUB(5-7) beginning at nucleotide position -794 and 3 SNPs at positions -173 (G to C), +254 (T to C), and +656 (C to G) of the MIF gene were identified. No JIA-specific mutations were found. Allele and genotype frequencies differed significantly between the controls and the JIA patients for the MIF-173 polymorphism. Individuals possessing a MIF-173*C allele had an increased risk of JIA (34.8% versus 21.6%) (odds ratio 1.9, 95% confidence interval 1.4-2.7; P = 0.0002). Furthermore, the MIF-173* G and C variants resulted in altered expression of MIF in a cell type-specific manner. Serum levels of MIF were also significantly higher in individuals who carried a MIF-173*C allele (P = 0.04). Conclusion. The -173-MIF*C allele confers increased risk of susceptibility to JIA. Our data suggest a cell type-specific regulation of MIF, which may be central to understanding its role in inflammation
    Internet : Rachelle@fs1.ser.man.ac.uk

13. FRENKEL J, KUIS W: Overt and occult rheumatic diseases: the child with chronic fever. Best Pract.Res.Clin.Rheumatol. 2002, 16:443-469.
    Organism:Department of General Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Suite KE041331, PO Box 85090, The Netherlands
    Abstract: Identification of the genes involved in hereditary periodic fever syndromes has led to the recognition of a new pathophysiological category, the autoinflammatory disorders. The main non-hereditary autoinflammatory disease in childhood is systemic juvenile idiopathic arthritis (sJIA), others being the chronic infantile neurological cutaneous arthropathy (CINCA) syndrome and the periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome. Familial Mediterranean fever (FMF) has been traced to mutations in the MEFV gene. Mutations in the MVK gene, encoding the enzyme mevalonate kinase, cause the hyper-IgD periodic fever syndrome (HIDS). The tumour necrosis factor(TNF)-receptor-associated periodic syndromes (TRAPS) have been linked to mutations in theTNFRSF1A gene, encoding a TNF-alpha receptor, and the CIAS1 gene is mutated in familial cold autoinflammatory syndrome. We discuss how this knowledge has influenced diagnosis and treatment of these rare genetic disorders and how it might change our approach to the more common rheumatic diseases
    Internet : PM:12387810

14. GANSER G: Physical therapy in juvenile chronic arthritis and connective tissue diseases in childhood. Aktuelle Rheumatologie 2002, 27:213-220.
    Organism:Dr. G. Ganser, St. Josef Stift Sendenhorst, Abt. Kinder- und Jugendrheumatologie, Postfach 1164/1165, 48318 Sendenhorst
    Abstract: Physical and occupational therapy programmes are vital to the management of rheumatic diseases in children. As early as possible these regimens should be initiated together with medical treatment of acutely inflamed joints. These therapies aim at decreasing of inflammation, pain, limitation of motion and disturbances of growth. A variety of modalities and treatment approaches are used in paediatric rheumatology units in Germany. The physical therapies are depending on the kind of the rheumatic disease, age of the patient, experience of the physical or occupational therapist in the treatment of children with chronic inflammatory diseases of the joints. Therapeutic exercises and occupational therapy are mentioned in the literature [1,2]. The physical therapies are mainly cold therapies in acute inflammatory diseases of the joints and the spine to reduce pain and the limitation of movement, heat therapies (e.g. in warm water of about 32degreesC) for chronic disease to reduce muscle pain, stiffness, to perform exercises in the water (easier weight bearing). The different modalities of electrotherapy are indicated in case of chronic pain, inflammation of the tendons (enthesopathy), as well as in muscular pain due to contractures or limitation of activities in daily living. Phonophoresis is used in enthesopathies as well. Massage of the muscles or of the connective tissue is also used to influence swelling, edema or limitation of motion according to muscle pain and to improve circulation in the lymphatic system. As an example for the treatment with physical therapies the therapeutic aims of the different methods are shown according to the symptoms of connective tissue diseases in childhood
    Internet : ganser@st-josef-stift.de

15. GONC EN, OZON A, ALIKASIFOGLU A, KANDEMIR N: Type 1 diabetes mellitus in a 3 1/2 year-old girl with Turner's syndrome. J.Pediatr.Endocrinol.Metab 2002, 15:1203-1206.
    Organism:Pediatric Endocrinology Unit, Hacettepe University Faculty of Medicine, Ankara, Turkey
    Abstract: Turner's syndrome is associated with autoimmune disorders. Autoimmune endocrinopathy in Turner's syndrome seems to be limited to autoimmune thyroiditis. A small number of patients with Turner's syndrome has also been associated with celiac disease, inflammatory bowel disease and juvenile rheumatoid arthritis. Type 1 diabetes mellitus in Turner's syndrome has been rarely reported. We present here the youngest patient with Turner's syndrome who developed type 1 diabetes mellitus. At the age of 3.5 years she was hospitalized with diabetic ketoacidosis. Anti-islet cell and anti-insulin antibodies were positive and C-peptide level was low. When she was investigated for recurrent urinary tract infections, horseshoe kidney was detected by ultrasonography. Karyotype analysis revealed 45,XO. She has been followed for 2 years with an insulin dose of 0.9 U/kg per day. The prevalence of type 1 diabetes mellitus associated with Turner's syndrome is still unknown
    Internet : PM:12387520

16. GOWING EC, MCKOWN KM: Myasthenia gravis in a patient with pauciarticular juvenile chronic arthritis. Journal of Clinical Rheumatology 2002, 8:269-272.
    Organism:Dr. E.C. Gowing, Department of Rheumatology, Univ. of WI Hospital and Clinics, 600 Highland Ave., Madison, WI 53792
    Abstract: There have been reported cases of children with histories of pauciarticular juvenile chronic arthritis (JCA) later developing myasthenia gravis (MG) as young adults. This is intriguing because it had been considered rare to diagnose a second autoimmune disease in a patient with pauciarticular JCA, unlike in those with adult-onset rheumatoid arthritis. We report a case of MG in a 20-year-old woman with a history of pauciarticular JCA. She presented with bilateral ptosis, weakness, and a history of dysphagia. The diagnosis was confirmed with positive serum acetylcholine receptor antibodies (2000 nm/L) and electromyography showing a decremental response to repetitive muscle stimulation. The patient's inflammatory arthritis was quiescent at diagnosis. The patient underwent a surgical thymectomy and was treated with pyridostigmine, intravenous immunoglobulin, and corticosteroids with a fluctuating clinical course. Previous cases have been reported of MG associated with this subtype of JCA, suggesting a connection in autoimmune pathology. The earlier recognition and management of MG in a patient with pauciarticular JCA presenting with weakness may improve the prognosis of this disease

17. IMAIZUMI S, MORITA T, KOBAYASHI H, ITO T, HIRATA Y: Synovial sarcoma with extended occult period treated as juvenile rheumatoid arthritis: a case report. J.Orthop.Sci. 2002, 7:570-573.
    Organism:Department of Orthopedic Surgery, Niigata Cancer Center Hospital, 2-15-3 Kawagishi-cho, Niigata 951-8566, Japan
    Abstract: Synovial sarcoma is a clinically high-grade malignant soft-tissue tumor; however, it demonstrates slow growth on occasion. A 2-year-old girl presented with persistent pain of the left forearm and was diagnosed with juvenile rheumatoid arthritis (JRA) by a pediatrician. Almost 10 years after the initial complaint, at the age of 11 years, a tumor mass appeared in her left forearm near the wrist joint, and the pain became more severe. Magnetic resonance imaging (MRI) showed a sarcomatous lesion, circumscribed by extensor tendons. A biopsy specimen revealed synovial sarcoma. The tumor was resected widely. Reconstruction was effected via free vascularized fibula grafting. The patient is well 13 months after surgery, with neither recurrence nor distant metastases
    Internet : PM:12355132

18. KOIVUSALO A, LINDAHL H, RINTALA RJ: Morbidity and quality of life in adult patients with a congenital abdominal wall defect: a questionnaire survey. J.Pediatr.Surg. 2002, 37:1594-1601.
    Organism:Hospital for Children and Adolescents, Helsinki University, Helsinki, Finland
    Abstract: BACKGROUND: In children with congenital abdominal wall defects (CAWD), surgical treatment of the abdominal defect and the associated anomalies cause considerable morbidity in the first years of life. Afterward, most of the CAWD patients with correctable anomalies develop as other children. The morbidity and quality of life (QoL) of CAWD patients who have reached their adulthood is less well known and the subject of this study. METHODS: A 3-part questionnaire was sent to 75 former patients with CAWD, aged 17 years or more. The first part included questions about health, symptoms, and education; the second part consisted of 3 tests of psychosocial functioning; and the third part was a SF-36 questionnaire measuring the QoL. RESULTS: Of the 75 patients, 57 (76%) answered: (25 males, 32 females); omphalocele (n = 16) gastroschisis (n = 11); median age, 27 (range, 17 to 48) years. With the exception of rheumatoid arthritis (in 7% of patients), the prevalence of acquired diseases in CAWD patients was comparable with that of the general population; 50 of 57 (88%) considered their health good. The most frequent causes of morbidity were disorders in the abdominal scar in 21 (37%) patients, and functional gastrointestinal disorders in 29 (51%) of patients. Low self-esteem was found in 12% of patients, but the QoL and educational level of CAWD patients were not different from that of the general population. CONCLUSIONS: In CAWD patients the morbidity from acquired disorders is similar to morbidity in the general population. Disorders with the abdominal scar and various functional gastrointestinal disorders are common, but they rarely cause serious problems. The majority of CAWD patients have a quality of life not different from the general population
    Internet : PM:12407546

19. KURTEV A, MIREVA N: Autoimmune thyroiditis in children with juvenile chronic arthritis and systemic lupus erythematosus. Endocrinologia 2001, 6:21-26.
    Organism:Dr. A. Kurtev, Clin. Pediatric Endocrinol./Diabetes, University Pediatric Hospital, 11 Akad. Ivan Geshov Str., 1612 Sofia
    Abstract: The underlying causes of the frequent association of some systemic connective tissue diseases, such as juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE), with autoimmune thyroiditis (AT) in children are still not well enough clarified. This is a report on 29 children with JCA aged 12,81 +/- 3,4 yrs, range 5, 7 to 19, and three children aged 10,4-17,64 yrs presenting SLE with different evolution, undergoing appropriate treatment - non-steroid antiinflammatory drugs (NSAID) plus glucocorticoids and/or cytostatics. As shown by the results there is moderate thyroid hyperplasia (mainly I B degree) in half of the JCA children, and in three with SLE, subclinical hypothyroidism in 17,9 % of JGA cases; increased antithyroid antibodies - TAT in 24,1 %, MAT in 54,4% of JCA, and in 2/5 children with SLE. AT is diagnosed in 41,4 % of the children with JCA (nearly half of them presenting atrophic version) and in two of the children with SLE. Among JCA children no changes in OGTT and in cell mediated and humoral immunity indicators are documented. In all children with JCA and SLE periodical testing of the thyroid gland - size, function and antibody formation - is strongly recommended with a special reference to the ever increasing association of the diseases with AT
    Internet : alkurtev@yahoo.com

20. KYNGAS H: Motivation as a crucial predictor of good compliance in adolescents with rheumatoid arthritis. Int.J.Nurs.Pract. 2002, 8:336-341.
    Organism:Department of Nursing and Health Administration, University of Oulu, University Hospital, Oulu, Finland helvikyngas@oulufi
    Abstract: The purpose of this study was to identify the predictors of compliance shown by adolescents with juvenile rheumatoid arthritis (JRA). Three hundred individuals aged 13-17 years were randomly selected from the Finnish Social Insurance Institution's register. The questionnaires were sent to the adolescents selected from the register to be returned directly to the researcher in a self-addressed envelope. Ninety-one per cent (n = 274) returned the questionnaire. Logistic regression was used to find the factors that predicted compliance with health regimens. The compliance of adolescents with JRA was predicted on the basis of motivation, fear of acute problems, support from nurses, energy and willpower, and threat to social well-being. The most powerful predictor was motivation. The likelihood of the adolescents who had good motivation to comply with health regimens was 29.13-fold compared to the adolescents who did not have good motivation. The next powerful predictor was fear of acute problems. The adolescents who felt fear of acute problems complied with health regimens with a 20.35-fold likelihood compared to the adolescents who did not have fears. The third powerful predictor was support from nurses. The likelihood of adolescents supported by nurse to comply with health regimens was 17.03-fold compared to the adolescents who did not receive support from nurses. The likelihood of adolescents who had energy and willpower to comply with health regimens was 7.56-fold compared to the adolescents who did not have energy and willpower. Also, the threat to social well-being predicted good compliance
    Internet : PM:12390587

21. LAUNAY F, JOUVE JL, GUILLAUME JM, VIEHWEGER E, JACQUEMIER M, BOLLINI G: [Total hip arthroplasty without cement in children and adolescents: 17 cases]. Rev.Chir Orthop.Reparatrice Appar.Mot. 2002, 88:460-466.
    Organism:Service de Chirurgie Infantile et Orthopedie, Hopital d'Enfants de la Timone, 264, rue Saint-Pierre, 13385 Marseille Cedex 05
    Abstract: PURPOSE OF THE STUDY: Hip disease can produce major pain and functional disorders in children who should benefit from total hip arthroplasty. We report our experience with total hip prostheses implanted without cement in children.MATERIAL AND METHODS: We performed 17 total hip arthroplasties in 13 children who had various conditions, mainly chronic juvenile osteoarthritis and aseptic osteonecrosis secondary to sickle cell anemia. The acetabular inserts were not cemented. All the femoral stems were custom-made using computer-assisted preoperative planning based on standard x-rays and computed tomography findings. Outcome was assessed on the basis of patient satisfaction, pain, and function. Radiographically, we assessed stem implantation, stability and integration. Results were classed with the Harris score and also with the Steinbrocker classification in order to take into account the child's overall functional handicap.RESULTS: Mean follow-up was 36.4 months. There were three cases of superficial hematoma, one case of superficial sepsis and one acetabular loosening. All patients were satisfied. Unsatisfactory function, observed in 80% of the children preoperatively, was found in only 17% postoperatively. The Harris score improved from 23.8 preoperatively to 87.7 at last follow-up. There were no cases of stem loosening and integration was achieved in 85.3% of the cases proximally in the area with hydroxyapatite surfacing.DISCUSSION: The problem with these children is to determine when total hip arthroplasty should be proposed. We retain three important indications: uncontrollable chronic pain, normal school attendance impossible, no other possibility for conservative surgery. Several studies have reported only mediocre results with cemented stems. We opted for custom-made stems without cement for three reasons: preservation of bone stock, better adaptation to bone whose quality and morphology had been remodeled by the underlying condition and repeated osteotomies which also affect the gluteus medius, and finally, better chance of success for future revisions in these active young patients.CONCLUSION: The question on whether or not total hip arthroplasty should be performed early in these children to avoid osteotomies which could alter the longevity of a future prosthesis remains open. Custom-made stems inserted without cement have provided satisfactory results in our experience. To date, follow-up is too short to compare our results with those obtained by others using cemented stems
    Internet : PM:12399710

22. LEE JH, SLIFMAN NR, GERSHON SK, EDWARDS ET, SCHWIETERMAN WD, SIEGEL JN, WISE RP, BROWN SL, UDALL JN, Jr., BRAUN MM: Life-threatening histoplasmosis complicating immunotherapy with tumor necrosis factor alpha antagonists infliximab and etanercept. Arthritis Rheum. 2002, 46:2565-2570.
    Organism:Office of Biostatistics and Epidemiology, Center for Biologics Evaluation and Research, FDA, 1401 Rockville Pike, Rockville, MD 20852-1448, USA
    Abstract: OBJECTIVE: Two tumor necrosis factor alpha (TNFalpha) antagonists were recently licensed in the US. Infliximab was licensed in 1998 for the treatment of Crohn's disease (CD), and since 1999, it has been licensed in combination with methotrexate for treatment of rheumatoid arthritis (RA). Etanercept was licensed in 1998 for treatment of RA and, more recently, for juvenile RA and psoriatic arthritis. Because of potential immunosuppression related to use of anti-TNFalpha agents, we sought to identify postlicensure cases of opportunistic infection, including histoplasmosis, in patients treated with these products. METHODS: The US Food and Drug Administration's (FDA) passive surveillance database for monitoring postlicensure adverse events was reviewed to identify all reports received through July 2001 of histoplasmosis in patients treated with either infliximab or etanercept. RESULTS: Ten cases of Histoplasma capsulatum (HC) infection were reported: 9 associated with infliximab and 1 associated with etanercept. In patients treated with infliximab, manifestations of histoplasmosis occurred within 1 week to 6 months after the first dose and typically included fever, malaise, cough, dyspnea, and interstitial pneumonitis. Of the 10 patients with histoplasmosis, 9 required treatment in an intensive care unit, and 1 died. All patients had received concomitant immunosuppressive medications in addition to infliximab or etanercept, and all resided in HC-endemic regions. CONCLUSION: Postlicensure surveillance suggests that acute life-threatening histoplasmosis may complicate immunotherapy with TNFalpha antagonists, particularly infliximab. Histoplasmosis should be considered early in the evaluation of patients who reside in HC-endemic areas in whom infectious complications develop during treatment with infliximab or etanercept
    Internet : PM:12384912

23. LOFTHOUSE CM, AZAD F, BAILDAM EM, AKOBENG AK: Measuring the nutritional status of children with juvenile idiopathic arthritis using the bioelectrical impedance method. Rheumatology (Oxford) 2002, 41:1172-1177.
    Organism:Department of Gastroenterology, Booth Hall Children's Hospital, Charlestown Road, Blackley M9 7AA, UK
    Abstract: OBJECTIVE: To assess the nutritional status of children with juvenile idiopathic arthritis (JIA) using anthropometric measurements and bioelectrical impedance. METHODS: Twenty-two consecutive JIA patients (seven pauciarticular, 15 polyarticular) attending the rheumatology clinic at Booth Hall Children's Hospital were compared with 22 age- and sex-matched controls attending the accident and emergency department of the same hospital. There were no patients with systemic-onset JIA in the cohort. Height, weight, head circumference and skinfold thickness at four sites (biceps, triceps, subscapular and suprailiac) were measured. Regression equations were used to calculate body fat as a percentage of weight, and arm muscle circumference. In addition, bioelectrical impedance measurements were made using a Holtain body composition analyser. These measurements were then used to calculate the total body water, which could be used as an indirect estimate of the lean body mass. RESULTS: Of the JIA patients, 22.7% were below the third centile for height, 18.1% had a weight less than the third centile. Mid-arm circumference was below the fifth centile in 36.4% of the patients. Patients with polyarticular disease showed significantly more signs of malnutrition than patients with pauciarticular disease. In the polyarticular group, comparison with controls revealed significant P values for reduction in height (0.047), weight (0.045), mid-arm circumference (0.002), arm muscle circumference (0.012), percentage body fat (0.008) and total body water (0.031). CONCLUSIONS: In view of the findings of lower total body water, indicating lower lean mass, in more nutritionally deprived JIA patients (as deduced by the other physical parameters measured), we conclude that bioelectrical impedance is a useful adjunct to anthropometric measures in assessing nutritional status in JIA
    Internet : PM:12364639

24. MAENO N, TAKEI S, NOMURA Y, IMANAKA H, HOKONOHARA M, MIYATA K, YAMAMURA M, KAWASHIMA M, MAKINO H: Highly elevated serum levels of interleukin-18 in systemic juvenile idiopathic arthritis but not in other juvenile idiopathic arthritis subtypes or in Kawasaki disease: Comment on the article by Kawashima et al [2] (multiple letters). Arthritis And Rheumatism 2002, 46:2539-2542.
    Organism:Dr. N. Maeno, Kagoshima University, Kagoshima

25. MANZI S, WASKO MC: Getting to the heart of the matter in systemic lupus and rheumatoid arthritis. Bull.Rheum.Dis. 2001, 50:1-4.
    Organism:Department of Medicine, Department of Epidemiology, Graduate School of Public Health, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
    Internet : PM:12387096

26. MARTIN V, LEE LA, ASKANASE AD, KATHOLI M, BUYON JP: Long-term followup of children with neonatal lupus and their unaffected siblings. Arthritis And Rheumatism 2002, 46:2377-2383.
    Organism:Dr. J.P. Buyon, Department of Rheumatology, Hospital for Joint Diseases, 301 East 17th Street, New York, NY 10003
    Abstract: Objective. To determine in a longitudinal cohort study whether children with varied manifestations of neonatal lupus or their unaffected siblings later develop autoantibodies and/or rheumatic diseases. Methods. To obtain information on the health of children ages >=8 years who had manifestations of neonatal lupus (affected group) and their unaffected siblings (unaffected group), questionnaires were sent to mothers (with anti-SSA/Ro and/or anti-SSB/La antibodies) who were enrolled in the National Institute of Arthritis and Musculoskeletal and Skin Diseases/Hospital for Joint Diseases Research Registry for Neonatal Lupus. Children of healthy mothers referred by the Registry enrollees comprised the control group. Further data were provided by review of medical records. Results. Fifty-five mothers enrolled in the Registry returned questionnaires on 49 children with neonatal lupus and their 45 unaffected siblings. Six children with definite rheumatic/autoimmune diseases were identified: 2 with juvenile rheumatoid arthritis, 1 with Hashimoto thyroiditis, 1 with psoriasis and iritis, 1 with diabetes mellitus and psoriasis, and 1 with congenital hypothyroidism and nephrotic syndrome. All had neonatal lupus, and their mothers had manifestations of autoimmune diseases (Sjogren's syndrome in 4, systemic lupus erythematosus/Sjogren's syndrome in 1, and undifferentiated autoimmune disease in 1). Antinuclear antibodies were present in 4 of 55 sera tested (2 of 33 affected children and 2 of 22 unaffected children). No serum contained antibodies reactive with SSA/Ro or SSB/La antigens. Conclusion. These data suggest that children with neonatal lupus require continued followup, especially prior to adolescence and if the mother herself has an autoimmune disease. While there was no apparent increased risk of systemic lupus erythematosus, the development of some form of autoimmune disease (systemic or organ-specific) in early childhood may be of concern. During adolescence and young adulthood, individuals with neonatal lupus and their unaffected siblings do not appear to have an increased risk of developing systemic rheumatic diseases
    Internet : jill.buyon@med.nyu.edu

27. MASSA M, MAZZOLI F, PIGNATTI P, DE BENEDETTI F, PASSALIA M, VIOLA S, SAMODAL R, LA CAVA A, GIANNONI F, OLLIER W, MARTINI A, ALBANI S: Proinflammatory responses to self HLA epitopes are triggered by molecular mimicry to Epstein-Barr virus proteins in oligoarticular juvenile idiopathic arthritis. Arthritis Rheum. 2002, 46:2721-2729.
    Organism:IRCCS Policlinico San Matteo, Pavia, Italy
    Abstract: OBJECTIVE: To evaluate whether abnormal T cell recognition may be generated by exposure to exogenous antigens presenting sequence homology with epitopes contained in self HLA alleles, and if such recognition may be part of the mechanisms that fuel inflammation in autoimmune diseases associated with certain HLA alleles. METHODS: Cytotoxic responses of peripheral blood mononuclear cells to 9-mer peptides derived from HLA molecules (DRB1*1101, DRB1*0801, or DPB1*0201) associated with oligoarticular juvenile idiopathic arthritis (JIA) or homologous peptides derived from Epstein-Barr virus (EBV) proteins (Bolf1 or Balf2) were analyzed in patients with oligoarticular JIA and in healthy controls matched for HLA-DRB1*1101, DRB1*0801, or DPB1*0201. Production of proinflammatory cytokines in culture supernatants was determined by enzyme-linked immunosorbent assay. RESULTS: T cell cytotoxic responses and production of proinflammatory cytokines in response to stimulation with self HLA-derived peptides were found only in patients with oligoarticular JIA, and not in controls. Patients with oligoarticular JIA, but none of the healthy controls, had EBV-self HLA cross-reactive T cells. CONCLUSION: Our data suggest a dis
    Internet : PM:12384932

28. MCCURDY DK, TAI L-Q, IMFELD KL, SCHWARTZ M, ZALDIVAR F, BERMAN MA: Expression of melanoma antigen gene by cells from inflamed joints in juvenile rheumatoid arthritis. Rinsho Ganka 2002, 29:2219-2224.
    Organism:Dr. D.K. McCurdy, Division of Rheumatology, Children's Hospital of Orange County, 455 South Main Street, Orange, CA 92613-5700
    Abstract: Objective. To determine if synovial fluid (SF) cells from inflamed joints of patients with juvenile rheumatoid arthritis (JRA) express melanoma antigen gene (MAGE) RNA and protein. Methods. The pattern of MAGE-A1 expression was analyzed in inflammatory synovial tissue and peripheral blood mononuclear cells (PBMC) from patients with JRA by immunocytochemistry, reverse transcription-polymerase chain reaction (RT-PCR), and flow cytometry. Results. MAGE-A1, previously detected only in tumor cells, is strongly expressed in SF cells from patients with JRA. Immunocytochemistry revealed strong staining of SF cells in all of 22 specimens tested. PBMC from patients (7 of 7) also expressed MAGE-A1, but not as strongly as SF cells. Two-color immunofluorescence showed colocalization with CD4 and CD14. Flow cytometry on 3 samples of SF cells confirmed the presence of MAGE-A1 on the cell surface and intracellularly. Five of 5 SF cell samples were positive for MAGE-A1 by RT-PCR. Conclusion. Mononuclear cells from inflamed joints and blood from patients with JRA express MAGE-A1. MAGE family proteins were previously thought to be expressed only by certain tumors and presented by HLA Class I, resulting in tumor cell lysis by cytotoxic T cells. The observation of MAGE-A1 expression in JRA suggests an association with autoimmune disease and a possible causal role for MAGE antigen in the chronic inflammation of JRA
    Internet : dmccurdy@choc.org

29. MILLER ML: Use of imaging in the differential diagnosis of rheumatic diseases in children. Rheumatic Disease Clinics of North America 2002, 28:483-492.
    Organism:Division of Immunology and Rheumatology, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL, 60614 USA^E-Mail: millermd@northwestern.edu

30. MINDEN K, NIEWERTH M, LISTING J, BIEDERMANN T, BOLLOW M, SCHONTUBE M, ZINK A: Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis And Rheumatism 2002, 46:2392-2401.
    Organism:Dr. K. Minden, Deutsches Rheuma-Forsch. Ztr. Berlin, Schumannstrasse 21/22, 10117 Berlin
    Abstract: Objective. To describe the long-term outcome of juvenile idiopathic arthritis (JIA). Methods. All patients with JIA referred to a pediatric rheumatology center between 1978 and 1988 were identified and invited to undergo an assessment. Patients with JIA from a population-based cohort from East Berlin were included. The outcome assessment considered changes in body function and structure (e.g., mortality, joint abnormalities, disease activity), activities at the individual level (Health Assessment Questionnaire), and participation in society (e.g., mobility, educational and vocational background). Results. Of 260 eligible patients, 215 (83%) were evaluated. Subtypes of JIA at disease onset included oligoarthritis (40%), polyarthritis (14%), systemic arthritis (14%), psoriatic arthritis (1%), enthesitis-related arthritis (15%), and other arthritis (16%). Followup was conducted after a median of 16.5 years. No deaths occurred in this cohort. At followup, approximately half of the patients had active disease and/or changes in body structures to a variable extent. Approximately one-third of patients rated themselves as being functionally limited. Patients demonstrated good social integration: few mobility problems were reported, and the educational achievements of patients were higher and their rate of unemployment was lower compared with the age-matched population. No significant differences in outcome were found between the population-based and the referral-based cohorts. Conclusion. Even though approximately half of the JIA patients had more or less distinctive changes in body function and/or structure after a disease duration of < 15 years, fewer than 10% were severely disabled or handicapped. Because JIA often persists into adulthood, long-term followup and care are necessary
    Internet : minden@drfz.de

31. MORHART R: Juvenile systemic lupus erythematosus - A case report. Aktuelle Rheumatologie 2002, 27:198-200.
    Organism:Dr. R. Morhart, Klin. fur Kinder/Jugendrheumatologie, Gehfeldstr. 24, 82467 Garmisch-Partenkirchen
    Abstract: We present the case of a now 20-year old young woman suffering from juvenile systemic lupus erythematosus (JSLE) which began 7 years ago. The course of the disease was complicated by a macrophage activation syndrome (MAS), a reactive haemophagocytic lymphohistiocytosis. The MAS is a rare, but due to the high mortality, important complication of rheumatic disorders in childhood, especially the systemic onset of juvenile chronic arthritis and SLE. On treating the MAS with high dose steroid pulse therapy the patient developed an acute pancreatitis. This is a well known rare complication in SLE but also a possible adverse effect of the steroid pulse therapy. With adequate therapy we were fortunately able to control the disease effectively. The patient is under continual treatment and is now feeling very well and able to work full-time

32. MORIMOTO Y, TANAKA T, YOSHIOKA I, MASUMI S, YAMASHITA M, OHBA T: Virtual endoscopic view of salivary gland ducts using MR sialography data from three dimension fast asymmetric spin-echo (3D-FASE) sequences: a preliminary study. Oral Dis. 2002, 8:268-274.
    Organism:Department of Dental Radiology, Kyushu Dental College, Kitakyushu, Japan rad-mori@mailkyu-dentacjp
    Abstract: OBJECTIVES: We performed magnetic resonance (MR) sialography of parotid gland and/or submandibular gland ducts using three-dimensional fast asymmetric spin-echo (3D-FASE) sequencing. The objective was to make three-dimensional (3D) reconstruction images and virtual endoscopic views of the parotid gland ducts using MR sialography data sets of 3D-FASE sequences. METHODS: We reviewed the MR sialography data sets with 3D-FASE sequencing of 10 control volunteers and six patients. Three-dimensional reconstruction images and virtual endoscopic views of the parotid gland and/or submandibular gland ducts were generated with maximum intensity projection (MIP), shaded surface display (SSD), and volume rendering techniques (VRT). RESULTS: The main parotid gland and/or submandibular gland ducts, large branches within the glands, and small branches were fairly well defined in a very short acquisition time on MR sialographic images with 3D-FASE sequencing in nine of the 10 healthy volunteers. The 3D-reconstruction images of the parotid gland ducts and/or submandibular gland ducts showed the entire length of the branch paths and complete images from all angles, and the virtual endoscopic views showed the endoluminal tracts of the main ducts and the large branches in nine. In the patient with Sjogren's syndrome, chronic sialoadenitis, and salivary calculi in the Wharton ducts, the MR sialographic images showed diffuse areas of punctate high signal intensity, dilatation of Stensen's duct, or stones of Wharton's duct, respectively. Furthermore, the 3D-reconstruction images of the salivary gland ducts showed the stenoses and stones in the branch paths and complete images from all angles, and the virtual endoscopic views showed the stenoses and stones in the endoluminal tracts of the main and large branches. CONCLUSIONS: Our initial experience showed that virtual MR endoscopy could be performed to observe the endoluminal tracts of parotid and submandibular glands. The clinical use of the virtual MR endoscopy for salivary gland ducts has not been established yet. Future applications of the 3D-reconstruction images and virtual endoscopic views using MR sialography data sets of 3D-FASE sequences are very attractive and further expansion of this field is expected
    Internet : PM:12363112

33. MURRAY KJ, LOVELL DJ: Advanced therapy for juvenile arthritis. Best Pract.Res.Clin.Rheumatol. 2002, 16:361-378.
    Organism:Paediatric Rheumatology Unit, Great Ormond St Hospital for Children, London WC1N 3JH, UK
    Abstract: The management of juvenile idiopathic arthritis (JIA) has undergone dramatic changes in the last decade with undoubtedly great benefit for many patients. In particular, more effective use of available drugs and the application of newly discovered drugs have been responsible for much of this improvement. Methotrexate is the gold standard for management of moderate to severe polyarthritis. Other disease-modifying antirheumatic drugs (DMARDs) such as sulphasalazine and cyclosporine are finding a specific role for resistant disease where they may be used in combination with methotrexate, for example. The introduction of anti-TNF agents, such as etanercept, is likely to herald a major shift to the use of biological agents in those intolerant to, or unresponsive to, standard DMARD therapy. DMARDs provide major steroid spring effect in many children with severe JIA with the hope that osteoporosis and growth failure will be reduced. More judicious use of corticosteroids and techniques such as intravenous 'pulse therapy' rather than long-term high-dose use of oral corticosteroids are also major changes. Intra-articular corticosteroids are commonly used in children with oligoarticular JIA and as a useful adjunct to DMARD therapy in children with other forms of JIA. Autologous stem cell transplantation is an exciting new development currently restricted to use in patients with very severe, resistant disease. Modifications of technique, experience and increased safety may make this a more widely applicable technique, in particular for patients with a poor prognosis, such as those with systemic JIA. Although the focus of this chapter is on drug therapy, multidisciplinary team management for children with chronic arthritis focusing on the physical, nutritional, intellectual and psychosocial wellbeing of the child will continue to be important
    Internet : PM:12387805

34. NAZLI GE, OZON A, ALIKASIFOGLU A, KANDEMIR N: Type 1 diabetes mellitus in a 3 1/2 year-old girl with Turner's syndrome. Journal of Pediatric Endocrinology and Metabolism 2002, 15:1203-1206.
    Organism:E. Nazil Gonc, Hacettepe University, Faculty of Medicine, Pediatric Endocrinology Unit, 06100 Sihhiye
    Abstract: Turner's syndrome is associated with autoimmune disorders. Autoimmune endocrinopathy in Turner's syndrome seems to be limited to autoimmune thyroiditis. A small number of patients with Turner's syndrome has also been associated with celiac disease, inflammatory bowel disease and juvenile rheumatoid arthritis. Type 1 diabetes mellitus in Turner's syndrome has been rarely reported. We present here the youngest patient with Turner's syndrome who developed type 1 diabetes mellitus. At the age of 3.5 years she was hospitalized with diabetic ketoacidosis. Anti-islet cell and anti-insulin antibodies were positive and C-peptide level was low. When she was investigated for recurrent urinary tract infections, horseshoe kidney was detected by ultrasonography. Karyotype analysis revealed 45,XO. She has been followed for 2 years with an insulin dose of 0.9 U/kg per day. The prevalence of type 1 diabetes mellitus associated with Turner's syndrome is still unknown

35. OPENSHAW H: Conference synopsis: Hematopoietic stem cell therapy in autoimmune diseases, October 2001. Biology of Blood and Marrow Transplantation 2002, 8:407-411.
    Organism:Dr. H. Openshaw, City Hope National Medical Center, 1500 Duarte Road, Duarte, CA 91010
    Abstract: Since 1996, patients with autoimmune diseases have been treated on single-arm investigational protocols with high-dose immunosuppressive therapy and autologous peripheral blood stem cell transplantation (HSCT). In a conference held in October 2001 at the City of Hope National Medical Center, participants discussed current laboratory studies in autoimmunity, the rationale of HSCT in autoimmune diseases, results of phase I-II studies, and the prospects for controlled trials. This conference synopsis summarizes major discussion points in clinical sessions and in sessions devoted to ethical and regulatory aspects of this investigational treatment. Protocols for controlled studies in multiple sclerosis (MS) and systemic sclerosis (SSc), originating in Europe and in the United States, have been designed or are in the final stages of design. The only controlled trial presently underway is for SSc in Europe (Autologous Stem Cell Transplantation International Scleroderma Trial [ASTIS]). There are current plans for a controlled trial for rheumatoid arthritis (RA) in Europe (ASTIRA) but not in the United States. Eventual cross-study analysis of the European and United States trials may give valuable comparative information on the different mobilization and immunosuppressive regimens used. Recognition of the importance of axonal degeneration in secondary progressive MS and the use of mitoxantrone as a rescue medication are two relatively recent developments now being considered in the design of controlled HSCT protocols in MS. The importance of informed consent and study accessibility was discussed as well as the continuing role of the US Food and Drug Administration in regulating these protocols in the United States
    Internet : hopenshaw@coh.org

36. PATEL SJ, LUNDY DC: Ocular manifestations of autoimmune disease. American Family Physician 2002, 66:991-998.
    Organism:S.J. Patel, Naval Medical Center, San Diego, CA
    Abstract: Rheumatoid arthritis, juvenile rheumatoid arthritis, Sjogren's syndrome, the seronegative spondyloarthropathies, systemic lupus erythematosus, multiple sclerosis, giant cell arteritis, and Graves' disease are autoimmune disorders commonly encountered by family physicians. These autoimmune disorders can have devastating systemic and ocular effects. Ocular symptoms may include dry or red eyes, foreign-body sensation, pruritus, photophobia, pain, visual changes, and even complete loss of vision. Because a number of these diseases may initially present with ocular symptoms, physicians should maintain a high index of suspicion to make a timely diagnosis. A thorough ophthalmic examination, including visual acuity, pupillary reaction, ocular motility, confrontation field testing, external inspection, and direct ophthalmoscopy with fluorescein staining, should be completed. In the patient with the complaint of a "dry eye" or a "red eye," simple tools such as the Schirmer's test or the blanching effect of phenylephrine can be useful in diagnosis. In general, managing the systemic effects with nonsteroidal anti-inflammatory drugs, corticosteroids, and immunosuppressive agents controls the ocular symptoms. When visual function is threatened, surgical therapy may be necessary. Early and accurate diagnosis with prompt treatment or referral to an ophthalmologist may prevent systemic and ocular disabilities. Copyright (c) 2002 American Academy of Family Physicians

37. RABINOVICH CE: Bone metabolism in childhood rheumatic disease. Rheumatic Disease Clinics of North America 2002, 28:655-667.
    Organism:Division of Pediatric Rheumatology, Department of Pediatrics, Duke University Medical Center, Box 3212, Durham, NC, 27710 USA^E-Mail: rabin001@mc.duke.edu

38. RIDDER GJ, FRADIS M, BERNER R, LOHLE E: PFAPA syndrome: Current standard of knowledge and relevance for the ENT specialist. Laryngo- Rhino- Otologie 2002, 81:635-639.
    Organism:Dr. G.J. Ridder, Univ.klin. fur H-, N-/O./Poliklinik, Universitatsklinikum Freiburg, Killianstrasse 5, 79106 Freiburg
    Abstract: Background: The symptoms fever, aphthous stomatitis, pharyngitis and cervical lymph node enlargement are among the most frequent complaints that particularly make children and adolescents consult an ENT specialist. In cases where these symptoms occur periodically, a PFAPA syndrome may be present. Patients and Methods: We report on an atypical process in a 22 year old woman in whom the PFAPA syndrome first occurred during early infancy with recurrences since adolescence. Results: Since the age of eight months the patient suffered from periodically occurring fever and pharyngitis with cervical lymph node enlargement. Therefore, by age four a tonsillectomy was performed and the symptoms disappeared. At age 15 similar symptoms occurred again with fever thrusts over approximately four to five days with temperatures up to 39.8 degreesC associated with pharyngitis, weakness and cervical lymphadenitis. Those symptoms relapsed periodically every four weeks. Treatment regimens with different antibiotics, Dimepranol and Inosin did not show any effect. Hyper-IgD syndrome and cyclic neutropenia were excluded. There were no signs for a humoral or cellular immune defect or a juvenile idiopathic arthritis. Conclusions: It is often very difficult to make the right diagnosis in a child or a young adolescent presenting with periodically occurring fever. So far the PFAPA syndrome is relatively unknown. Nevertheless, we think that it should be considered in the differential diagnosis by the ENT specialist patients with periodic fever, aphthous stomatitis, pharyngitis and cervical lymphadenopathy
    Internet : ridder@hno.ukl.uni-freiburg.de

39. SCHILLING F, FEDLMEIER M, ECKARDT A, KESSLER S: [Vertebral manifestation of chronic recurrent multifocal osteomyelitis (CRMO)]. Rofo Fortschr.Geb.Rontgenstr.Neuen Bildgeb.Verfahr. 2002, 174:1236-1242.
    Organism:Klinik und Poliklinik fur Radiologie, Johannes Gutenberg-Universitat Mainz
    Abstract: Chronic recurrent multifocal osteomyelitis (CRMO) is a systemic osteo-articular disease that is characterized by a sterile, primarily chronic osteomyelitis with various distribution patterns of the individual lesions. In this article, we describe the "axial type" with predominant involvement of the spine, which represents 13 of our 41 CRMO cases of different age groups. The important element of its diagnosis is the typical lympho-plasmacellular spondylitis that can be detected and staged by scintigraphy, MRI and conventional radiography. Potentially affected are all vertebrae from the mid-cervical spine to the sacrum. One or several segments can be involved, sometimes as transient inflammatory edema, sometimes as "migratory spondylitis" or "saltatory spondylitis", but also as chronic sclerosing type with early radiographically detectable manifestation. Vertebral deformity due to compression and total collapse (vertebra plana) are rare. A complicated course with patulous perivertebral edema can lead to concomitant symptomatic inflammatory changes in adjacent regions and organs. In the course of CRMO, spondylodiscitis only develops as secondary destruction following the spondylitis. This can help to differentiate spondyloarthropathies from CRMO that is initially detected as primary lesion in the spine. While CRMO generally has a good prognosis, its radiological differentiation from rheumatologic conditions plays an important role
    Internet : PM:12375195

40. SCHNEIDER R, PASSO MH: Juvenile rheumatoid arthritis. Rheumatic Disease Clinics of North America 2002, 28:503-530.
    Organism:Dr. R. Schneider, Division of Rheumatology, Department of Pediatrics, Hospital for Sick Children, 555 University Avenue, Toronto, Ont. M5G 1X8
    Abstract: Progress in achieving international consensus concerning the classification of juvenile idiopathic arthritis has been made, although further refinement and validation of these criteria is needed. It is hoped that this will facilitate more effective international collaboration in the study of these diseases, because much remains to be learned about genetic susceptibility, causation, pathogenesis, and treatment. Attention to the unique aspects of chronic arthritis in children such as impaired growth and macrophage activation syndrome may help to reduce disease-related morbidity and mortality. New biologic agents have substantially enhanced the treatment of JRA. The identification of reliable predictors of disease course and outcome is important in the rational and timely application of new therapies

41. SIMON D, LUCIDARME N, PRIEUR AM, RUIZ JC, CZERNICHOW P: Treatment of growth failure in juvenile chronic arthritis. Horm.Res. 2002, 58 Suppl 1:28-32.
    Organism:Service d'Endocrinologie et de Diabetologie Pediatriques, Hopital Robert Debre, Paris, France dominiquesimon@rdbap-hop-parisfr
    Abstract: We retrospectively assessed linear growth and final height in a group of 24 patients suffering from juvenile idiopathic arthritis (JIA) during childhood, receiving steroid therapy. In these patients, a significant loss of height (-2.7 +/- 1.5 SDS) occurred in the first years of the disease which was positively correlated with prednisone therapy duration. After remission of the disease and prednisone discontinuation, most of the patients (70%) had catch-up growth but 30% had a persistent loss of height. Their mean final height was strongly correlated with their mean height at the end of steroid therapy and was significantly different between the group of patients with catch-up growth (-1.5 +/- 1.6 SDS) and the group without catch-up growth (-3.6 +/- 1.2 SDS). This pattern of growth observed in JIA patients should help us to define strategies of GH treatment in these patients in order to improve their final height. We have previously reported the beneficial effects on growth and body composition of a 1-year GH treatment in a group of 14 growth-retarded patients suffering from juvenile idiopathic arthritis, receiving glucocorticoid therapy. These patients (n = 13) were treated again with GH at the same dosage (0.46 mg/kg/week) for another 3-year period. GH treatment markedly increased growth velocity in these patients, but had a minor effect on SDS height suggesting that these children will remain short at adult age. Using GH earlier in these patients during the course of their disease may prevent growth deterioration and metabolic complications induced by chronic inflammation and long-term steroid therapy
    Internet : PM:12373011

42. STONE JH: Polyarteritis nodosa. JAMA (Journal of the American Medical Association) 2002, 288:1632-1639.
    Organism:Johns Hopkins Vasculitis Center, 5501 Hopkins Bayview Circle, JHAAC 1B.23, Baltimore, MD, 21224 USA^E-Mail: jstone@jhmi.edu
    Abstract: Polyarteritis nodosa (PAN) is regarded rightly as the grandfather of the vasculitides. In this Grand Rounds, the case of a 30-year-old man with a 12-year illness is described. The patient presented with daily fevers, tachycardia, and cutaneous ulcers on his distal extremities. He eventually developed mononeuritis multiplex. Because of the striking pattern of his fevers, he was diagnosed for many years as having adult-onset Still disease. Following the addition of daily cyclophosphamide to his long-standing regimen of prednisone, the patient's disease entered remission for the first time in more than a decade. He was ultimately able to discontinue all of his immunosuppressive medications. The case is discussed in the context of the first patient ever described with PAN, the classic report of Kussmaul and Maier

43. TAKAHI K, HASHIMOTO J, HAYASHIDA K, SHI K, TAKANO H, TSUBOI H, MATSUI Y, NAKASE T, TOMITA T, OCHI T, YOSHIKAWA H: Early closure of growth plate causes poor growth of long bones in collagen-induced arthritis rats. Journal of Musculoskeletal Neuronal Interactions 2002, 2:344-351.
    Organism:Dr. J. Hashimoto, Department of Orthopaedic Surgery, Osaka University, Graduate School of Medicine, 2-2 Yamada-oka, Suita, Osaka 565-0871
    Abstract: Abnormalities of the epiphyseal growth plate that occur in collagen-induced arthritis (CIA) were studied. CIA was induced in 6-week-old Lewis rats by immunization with type II collagen. Radiographic examination revealed the early closure of the epiphyseal growth plate with growth retardation of the femur and tibia. Histological evaluation confirmed the early closure of the epiphyseal growth plate accompanied by decreased intensity of safranin-O staining indicating decreased amounts of proteoglycans in the extracellular matrix (ECM) of the cartilage. Immunohistochemical methods showed that the number of chondrocytes expressing matrix metalloproteinase (MMP)-3 and/or vascular endothelial growth factor (VEGF) increased in the growth plates of CIA rats. This study confirmed that disturbances of long bone growth with early closure of the epiphyseal growth plates occur in CIA. There appeared to be overexpression of MMP-3, which may be involved with proteoglycan degradation. Additionally, VEGF, which is associated with cartilage ossification and angiogenesis, might also play a role in this event. Further clarification of the mechanism of the growth disturbance in CIA may yield clinical benefits, especially in prevention of the premature closure of growth plate that is seen in juvenile rheumatoid arthritis and other diseases
    Internet : junha@ort.med.osaka-u.ac.jp

44. THOMSON W, BARRETT JH, DONN R, PEPPER L, KENNEDY LJ, OLLIER WE, SILMAN AJ, WOO P, SOUTHWOOD T: Juvenile idiopathic arthritis classified by the ILAR criteria: HLA associations in UK patients. Rheumatology (Oxford) 2002, 41:1183-1189.
    Organism:Arthritis Research Campaign Epidemiology Unit, School of Epidemiology and Health Sciences, University of Manchester, Stopford Building, Oxford Road, Manchester M13 9PT, UK
    Abstract: OBJECTIVE: To determine the HLA associations with juvenile idiopathic arthritis (JIA) and its subgroups as defined by the International League of Associations for Rheumatology (ILAR) classification criteria. METHODS: Five hundred and twenty-one UK Caucasian JIA patients and 537 UK Caucasian controls were typed for HLA class II alleles. Phenotype and haplotype frequencies were compared between all JIA cases and controls and between the seven ILAR-defined JIA subgroups. RESULTS: Three haplotypes (DRB1*08-DQA1*0401-DQB1*0402; DRB1*11-DQA1*05-DQB1*03; DRB1*1301-DQA1*01-DQB1*06) were associated with increased risk and one (DRB1*04-DQA1*03-DQB1*03) with decreased risk of JIA. However, in each case the frequencies also varied between JIA subgroups. CONCLUSION: This study categorically demonstrates that there are multiple HLA class II associations with JIA. It has also, for the first time, defined these associations in the seven different ILAR subgroups in UK JIA cases. Although there are a number of common associations, each ILAR subgroup exhibits different patterns of HLA associations, suggesting that the ILAR classification system does define genetically distinct groups of patients
    Internet : PM:12364641

45. TREMLETT HL, EVANS J, WILES CM, LUSCOMBE DK: Asthma and multiple sclerosis: an inverse association in a case-control general practice population. QJM. 2002, 95:753-756.
    Organism:Department of Medicine (Neurology), University of Wales College of Medicine, Cardiff, Wales, UK tremlett@interchangeubcca
    Abstract: BACKGROUND: Th1/Th2 imbalance is hypothesized to up-regulate some diseases and down-regulate others. Compared to controls, multiple sclerosis (MS) (Th1-mediated) has been linked to a reduced risk of allergy and asthma (Th2-mediated), based on patient questionnaire studies and a review of asthma medication. AIM: To investigate whether MS is associated with a reduced risk of Th2-associated diseases and an increased risk of Th1-associated diseases. DESIGN: Retrospective matched case-control study. METHODS: Three hundred and twenty MS patients and controls matched for age, gender, location and smoking were selected from the Welsh General Practice Morbidity Database from 1995-99. Case and control records were assessed for Th1-mediated and Th2-mediated diseases. RESULTS: Overall, 346 MS patients were identified, giving a prevalence of 127 per 100 000. There was an inverse relationship between multiple sclerosis (MS) and asthma (OR 0.33; 95%CI 0.15-0.77). No statistically significant relationships emerged between other Th2-associated (eczema, dermatitis) or any Th1-associated (rheumatoid arthritis, thyroid disorders, inflammatory bowel disease [IBD], type 1 diabetes) diseases and MS, although no patient in either group had treated type 1 diabetes. A trend existed for IBD, with 5/320 of cases affected and no controls; OR infinity; 95%CI 1.30-infinity; p=0.063. DISCUSSION: This inverse association between MS and asthma is compatible with a Th1/Th2 imbalance. Although the Th1/Th2 theory is probably an over-simplification in MS, a shift from Th1 cytokine dominance towards Th2 may provide drug-targeting routes for MS
    Internet : PM:12391388

46. VARBANOVA B, KOLAROV ZI: Clinical aspects of anticardiolipin antibodies in juvenile chronic (idiopathic) arthritis and rheumatoid arthritis. Rheumatology 2002, 10:40-43.
    Organism:Dr. B. Varbanova, Department of Pediatrics, Medical University, 55, Marin Drinov Str., Bg - 9002 Varna
    Abstract: Anticardiolipin antibodies (aCL) have been considered to be a risk factor for development of antiphosphlipid/anticardiolipin syndrome, which is characterized by deep thromboses, thromboembolic complications, hemolytic anemia and thrombocytopenia. Although aCL are specified mainly for SLE patients, they have been found in some other conditions such as autoimmune disorders, ischemic coronary and cerebrovascular diseases, infectious diseases, etc. with unclear clinical significance. IgG- and IgM- aCL were measured by ELISA in 71 children with juvenile chronic/idiopathic arthritis (JCA/JIA) and in 105 patients with RA and compared with age corresponding control groups. The results showed significantly elevated levels and prevalence of both isotypes in JCA/JIA with no clinical correlations, On the contrary, serum samples from RA patients were found to have slightly increased aCL concentrations with no significant difference as compared to the control groups but with clinical correlation with seropositivity, the symptoms of joint inflammation and severe erosive arthritis. Antiphospholipid/anticardiolipin syndrome wasn't found in any of the patients

47. VERVAEREN J: [A new treatment for rheumatoid arthritis]. J.Pharm.Belg. 2002, 57:82-88.
    Internet : PM:12357696

48. YANG YH, WANG SJ, CHUANG YH, LIN YT, CHIANG BL: The level of IgA antibodies to human umbilical vein endothelial cells can be enhanced by TNF-alpha treatment in children with Henoch-Schonlein purpura. Clin.Exp.Immunol. 2002, 130:352-357.
    Organism:Department of Paediatrics, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
    Abstract: Anti-endothelial cell antibodies (AECA) have been found to play an important role in many vascular disorders. In order to determine the presence of AECA in children with Henoch-Schonlein purpura (HSP), and to elucidate the pathogenic and clinical value of their measurement in this disease, AECA were detected by immunofluorescence staining and a human umbilical vein endothelial cell (HUVEC)-based enzyme-linked immunosorbent assay (ELISA) in 20 children with HSP, 10 children with juvenile rheumatoid arthritis (JRA) without vasculitis and 10 normal healthy children. Antibodies against another endothelial cells, human dermal microvascular endothelial cells (HMVEC-d) were also detected by cell-based ELISA. In some experiments, we compared the binding activity of antibodies to HUVEC with and without tumour necrosis factor-alpha (TNF-alpha) or interleukin-1 (IL-1) pretreatment. Patients with acute onset of HSP had higher serum levels of IgA antibodies, both against HUVEC and against HMVEC-d, than healthy controls (P = 0.001, P = 0.008, respectively). Forty-five per cent of patients had positive IgA AECA to HUVEC, and 35% had positive IgA AECA to HMVEC-d. The titres of IgA antibodies to HUVEC paralleled the disease activity. After TNF-alpha treatment, the values of IgA AECA to HUVEC in HSP patients were significantly increased (P = 0.02). For IgG and IgM AECA, there was no difference between HSP patients and controls (P = 0.51, P = 0.91). Ten JRA children without vasculitis had no detectable IgG, IgM or IgA AECA activity. The results of this study showed that children with HSP had IgA AECA, which were enhanced by TNF-alpha treatment. Although the role of these antibodies is not clear, IgA AECA provide another immunological clue for the understanding of HSP
    Internet : PM:12390327

49. ZEGGINI E, DONN RP, OLLIER WE, THOMSON W: Evidence for linkage of HLA loci in juvenile idiopathic oligoarthritis: independent effects of HLA-A and HLA-DRB1. Arthritis Rheum. 2002, 46:2716-2720.
    Organism:Arthritis Research Campaign Epidemiology Unit, University of Manchester, Stopford Building, Oxford Road, Manchester M13 9PT, UK eleftheria@fs1sermanacuk
    Abstract: OBJECTIVE: Although multiple associations between HLA loci and juvenile oligoarthritis have previously been documented, evidence for linkage of HLA loci in oligoarthritis in UK Caucasians with juvenile idiopathic arthritis (JIA) has not been described. The aim of this study was to investigate linkage of HLA-A, B, and DRB1 in UK Caucasian JIA patients with oligoarthritis. METHODS: One hundred forty-two families comprising affected offspring and their parents (45 with one parent available and 97 with both parents available) were typed for HLA-A, B, and DRB1 by polymerase chain reaction-sequence-specific oligonucleotide probe. Single-point and multipoint analyses were performed using various modifications of the extended transmission disequilibrium test. Linkage disequilibrium (LD) analysis was performed using the EH-Plus system. RESULTS: Linkage to HLA-A and HLA-DRB1 was seen in oligoarthritis and in the International League of Associations for Rheumatology-defined subgroups of persistent oligoarthritis and extended oligoarthritis. Linkage to HLA-A, B, and DRB1 was also observed in female patients with oligoarthritis. Linkage of the HLA loci seemed to be attributable to maternal preferential transmission of alleles. Furthermore, LD patterns between the HLA-A, B, and DRB1 loci were investigated. HLA-A and HLA-DRB1 were confirmed as independent susceptibility loci for oligoarthritis. CONCLUSION: This is the first study to establish linkage of HLA-A and HLA-DRB1 in oligoarthritis and to show that the 2 loci contribute independently to disease. Further studies are being performed to determine whether it is these loci or other genes in LD with them that are responsible for susceptibility to oligoarthritis in UK Caucasian patients with JIA
    Internet : PM:12384931