Bibliography August 2003
- Useful after methotrexate failure in inflammatory rheumatism. Prescrire International (France ) 12:66 127-132, 2003
Abstract: * There is no reference second-line treatment for patients with rheumatoid arthritis, juvenile chronic arthritis, psoriatic arthropathy or ankylosing spondylitis after failure or intolerance of a slow-acting antirheumatic drug such as methotrexate. * Etanercept, a immunosuppressant targeting TNF-alpha (like infliximab), is now approved in France for use in these situations, with the exception of spondylitis. * In the second-line treatment of adults with rheumatoid arthritis, the clinical evaluation dossier on etanercept contains data from dose-finding studies and two placebo-controlled trials involving patients in whom several single-agent treatments had failed. At a dose of 25 mg subcutaneously twice a week, etanercept worked partially in about half the patients. Without direct comparisons, the place of etanercept relative to other slow-acting antirheumatic drugs is difficult to establish. From indirect comparisons, etanercept seems a slightly better treatment option than infliximab. * In the first-line treatment of rheumatoid arthritis, one trial showed that etanercept worked faster than methotrexate, but there was no significant difference between the two treatments after two years. * Little is known about the efficacy of etanercept in patients with juvenile chronic arthritis who do not respond adequately to methotrexate. There are no comparative trials. One double-blind placebo-controlled trial showed that etanercept, when it worked, remained active for at least 7 months. * In one trial, etanercept was more effective than placebo in patients with psoriatic arthropathy and ankylosing spondylitis who continued to receive their usual treatment, which included a slow-acting antirheumatic drug in about 50% of cases. * More than 50% of patients treated with etanercept have a cutaneous reaction to the injection. These reactions are usually mild or moderate. * Active pharmacovigilance is needed, given its mechanism of action, and previous notifications of a wide variety of adverse effects (even though it is sometimes difficult to establish a foolproof link between etanercept and the adverse effect). Long-term studies of large numbers of patients are needed to determine the precise risk of side effects including haematological, infectious, neurological, oncological and immunological effects. * In practice, methotrexate remains the first-line treatment for inflammatory arthritis. Etanercept can be a useful second-line treatment, especially in juvenile chronic arthritis - Etanercept: new preparation. Useful after methotrexate failure in inflammatory rheumatism. Prescrire Int 12:66 127-132, 2003
Abstract: There is no reference second-line treatment for patients with rheumatoid arthritis, juvenile chronic arthritis, psoriatic arthropathy or ankylosing spondylitis after failure or intolerance of a slow-acting antirheumatic drug such as methotrexate. Etanercept, a immunosuppressant targeting TNF-alpha (like infliximab), is now approved in France for use in these situations, with the exception of spondylitis. In the second-line treatment of adults with rheumatoid arthritis, the clinical evaluation dossier on etanercept contains data from dose-finding studies and two placebo-controlled trials involving patients in whom several single-agent treatments had failed. At a dose of 25 mg subcutaneously twice a week, etanercept worked partially in about half the patients. Without direct comparisons, the place of etanercept relative to other slow-acting antirheumatic drugs is difficult to establish. From indirect comparisons, etanercept seems a slightly better treatment option than infliximab. In the first-line treatment of rheumatoid arthritis, one trial showed that etanercept worked faster than methotrexate, but there was no significant difference between the two treatments after two years. Little is known about the efficacy of etanercept in patients with juvenile chronic arthritis who do not respond adequately to methotrexate. There are no comparative trials. One double-blind placebo-controlled trial showed that etanercept, when it worked, remained active for at least 7 months. In one trial, etanercept was more effective than placebo in patients with psoriatic arthropathy and ankylosing spondylitis who continued to receive their usual treatment, which included a slow-acting antirheumatic drug in about 50% of cases. More than 50% of patients treated with etanercept have a cutaneous reaction to the injection. These reactions are usually mild or moderate. Active pharmacovigilance is needed, given its mechanism of action, and previous notifications of a wide variety of adverse effects (even though it is sometimes difficult to establish a foolproof link between etanercept and the adverse effect). Long-term studies of large numbers of patients are needed to determine the precise risk of side effects including haematological, infectious, neurological, oncological and immunological effects. In practice, methotrexate remains the first-line treatment for inflammatory arthritis. Etanercept can be a useful second-line treatment, especially in juvenile chronic arthritis - ALSAEID KM, HAIDER MZ, AL AWADHI AM, SRIVASTVA BS, AYOUB EM: Role of human leukocyte antigen DRB1*0307 and DRB1*0308 in susceptibility to juvenile rheumatoid arthritis. Clin Exp Rheumatol 21:3 399-402, 2003
Organism: Kuwait University, Faculty of Medicine, Pediatrics Department, PO Box 24923, Safat 13110, Kuwait khaled7691@hotmailcomFAU - Alsaeid, K M
Abstract: OBJECTIVE: To study the prevalence of Human Leukocyte Antigen (HLA) DR alleles in children with juvenile rheumatoid arthritis (JRA). METHODS: DNA samples from 64 children with oligoarticular and seronegative polyarticular JRA and 64 controls of the same ethnic background were analyzed using PCR-sequence specific primers (PCR-SSP) method. Analysis took into account the onset subtype, the presence of antinuclear antibodies (ANA) and the presence of chronic anterior uveitis, a recognised serious complication of JRA. RESULTS: A high prevalence of DR3 alleles were detected in children with oligoarticular JRA compared to controls (p < 0.05). DR3 alleles were the commonest also in patients with positive ANA as well as those with chronic anterior uveitis. The interesting finding in this study is the absence of two DR3 alleles, namely DRB1*0307 and DRB1 *0308 in the control group while present in significant proportion in children with JRA. DRB1*0307 was present in 16% of children with oligoarticular subtype and 15% of those with polyarticular JRA. DRB1*0308 was only detected in children with oligoarticular JRA, none of the children with polyarticular JRA or the controls had this allele. CONCLUSION: These findings support earlier observations linking these two DR3 alleles, namely 0307 and 0308, to the genetic susceptibility to JRA - BAUMAN AE, FARDY HJ, HARRIS PG: Getting it right: why bother with patient-centred care? Med J Aust 179:5 253-256, 2003
Organism: University of New South Wales, Sydney abauman@unsweduauFAU - Bauman, Adrian E
Abstract: Patient-centred care is about sharing the management of an illness between patient and doctor; it is not new but is increasingly evidence-based, especially for chronic problems such as diabetes, asthma and arthritis. Systematic reviews show that patient-centred care results in increased adherence to management protocols, reduced morbidity and improved quality of life for patients. Key features of the doctor-patient interaction are shared goal setting, written management plans and regular follow-up. Supportive community-based services and programs, combined with healthcare system commitment, are also required to make this approach effective in improving population health - BERESFORD BA, SLOPER P: Chronically ill adolescents' experiences of communicating with doctors: a qualitative study. J Adolesc Health 33:3 172-179, 2003
Organism: Social Policy Research Unit, University of York, Heslington, York, United Kingdom bab3@yorkacukFAU - Beresford, Bryony A
Abstract: PURPOSE: To explore the experiences of chronically ill adolescents in communicating with health professionals, including the identification of factors which hinder or facilitate their use of health professionals as an information source. METHODS: 63 adolescents (11-16 years) with a chronic condition (cystic fibrosis, diabetes, epilepsy, juvenile chronic arthritis, or Duchenne muscular dystrophy) were interviewed, and subsequently took part in two group discussion meetings with other adolescents of a similar age with the same condition. Data were analyzed using the framework method involving data reduction, data display, and conclusion-drawing and verification. RESULTS: Factors affecting the openness and effectiveness of communication included: duration and frequency of contact; gender; perceived attitudes towards adolescents; the communication skills of the adolescent and health professional; and the presence of parents and medical students/trainee doctors. The type of information needed also affected whether the adolescents felt able to discuss an issue with a health professional. Adolescents were reluctant to raise personal or sensitive issues or to ask questions that revealed poor adherence. A perceived lack of interest in the wider impacts of having a chronic condition on day-to-day life was also a barrier to adolescents discussing difficulties at school and socio-emotional problems. CONCLUSIONS: Communication and information provision are key roles for health professionals, yet this study highlights a population with potentially high levels of information need who are facing considerable barriers to addressing these needs. Steps taken to improve adolescent-health professional communication need to address a range of practical, attitudinal and behavioral factors - BLACK AP, BHAYANI H, RYDER CA, PUGH MT, GARDNER-MEDWIN JM, SOUTHWOOD TR: An association between the acute phase response and patterns of antigen induced T cell proliferation in juvenile idiopathic arthritis. Arthritis Res Ther 5:5 R277-R284, 2003
Organism: Department of Rheumatology, Division of Immunity and Infection, University of Birmingham, Birmingham, UKFAU - Black, Antony P B
Abstract: The aim of this research was to determine whether all memory T cells have the same propensity to migrate to the joint in patients with juvenile idiopathic arthritis. Paired synovial fluid and peripheral blood mononuclear cell proliferative responses to a panel of antigens were measured and the results correlated with a detailed set of laboratory and clinical data from 39 patients with juvenile idiopathic arthritis. Two distinct patterns of proliferative response were found in the majority of patients: a diverse pattern, in which synovial fluid responses were greater than peripheral blood responses for all antigens tested; and a restricted pattern, in which peripheral blood responses to some antigens were more vigorous than those in the synovial fluid compartment. The diverse pattern was generally found in patients with a high acute phase response, whereas patients without elevated acute phase proteins were more likely to demonstrate a restricted pattern. We propose that an association between the synovial fluid T cell repertoire and the acute phase response suggests that proinflammatory cytokines may influence recruitment of memory T cells to an inflammatory site, independent of their antigen specificity. Additionally, increased responses to enteric bacteria and the presence of alphaEbeta7 T cells in synovial fluid may reflect accumulation of gut associated T cells in the synovial compartment, even in the absence of an elevated acute phase response. This is the first report of an association between the acute phase response and the T cell population recruited to an inflammatory site - BRUNNER HI, MAKER D, GRUNDLAND B, YOUNG NL, BLANCHETTE V, STAIN AM, FELDMAN BM: Preference-based measurement of health-related quality of life (HRQL) in children with chronic musculoskeletal disorders (MSKDs). Med Decis Making 23:4 314-322, 2003
Organism: William Rowe Division of Rheumatology, Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USAFAU - Brunner, H I
Abstract: BACKGROUND: Health-related quality of life can be measured by patients' health preferences (utilities or values). No method for measuring health state preferences has been standardized for children with arthritis or other musculoskeletal disorders (MSKDs). Such a method is needed for economic evaluations of current and new pediatric treatments. OBJECTIVES: 1) To assess the feasibility of utility measurements in children with MSKDs, 2) to test the validity of the Health Utility Index (HUI) for these children, 3) to assess whether rating scale values can be mathematically converted into meaningful standard gamble (SG) utilities, and 4) to study whether parents can act as proxies for their children with respect to health state preferences. METHODS: Eighty parents of children with MSKDs were consecutively sampled. Their children, if 8 years of age or older (n = 55), were studied concurrently. Utilities of current health states were obtained by using the SG and the HUI in random order. In addition, health state preferences were assessed using categorical and analog rating scales. Traditional nonutility measures of health status (the Childhood Health Assessment Questionnaire [CHAQ] and the Activities Scale for Kids [ASK]) were also completed. Intraclass correlation coefficients (ICCs) were calculated to assess concordance between the different utility measures and also between the ratings of the parents and their children. RESULTS: Children 8 years of age or older were able to express the strength of their health state preferences using the HUI and rating scales. Children older than 10 years of age were able to use the SG method. The health state utilities of the parents were higher than those of their children. The utilities varied widely depending on the elicitation method. The expected high agreement between the SG and the HUI was not found (ICC = 0.028 for parents, ICC = 0.016 for patients). Unlike the SG, the global utilities derived from the HUI agreed better with preferences derived from rating scales (ICC = 0.23-0.25) and correlated with traditional health status measures (with CHAQ, r = -0.56; with ASK, r = 0.46) both for parents and children. It was not possible to mathematically convert rating scale preferences into SG utilities. The SG utilities were unrelated to results from the rating scales, the CHAQ, and the ASK. Especially for parents, the SG utilities were very high, even when ratings of the other measures indicated poor health. CONCLUSIONS: Although it is possible to measure health utilities for children with MSKDs, the results are highly method dependent. The properties of the HUI in this population are more like those of the traditional health status measures rather than those of the SG. Preferences derived from rating scales, although easily performed, cannot readily be converted into SG utilities. Parents' ratings for their children are impaired by risk aversion - CHOUNG Y-H, PARK K, MOON S-K, KIM C-H, RYU SJ: Various causes and clinical characteristics in vertigo in children with normal eardrums. International Journal of Pediatric Otorhinolaryngology (Ireland ) 67:8 889-894, 2003
Abstract: Objective: The differential diagnosis of vertigo in children is extensive. Otitis media and middle ear effusion could be the most common causes of vertigo in children, but there are some problems in detecting the other causes for vertigo because they are one of most frequent diseases of childhood. The purpose of this study is to review the clinical characteristics and both the audiological and vestibular findings of vertigo in children with normal eardrums, who do not show otitis media or middle ear effusion, and to assist in making a differential diagnosis of vertigo. Methods: The fifty five children (<16 years old) with vertigo, who visited the Department of Otolaryngology, Ajou University Hospital, Suwon, South Korea between January 1995 and December 2001 were selected for this study. These excluded the patients with abnormal eardrums/tympanograms or those that did not perform questionnaires, audiological, or vestibular evaluations. They were retrospectively analyzed for clinical symptoms, vestibular functions, and differential diagnosis. Results: The most common causes for vertigo in children were migraine in 17 (30.9%) and benign paroxysmal vertigo of childhood (BPVC) in 14 (25.5%). Other less frequent causes included four cases of trauma, two cases each of Meniere's disease, delayed endolymphatic hydrops, benign positional vertigo, and one case only for cerebellopontine angle tumor, seizure, acute vestibular neuritis, juvenile rheumatoid arthritis, leaving ten cases (18.2%) as unclassified. Abnormal findings were noted in 13 (23.6%) in pure tone audiogram, 3 (5.5%) in positioning test, 6 (10.9%) in bithermal caloric test, and 36 (65.5%) in rotation chair test. Conclusions: The vertigo in children with normal eardrums, who did not show otitis media or middle ear effusion, was most commonly caused by migraine and BPVC. These findings have shown to be very different from those with adult vertigo. The evaluation of vertigo in children requires a questionnaire for extensive and complete history taking, audiograms and vestibular function tests. And in selected cases, electroencephalography, hematological evaluation, imaging of the brain or temporal bone should be performed. (c) 2003 Elsevier Science Ireland Ltd. All rights reserved - CONSTANTIN T, PONYI A, GARAMI M, GERGELY L, FEKETE G, DANKO K: [Clinical characteristics of juvenile dermatomyositis]. Orv Hetil 144:25 1245-1250, 2003
Organism: Semmelweis Egyetem, Altalanos Orvostudomanyi Kar, II sz Gyermekgyogyaszati Klinika, Budapest ctamas@gyer2sotehuFAU - Constantin, Tamas
Abstract: INTRODUCTION: Dermatomyositis, belonging to the group of the idiopathic inflammatory myopathies, is characterized by bimodal pattern of age-specific incidence of rates, with peaks in age group from 5 to 14 years (juvenile dermatomyositis) and in age group from 45 to 64 years (adult dermatomyositis). THE AIM OF THIS STUDY: Is to evaluate the clinical characteristics of 12 patients with juvenile dermatomyositis followed by the 3rd Department of Internal Medicine, University of Debrecen and the 2nd Department of Pediatrics, Semmelweis University, Budapest. METHODS: The authors analyzed the medical records of the patients with juvenile and adult form of dermatomyositis retrospectively. RESULTS: All of the children had symmetrical weakness of the proximal muscles. The most frequent cutaneous features were facial erythema and Gottron papules (11/12). The extramuscular manifestations were also assessed. 7 children had arthralgia. There were observed pulmonary fibrosis, Raynaud-syndrome, dysphagia and sicca-syndrome in the same patient, whose disease is overlapped with progressive systemic sclerosis. In view of the clinical course, the authors found that prevalence of polycyclic (relapsing-remitting) and monophasic subtypes of the disease were similar (6/12 and 5/12). Finally, all of the patients achieved remission, however, 2 patients have to take low-dose corticosteroid therapy permanently to maintain remission. One patient's cutaneous symptoms proved to be persistent and in further 2 cases, regression of the calcinosis is slow, but continuous. DISCUSSION: The authors compare their data of juvenile patients with the data of the relevant literature and to their experience with the management of adult DM patients. It seems to be reasonable to treat the patients in centres - CUSHMAN KE, MAQBOOL M, GERARD PD, BEDIR E, LATA HEMANT, MORAES RM: Variation of podophyllotoxin in leaves of Eastern red cedar (Juniperus virginiana). Planta Medica 69:5 477-478, 2003
Abstract: Leaves of Eastern red cedar (Juniperus virginiana L. Cupressaceae) have been reported to contain podophyllotoxin, a pharmaceutical compound used to manufacture drugs for treatment of cancer, rheumatoid arthritis, genital warts, psoriasis, and multiple sclerosis. Podophyllotoxin content of leaves of immature, mature male, and mature female plants (apprx1.45 mgcntdotg-1) was significantly higher than that of leaves of juvenile plants (0.60 mgcntdotg-1). Sampling date also affected podophyllotoxin content. Leaves harvested in January and April exhibited higher podophyllotoxin contents (1.56 and 1.45 mgcntdotg-1, respectively) than leaves harvested in February and June (1.06 and 1.08 mgcntdotg-1, respectively). There was no obvious pattern or trend in the data due to sampling date. There was no significant interaction between plant type and sampling date. These results indicate that foliage of mature Eastern red cedar, a waste product of the lumber industry, could be a low-yielding, but relatively stable, source of podophyllotoxin - DE BENEDETTI F, PIGNATTI P, BIFFI M, BONO E, WAHID SMAIRA, INGEGNOLI F, CHANG SHENG-YUNG P, ALEXANDER H, MASSA MARGHERITA, PISTORIO A, MARTINI A, PITZALIS C, SINIGAGLIA FRANCESCO, ROGGE L: Increased expression of alpha(1,3)-fucosyltransferase-VII and P-selectin binding of synovial fluid T cells in juvenile idiopathic arthritis. Rinsho Ganka 30:7 1611-1615, 2003
Abstract: Objective: The mechanisms controlling the recruitment of T helper type 1 (Th1) cells to the inflamed synovium are not fully understood. Here, we focus on alpha(1,3)-fucosyltransferase-VII (FucT-VII), an enzyme responsible for the generation of functional P- and E-selectin ligands that is upregulated in Th1 cells. Methods: Expression of transcripts encoding FucT-VII, interferon-gamma (IFN-gamma), and interleukin 12Rbeta2 (IL-12Rbeta2) were analyzed in T cells purified from synovial fluid (SF) and from peripheral blood (PB) of children with juvenile idiopathic arthritis (JIA) using kinetic reverse transcriptase polymerase chain reaction analysis. Binding of SF and PB T cells to P-selectin was determined by flow cytometry using a soluble P-selectin/IgG1 fusion molecule. Recruitment of T cells to synovial tissue in vivo was studied by analyzing the migration of FucT-VII transfected Jurkat T cells into human rheumatoid synovial tissue grafted into SCID mice. Results: In patients with JIA, the mRNA levels of FucT-VII, as well as of IFN-gamma and IL-12Rbeta2, were up-regulated in SF T cells compared to paired PB T cells. A higher expression of FucT-VII mRNA in SF T cells was associated with increased binding of T cells to P-selectin. Moreover, FucT-VII expression and increased P-selectin binding capacity of T cells were associated with a polyarticular course of oligoarticular JIA. Expression of FucT-VII in Jurkat T cells resulted in an increased accumulation of these cells in human rheumatoid synovial-tissue grafted into SCID mice. Conclusion: Our data indicate an important role of FucT-VII in the enhanced homing of T cells to the inflamed synovium - DE CASTRO TC, TERRERI MT, LEN C, HILARIO MO: Treatment of refractory juvenile idiopathic arthritis via pulse therapy using methylprednisolone and cyclophosphamide. Sao Paulo Med J 121:3 117-120, 2003
Organism: Clinical Immunology and Rheumatology, Department of Pediatrics, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Sao Paulo, BrazilFAU - de Castro, Tania Caroline Monteiro
Abstract: CONTEXT: Patients with refractory juvenile idiopathic arthritis can benefit from aggressive therapy. CASE REPORT: We followed the clinical course of 4 patients (2 male, 2 female) aged 9.1-17.8 years (mean of 14.5 years) with polyarticular onset of juvenile rheumatoid arthritis and one 16-year-old boy with juvenile spondyloarthropathy associated with inflammatory bowel disease. All the juvenile rheumatoid arthritis patients fulfilled the diagnostic criteria established by the American College of Rheumatology. All patients had unremitting arthritis despite maximum therapy. All patients began receiving treatment using intravenous cyclophosphamide at 500-750 mg/m and intravenous methylprednisolone at 30 mg/kg, for 3 days monthly (1 g maximum). The patients received between 3 and 11 monthly treatments, and/or 3-5 treatments every two months for 12 months, according to the severity of the disease and/or response to the therapy. All but one patient were evaluated retrospectively at the start (time 0) and 6 months (time 1), and 12 months (time 2) after the beginning of the treatment. A rapid and clinically significant suppression of systemic and articular manifestations was seen in all patients. Our results showed the favorable effect of this treatment on the clinical and some laboratory manifestations of juvenile idiopathic arthritis - DORIA AS, DE CASTRO CC, KISS MH, SERNIK RA, VITULE LF, SILVA CH, ZERBINI CA, ARANTES PR, LUCATO L, GERMANO MA, CERRI GG: Inter- and intrareader variability in the interpretation of two radiographic classification systems for juvenile rheumatoid arthritis. Pediatr Radiol 33:10 673-681, 2003
Organism: Department of Diagnostic Imaging, Heart Institute (InCor) and Radiology Institute (InRad), Hospital das Clinicas da Universidade de Sao Paulo, Sao Paulo, Brazil, andreadoria@sickkidscaFAU - Doria, Andrea S
Abstract: OBJECTIVE. To evaluate the inter- and intrareader variability for interpretation of a modified Larsen's radiographic classification system for juvenile rheumatoid arthritis (JRA) focused on osteochondral lesions and a conventional Larsen's classification system, compared to a reference MR scoring system of corresponding images. MATERIALS AND METHODS. Seventy-five radiographs of 60 children with JRA, performed within a short interval of time from the MR examinations, were independently evaluated by three experienced radiologists, three diagnostic imaging residents and three rheumatologists, in two separate sessions, according to the two different classification methods, blinded to the corresponding MR images. RESULTS. The inter- and intrareader concordance rates between the two radiographic classification systems and the MR-related radiographs were respectively poor and poor/moderate. The interobserver range of weighted kappa values for the conventional and the modified Larsen's system respectively was 0.25-0.37 vs 0.19-0.39 for radiologists, 0.25-0.37 vs 0.18-0.30 for residents and 0.19-0.51 vs 0.17-0.29 for rheumatologists. The intrareader rate ranged from 0.17-0.55 for radiologists, 0.2-0.56 for residents, and 0.14-0.59 for rheumatologists. CONCLUSION. Although the proposal of a new radiographic classification system for JRA focused on osteochondral abnormalities sounds promising, the low inter- and intrareader concordance rates with an MR-related radiographic system makes the clinical applicability of such a radiographic system less suitable - FLEISCHMANN R: Safety and efficacy of disease-modifying antirheumatic agents in rheumatoid arthritis and juvenile rheumatoid arthritis. Expert Opin Drug Saf 2:4 347-365, 2003
Organism: University of Texas Southwestern Medical Center at Dallas, St Paul University Hospital, 5939 Harry Hines Boulevard, Suite 400, Dallas, Texas 75235, USA royfleischmann@radiantresearchcomFAU - Fleischmann, Roy
Abstract: The definition of disease-modifying antirheumatic drugs (DMARDs) has changed dramatically over the last decade. Current expectations of efficacy now include amelioration of signs and symptoms of disease activity as well as slowing, if not complete cessation, of disease progression as evidenced by Xray progression and significant improvement of patient function. Rheumatologists assess the safety profile of these agents more critically in an attempt to increase the risk:benefit profile. Traditional agents, such as methotrexate (MTX), sulfasalazine and leflunomide have provided patients with substantial relief of symptoms and some decrease of X-ray progression but have been hampered by the frequent occurrence of significant adverse events (AEs) and inability to maintain benefit for a prolonged period of time. With the increased understanding of the basic mechanism of the disease process, there has been the introduction of four biological disease-modifying agents introduced into clinical practice which have substantially increased the risk:benefit ratio for patients with various rheumatic diseases - FOELDVARI I, KRUGER E, SCHNEIDER T: Acute, non-obstructive, sterile cholecystitis associated with etanercept and infliximab for the treatment of juvenile polyarticular rheumatoid arthritis. Ann Rheum Dis 62:9 908-909, 2003
- HACKETT J: Perceptions of play and leisure in junior school aged children with juvenile idiopathic arthritis: What are the implications for occupational therapy? British Journal of Occupational Therapy (United Kingdom ) 66:7 303-310, 2003
Abstract: Play is the dominant occupation in childhood and is recognised as having an important role in wellbeing. Past research suggests that children with a disability experience a variety of barriers to engagement in play. Despite this, play is often not assessed as a specific area of occupational performance. This qualitative study aimed to explore the perceptions of play and leisure in junior school aged children with juvenile idiopathic arthritis. Twelve children between the ages of 7 years and 11 years were interviewed in order to uncover the barriers to engagement in play and leisure and to consider the implications, if any, for occupational therapy. The results showed that all children, regardless of disease activity, reported difficulty in engaging in play and leisure activities. The symptoms of the disease, the treatment regimes and their side-effects, and psychosocial factors were all reported to affect play and leisure experiences. The children also reported that play behaviours were often restricted by parents, friends and school personnel. Fear of damage and unclear communication about the effects of activity also resulted in self-imposed restrictions, which further limited play experiences. Although the children reported a number of coping strategies to deal with these difficulties, they reported more indoor play and engagement in sedentary activities which often gave rise to feelings of being different, sadness and loneliness. These findings may have important consequences for occupational therapy and provide a timely reminder about the significance of assessing play and leisure as distinct areas of occupational performance in children - JOHNSON K: Commentary on "Inter- and intrareader variability in the interpretation of two radiographic classification systems for juvenile rheumatoid arthritis". Pediatr Radiol 33:10 671-672, 2003
Organism: Department of Radiology, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK, karljohnson@bchnhsukFAU - Johnson, Karl - KHODADOUST A, QUINTER AP: Microsurgical approach to the conjunctival flap. Arch Ophthalmol 121:8 1189-1193, 2003
Organism: Connecticut Eyecare Center, New Haven, CT 06519, USAFAU - Khodadoust, Ali
Abstract: OBJECTIVES: To describe a microsurgical approach to a selective pedunculated conjunctival flap for the treatment of chronic corneal ulceration with or without corneal perforation, and to present the results of 50 consecutive cases. DESIGN: Retrospective, noncomparative case series and case reports.Patients All patients who had microsurgical conjunctival flap procedures for the treatment of chronic corneal ulcers between 1982 and 1996 at the Connecticut Eyecare Center, New Haven. INTERVENTIONS: Partial pedunculated conjunctival flap surgery. METHODS: Review of the initial ocular diagnoses and characteristics as well as retrospective study of the postoperative course. MAIN OUTCOME MEASURES: Resolution of the corneal ulcer and postoperative stability of the conjunctival flap. RESULTS: Sixty-two percent of the corneal ulcers treated were nonperforated, and 38% were perforated. The diagnoses included herpes simplex virus (14 patients), bacterial ulcer (11 patients), rheumatoid arthritis (8 patients), aphakic bullous keratopathy (6 patients), graft rejection (4 patients), herpes zoster virus (5 patients), and other (2 patients). Postoperatively, 94% of the conjunctival flaps were stable, and 3% had failed. The procedures were definitive in 64% of cases and temporary in 36%; 61% of patients received a corneal transplant 6 to 24 months postoperatively, and in 38% the flaps were removed 6 to 12 months after the original lesion had healed. CONCLUSION: A selective pedunculated conjunctival flap is an effective and practical surgical approach to the treatment of perforated and nonperforated corneal ulcers that have not responded to other types of medical therapy - KYNGAS H: Patient education: perspective of adolescents with a chronic disease. J Clin Nurs 12:5 744-751, 2003
Organism: Department of Nursing and Health Administration, University Hospital, University of Oulu, Oulu, Finland helvikyngas@oulufiFAU - Kyngas, Helvi
Abstract: The purpose of this study was to describe patient education from the perspective of adolescents. Data were collected by interviewing adolescents who had asthma, epilepsy, juvenile rheumatoid arthritis, and insulin-dependent diabetes mellitus. The sample consisted of 40 Finnish adolescents aged between 13 and 17 years. The interview data were analysed with methods of content analysis. From the perspective of adolescents with a chronic disease, patient education can be divided into the following categories: routine programmes, problematic planning issues, atmosphere of patient education session and written patient education material. Some features of ideal patient education also emerged. In a routine programme, patient education was based on the professional knowledge of the physicians and nurses rather than the needs of the adolescents. It was provided at a time that was good for the nurses or physicians. The level of education was not compatible with each developmental level of the adolescent. Problematic planning issues included a poorly outlined plan of education and a lack of systematic and continuous education. Educational communication consisted of dialogue between the adolescent and the educator. An encouraging atmosphere developed when the educators motivated the adolescents, respected them and their opinions and encouraged them to express their feelings, to ask questions and to relate experiences. Also, it was important that the adolescents' opinions were respected. In ideal patient education, the sessions had been planned well beforehand based on the adolescents' needs and written patient education material. Ideal patient education helped adolescents to acquire skills to take care of themselves and provided information on how to adjust to different situations and problems. The results provided useful insight into patient education and served to raise awareness of the problems and difficulties experienced by adolescents with a chronic disease - LABYAK SE, BOURGUIGNON C, DOCHERTY S: Sleep quality in children with juvenile rheumatoid arthritis. Holist Nurs Pract 17:4 193-200, 2003
Organism: Department of Family and Child Nursing, School of Nursing, University of Washington, Seattle 98195-7262, USA slabyak@uwashingtoneduFAU - Labyak, Susan E
Abstract: Children with juvenile rheumatoid arthritis (JRA) report poor sleep quality, daytime sleepiness, fatigue, anxiety, and altered mood. Sleep disturbances in school-aged children are an issue of serious concern. Children are at an age when sleep is of primary importance to physical and intellectual growth, and sleep disturbances that begin in childhood may persist into adulthood. In this article we will review what is currently known about sleep in children with JRA, the influence of medications on sleep quality, the potential impact of poor sleep quality on daily life issues, and complementary/alternative modalities that may be effective in reducing sleep disturbances - LEE DM, SCHUR PH: Clinical utility of the anti-CCP assay in patients with rheumatic diseases. Ann Rheum Dis 62:9 870-874, 2003
Organism: Department of Medicine, Division of Rheumatology, Immunology and Allergy Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USAFAU - Lee, D M
Abstract: OBJECTIVES: To determine the frequency of antibodies to cyclic citrullinated peptides (CCP) in a group of patients with a diversity of rheumatic diseases. METHODS: 249 consecutive sera from an arthritis clinic sent for rheumatology testing were selected for testing with the anti-CCP2 assays and for the presence of rheumatoid factor (RF). Patient charts were reviewed for demographic information, clinical diagnosis, radiographic information, and other laboratory data. RESULTS: The sensitivity and specificity of anti-CCP reactivity for the diagnosis of rheumatoid arthritis (RA) were 66.0% and 90.4%, respectively. This compared with the sensitivity and specificity of RF for RA at 71.6% and 80.3%. Furthermore, 10/29 (34%) RF- patients with RA demonstrated reactivity to CCP. The presence of either anti-CCP or RF increased testing sensitivity for diagnosis of RA to 81.4%; the presence of both RF and anti-CCP demonstrated a testing specificity similar to that of anti-CCP reactivity alone for the diagnosis of RA (91.1%). CONCLUSIONS: The detection of anti-CCP is useful for the diagnosis of RA, in fact even more so than RF, because of its higher specificity - LEONE V, PRESANI G, PERTICARARI S, TOMMASINI A, CROVELLA S, LENHARDT A, PICCO P, LEPORE L: Chronic infantile neurological cutaneous articular syndrome: CD10 over-expression in neutrophils is a possible key to the pathogenesis of the disease. Eur J Pediatr 162:10 669-673, 2003
Organism: Clinica Pediatrica, IRCCS Burlo Garofolo, Via dell'Istria 65/1, 34137 Trieste, ItalyFAU - Leone, Valentina
Abstract: Chronic, Infantile, Neurological, Cutaneous and Articular Syndrome (CINCA) or Neonatal/Infantile Onset Multisystem Inflammatory Disease (NOMID/IOMID) is a rare, multisystem inflammatory disease characterised by neonatal onset of urticarial symptoms, persistent rash, ocular inflammatory lesions, progressive articular and neurological involvement and associated with characteristic overgrowth of the ossification nucleus of the patella. The tissues involved are extensively infiltrated by inflammatory cells, mostly neutrophils. This paper describes the clinical features of three new cases as well as a study of activation markers in neutrophils and search for mutations of the CIAS1gene in these patients. Clinical records of three cases of CINCA are reported. For genetic analysis, exon 3 of the CIAS1gene was amplified and sequenced. Immunophenotype, oxidative burst and phagocytosis were analysed in neutrophils obtained from all the three CINCA patients as well as from eight juvenile idiopathic arthritis (JIA) patients and eight healthy controls. Functional assays in neutrophils were normal in all three patients with CINCA syndrome and did not differ from those of JIA patients and healthy controls. The surface density of CD10 was significantly higher on neutrophils from CINCA patients as compared to those of JIA and controls (P<0.0005). In one subject a new missense mutation in the CIAS1gene was identified. CONCLUSION: the hyper expression of the activation antigen CD10/NEP in neutrophils from these three cases of CINCA, as compared to JIA patients and healthy controls, irrespective of the presence of mutations in CIAS1, could be a marker of the inflammatory disorder typical of some patients with CINCA syndrome - LEWIS JG, FLORKOWSKI CM, ELDER PA, HUNT PJ: Rheumatoid factor and false positive sex-hormone binding globulin. Clinica Chimica Acta 332:1-2 139-141, 2003
- LIAO C-H, HUANG J-L: Juvenile ankylosing spondylitis with uveitis. Asian Pacific Journal of Allergy and Immunology (Thailand ) 21:1 69-71, 2003
Abstract: A 17-year-old boy had suffered from right ankle arthralgia when he was 13 years old. He also had bilaterally congested conjunctivas and were erythematous around his right ankle joint. A soft tissue echo showed swelling of the right ankle joint. A Ga 67 scan revealed a focal elevated up-take in the right ankle, but a bone scan was negative. Reactive arthritis was suspected due to conjunctivitis, arthritis and a previous episode of watery diarrhea. An ophthalmologic examination showed no evidence of uveitis. Laboratory data were negative for rheumatoid factor, antinuclear antibody and anti-ds DNA. Erythrocyte sedimentation rate (ESR) was 40mm/hr and a histocompatibility test was positive for antigen B27. Based on the diagnosis of cellulitis and reactive arthritis, oxacillin and naproxen were given for 14 days. During follow-up at the OPD, bilateral arthralgia of the ankle joints was noted and a sonography showed bilateral edematous ankle joints. Juvenile ankylosing spondylitis (JAS) was suspected. Two years later, he had lower back pain and arthralgia of the knee joints with uveitis of the right eye. He was treated with naproxen and prednisolone. Because few JAS cases initially present as axial arthropathy or enthesopathy and uveitis is uncommon in children, we presented the case with a review of literature and conclusion that the possibility of JAS should be considered in young adolescent boys with arthritis of the lower limbs, enthesitis, a family history of related diseases and positive HLA-B27, as well as negative rheumatoid factor (RF) and anti-nuclear antibody (ANA) results - LIAO CH, HUANG JL: Juvenile ankylosing spondylitis with uveitis. Asian Pac J Allergy Immunol 21:1 69-71, 2003
Organism: Division of Allergy, Asthma, and Rheumatology, Department of Pediatrics, Chang Gung Children's Hospital, Chang Gung University, Taoyuan, TaiwanFAU - Liao, Chiang-Hua
Abstract: A 17-year-old boy had suffered from right ankle arthralgia when he was 13 years old. He also had bilaterally congested conjunctivas and were erythematous around his right ankle joint. A soft tissue echo showed swelling of the right ankle joint. A Ga 67 scan revealed a focal elevated uptake in the right ankle, but a bone scan was negative. Reactive arthritis was suspected due to conjunctivitis, arthritis and a previous episode of watery diarrhea. An ophthalmologic examination showed no evidence of uveitis. Laboratory data were negative for rheumatoid factor, antinuclear antibody and anti-ds DNA. Erythrocyte sedimentation rate (ESR) was 40 mm/hr and a histocompatibility test was positive for antigen B27. Based on the diagnosis of cellulitis and reactive arthritis, oxacillin and naproxen were given for 14 days. During follow-up at the OPD, bilateral arthralgia of the ankle joints was noted and a sonography showed bilateral edematous ankle joints. Juvenile ankylosing spondylitis (JAS) was suspected. Two years later, he had lower back pain and arthralgia of the knee joints with uveitis of the right eye. He was treated with naproxen and prednisolone. Because few JAS cases initially present as axial arthropathy or enthesopathy and uveitis is uncommon in children, we presented the case with a review of literature and conclusion that the possibility of JAS should be considered in young adolescent boys with arthritis of the lower limbs, enthesitis, a family history of related diseases and positive HLA-B27, as well as negative rheumatoid factor (RF) and anti-nuclear antibody (ANA) results - LIEN G, FLATO B, HAUGEN M, VINJE O, SORSKAAR D, DALE K, JOHNSTON V, EGELAND T, FORRE O: Frequency of osteopenia in adolescents with early-onset juvenile idiopathic arthritis: a long-term outcome study of one hundred five patients. Arthritis Rheum 48:8 2214-2223, 2003
Organism: Department of Rheumatology, Rikshospitalet University Hospital, Oslo 0027, Norway gunhildlien@rikshospitaletnoFAU - Lien, Gunhild
Abstract: OBJECTIVE: To determine the frequency of low bone mineral content (BMC) and low bone mineral density (BMD) as long-term complications in adolescents with early-onset juvenile idiopathic arthritis (JIA), and to identify disease variables, patient characteristics, and biochemical bone markers related to low bone mass. METHODS: One hundred five (87%) of 121 adolescent patients with early-onset JIA (ages 13-19 years, 80 girls and 25 boys, mean age at onset of JIA 2.8 years), from a cohort first admitted to the hospital between 1980 and 1985, were assessed after a mean disease duration of 14.2 years. BMC and BMD of the total body, the lumbar spine at L2-L4, and the femoral neck were measured by dual-energy x-ray absorptiometry. Age- and sex-specific reference values from a pooled, healthy reference population were used to calculate Z scores. Low bone mass was defined as a Z score less than -1 SD. RESULTS: Among the 103 adolescent JIA patients who underwent total-body imaging, 41% had low total-body BMC and 34% had low total-body BMD. Compared with adolescent JIA patients who had normal total-body BMC, those with low BMC had lower mean weight (P < 0.001), height (P < 0.001), lean mass (P < 0.001), and remission rates (P = 0.016), had longer duration of active disease (P = 0.013), had higher numbers of active and mobility-restricted joints (P < 0.001 and P = 0.001, respectively), had more disability (P = 0.011), had higher frequencies of joint erosions (P < 0.001), and had higher erythrocyte sedimentation rates (P = 0.033). In multiple linear regression analyses of total-body BMC, 88% of the variance was explained by the duration of active disease, the number of joints with restricted mobility, the bone area, urinary deoxypyridinoline values, age, weight, and height. CONCLUSION: Forty-one percent of the adolescents with early-onset JIA had low bone mass >11 years after disease onset. The development of low total-body BMC was related to the duration of active disease, disease severity, measures of bone resorption, weight, and height - MANNERS P, LESSLIE J, SPELDEWINDE D, TUNBRIDGE D: Classification of juvenile idiopathic arthritis: should family history be included in the criteria? J Rheumatol 30:8 1857-1863, 2003
Organism: Department of Paediatrics, University of Western Australia, Perth, AustraliaFAU - Manners, Prudence
Abstract: OBJECTIVE: (i) To determine the efficacy of the Durban classification for children with juvenile idiopathic arthritis (JIA) where < 5 joints were involved at onset (with systemic arthritis excluded) by determining the proportion of the cohort that proved to be "unclassifiable"; (ii) to define reasons for cases being "unclassifiable," particularly regarding family history; and (iii) to compare the efficacy of a proposed hierarchical system (an unofficial modification of the Durban classification) with the Durban classification, where family history details are included as descriptors, rather than as classification criteria. METHODS: Charts were reviewed of 50 children with fewer than 5 joints involved at presentation for JIA, followed for at least 12 months, with systemic arthritis excluded. Cases were classified according to the EULAR criteria, the Durban criteria, and by a proposed "modified Durban" classification subject to hierarchy, with exclusions in the following order: systemic arthritis, rheumatoid factor (RF) positive arthritis, psoriasis or a combination of dactylitis and psoriatic nail changes (psoriatic arthritis), and HLA-B27 positive arthritis (enthesitis related arthritis), with the remainder of children being classified as having either RF negative polyarthritis or RF negative oligoarthritis, depending on number of joints involved, with additional information noted as descriptors. The "modified Durban" classification was proposed only to stimulate discussion among clinicians. RESULTS: Of 50 children, 56% were "unclassifiable" by the Durban classification, mainly because of inadequate family history despite appropriate questioning. Using the proposed "modified Durban" classification, 2% were "unclassifiable." Family history was classified as inadequate for the following reasons: The parents did not know family history; the child or parent was adopted; the father was unknown or parent died early; parents never attended; extended family had lost communication with parents; or a relative was considered to have psoriasis, but not confirmed by dermatologists. Other reasons for "unclassifiable" included: dermatologists unable to confirm psoriasis; family history of inflammatory bowel disease and sacroiliitis but B27 status unknown; proband B27 negative but family history of B27-related disease; family history of psoriasis, but patient had insufficient criteria for psoriatic arthritis and therefore excluded from oligoarthritis, psoriatic arthritis and other groups. CONCLUSION: (i) The Durban classification showed poor efficacy for JIA where < 5 joints were involved at onset, with more than half the cases being "unclassifiable". (ii) The most common reason was that appropriate family history was not available despite being sought by the clinician. (iii) A proposed hierarchical system, an unofficial modification of the Durban classification, showed good efficacy, with only one of 50 cases being "unclassifiable." - MICHEL M, DUVOUX C, HEZODE C, CHERQUI D: Fulminant hepatitis after infliximab in a patient with hepatitis B virus treated for an adult onset Still's disease. Rinsho Ganka 30:7 1624-1625, 2003
Abstract: Infliximab, a chimeric anti-tumor necrosis factor-alpha monoclonal antibody, has been demonstrated to be efficient and safe in patients with active rheumatoid arthritis and in the management of severe bouts of Crohn's disease. However, the safety of infliximab has not been evaluated in patients infected with hepatitis B virus. We report the case of a 28-year-old woman, with a positive hepatitis B virus surface antigen, who developed fulminant hepatitis 2 weeks after receiving a second infliximab infusion for a refractory adult onset Still's disease - MOKKAPATI P, ARUMUGAM P, BABU R, NALLARI P: Superoxide dismutase (SOD) as a genetic marker for rheumatic diseases. European Journal of Human Genetics 9:Supplement 1 1305,
- MUHLHAUSER U, MUNZEL F, ESCHENHAGEN T: [Etanercept]. Dtsch Med Wochenschr 128:34-35 1775-1778, 2003
Organism: Institut fur Experimentelle und Klinische Pharmakologie, Universitatsklinikum Hamburg-Eppendorf, HamburgFAU - Muhlhauser, U - PRAKKEN BJ, ROORD S, RONAGHY A, WAUBEN M, ALBANI S, VAN EDEN W: Heat shock protein 60 and adjuvant arthritis: a model for T cell regulation in human arthritis. Springer Semin Immunopathol 25:1 47-63, 2003
Organism: Department of Paediatric Immunology, University Medical Centre Utrecht, Wilhelmina Children's Hospital, Home Mailbox KC030630, PO Box 85090, 3508 AB, Utrecht, The NetherlandsFAU - Prakken, Berent J
Abstract: Heat shock proteins (hsp) are highly conserved, immune-dominant microbial proteins, whose expression is increased at sites of inflammation. In the experimental model of adjuvant arthritis (AA) immune responses to hsp determine the outcome of disease. AA can be transferred with a single T cell clone specific for a sequence of mycobacterial hsp65 (Mhsp65). Immunization with whole Mhsp65 on the other hand, protects in virtually all forms of experimental arthritis, including AA. This protective effect seems the consequence of the induction of a T cell response directed against self-hsp60. A similar protective effect of self-hsp60-specific T cells seems present in patients with a spontaneous remitting form of juvenile idiopathic arthritis. Next to hsp60, other hsp have similar protective effects in arthritis, while other conserved microbial proteins lack such capacity. Nasal administration of hsp60 peptides induces IL-10-driven regulatory T cells that are highly effective in suppressing arthritis. Thus hsp60, or peptides derived from hsp60, are suitable candidates for immune therapy in chronic arthritis - ROIFMAN CM, MELAMED I: A novel syndrome of combined immunodeficiency, autoimmunity and spondylometaphyseal dysplasia. Clinical Genetics (United Kingdom ) 63:6 522-529, 2003
Abstract: We describe here four patients who appear to have similar clinical and immunological features which constitute a novel syndrome. The patients present with short stature owing to spondylometaphyseal dysplasia and with severe infections as the result of a combined humoral and cellular immune deficiency. Presumably because of dysregulation of the immune system, all patients also developed autoimmune manifestations - ROSE AJ: Bone and joint diseases around the world. France: rheumatoid polyarthritis, chronic juvenile idiopathic arthritis, and ankylosing spondylitis. A public health priority. J Rheumatol Suppl 67:45-6.: 45-46, 2003
Organism: French Association of Polyarthritis, Paris, FranceFAU - Rose, Aimee Jeanne
Abstract: Since its creation, the Association francaise des Polyarthrites is doing everything possible to come to the aid of people with polyarthritic diseases and to help medical research make advances against this illness. Each year, with the help of sponsors, we organize a national information campaign with first-hand accounts and presentations by sufferers on radio and television stations, so that polyarthritis should be better understood by the general public. Last year, together with other associations, we asked the Minister of Health about the barriers to receiving innovative treatment for polyarthritis because of the cost. Because of this action, the Minister has made additional funds available so that more sufferers can benefit from new treatment. Recently, several associations dealing with chronic and serious inflammatory rheumatic illnesses who came together as an action group presented a text to the Minister of Health about the urgent need to make these illnesses a public health priority. Working sessions between the Minister, patient associations, and rheumatologists to consider how to implement a public health plan are in progress - RUBBERT-ROTH A, PERNIOK A: [Treatment of patients with rheumatoid arthritis with the interleukin-1 receptor antagonist anakinra (Kineret)]. Z Rheumatol 62:4 367-377, 2003
Organism: Med Klinik I der Universitat zu Koln, Joseph-Stelzmann-Str 9, 50924 Koln, Germany AndreaRubbert@medizinuni-koelndeFAU - Rubbert-Roth, Andrea
Abstract: New treatment strategies in rheumatoid arthritis are targeted to interfere with critical mediators of inflammation. Proinflammatory cytokines like IL-1 beta and TNFalpha play a crucial role in induction and maintenance of synovitis, pannus formation and bone and cartilage destruction. Within a few years, these morphological changes may lead to joint destruction and consecutively to functional impairment.Since April 2002 a recombinant human interleukin-1 receptor antagonist (Anakinra) is available in Germany for treatment of patients with rheumatoid arthritis. Anakinra (Kineret(R)) is approved for therapy in combination with methotrexate and should be applied according to guidelines established by the German Rheumatology Society for the use of biologicals in treatment of patients with rheumatoid arthritis.The approval of anakinra as a new therapeutic is based on data obtained in large multicenter, placebo-controlled, and randomised trials in comparison to placebo. Treatment of Anakinra as monotherapy or in combination with methotrexate lead to significant improvement of signs and symptoms of disease as measured by the ACR 20 (or more) response and was associated with a slower radiographic progression with regard to joint space narrowing and development of erosions. Anakinra showed a favourable safety profile with injection side reactions as the predominant side effect that occurs in 70% of patients usually after 10-12 days of treatment and that are mostly mild to moderate and self-limiting. Patients with previous pneumonia or other risk factors for pulmonary infections such as chronic obstructive lung disease seem to show a slightly increased risk of developing infectious complications of the bronchopulmonary system while being on anakinra and should be monitored appropriately. Combining IL-1ra treatment with the use of anti-TNF agents showed an increased risk of infectious complications in clinical studies and is not recommended at present. Studies are currently assessing the use of anakinra for treatment of other rheumatic diseases like psoriatic arthritis, juvenile arthritis or spondylarthropathy - RYSAVA R, SPICKA I, MERTA M, TESAR V, TRNENY M: [Is the current treatment of primary and secondary amyloidosis effective?]. Cas Lek Cesk 142:6 370-372, 2003
Organism: I interni klinika 1 LF UK a VFN, Praha rysavar@msvfnczFAU - Rysava, R
Abstract: BACKGROUND: Retrospective study about results of treatment of patients (pts) with primary (AL) and secondary (AA) amyloidosis is given. 31 pts with systemic forms of amyloidosis have been treated and followed-up in our department since 1993. METHODS AND RESULTS: 6 men and 11 women were in the AL group with the mean age of 59 years. Multiple myeloma was diagnosed in 9 pts, monoclonal gammapathy of undetermined significance (MGUS) was found in 8 pts. The kidneys were affected in all pts, heart in 59% of pts, liver, joints and skin in 26% of pts and polyneuropathy was detected only in 1 pt. Progression of renal insufficiency with decrease of glomerular filtration rate (GFR) was detected in the AL group at the end of follow-up period compared with the initial level (p < 0.05) despite the intensive treatment. The difference did not reach statistical significance in other investigated parameters. Median of survival was 13 months from the assessment of diagnosis. Partial remission of amyloidosis was achieved in 9 pts, stable disease was in 5 pts and in 3 pts the disease progressed. 4 men and 10 women were in the AA group with mean age of 58 years. Underlying disease was rheumatoid arthritis in 7 pts, ankylosing spondylitis in 2 pts, juvenile chronic arthritis in 1 pt, Crohn's disease in 2 pts, eosinophilic fasciitis in 1 pt and chronic abscesses in NK cell deficiency in 1 pt. The kidneys were affected in all pts, bowels and heart in 36% of pts. GFR (p < 0.05) and plasma creatinine (p < 0.01) significantly decreased at the end of follow-up period compared with initial levels. Median of survival was 30 months. Partial remission was achieved in 2 pts, stable disease was in 3 pts and progression was detected in 9 pts despite the use of various treatment regimens. CONCLUSIONS: Both forms of systemic amyloidosis represent severe disease with limited response to treatment. The use of new drugs is promising and could lead to better response to treatment - SAHIN YT, TOKER K, AKANSEL G: Juvenile rheumatoid arthritis (still's disease) and difficult intubation (case report)
JUVENIL ROMATOID ARTRIT (STILL'S HASTALIGI) VE ZOR ENTUBASYON (OLGU SUNUMU). Anestezi Dergisi (Turkey ) 11:2 151-154, 2003
Abstract: Special care must be given to the patients with rheumatoid arthritis during airway management, as endotracheal intubation may be difficult with these patients. We present a case of rheumatoid arthritis who was taken into the operation room in emergency conditions with the diagnosis of acute abdominal distention and whose airway was difficulty managed. By means of this case we aimed to examine the possible causes of difficult intubation in patients with rheumatoid arthritis and to search alternative methods for the endotracheal intubation - SALLFORS C, HALLBERG LRM: A parental perspective on living with a chronically III child: A qualitative study. Families Systems and Health (United States ), 21:2 193-204, 2003
Abstract: This study explored parents' experience of living with a child with Juvenile Chronic Arthritis (JCA). Taped, open interviews with 22 parents (6 fathers) were transcribed and analyzed using the constant comparative method for grounded theory. A core category, labeled "parental vigilance," and two related categories labeled "emotional challenges" and "continual adjustment" were identified, describing the parents' management of illness-related demands, the child's vulnerability, and others' misunderstandings. Seeking information, using day-to-day strategies, seeking social support, and communicating with others are the most efficient strategies adopted by the parents. The findings indicate complementary parental roles labeled "the managing mother" and "the waiting father." The study contributes to a deeper understanding of experienced parental stress in daily living with a chronically ill child, and reflects traditional gender-based parenting roles, complementary and necessary for the family. The findings have implications for professionals who provide care to children with JCA - SELVAAG AM, FLATO B, LIEN G, SORSKAAR D, VINJE ODD, FORRE O: Measuring health status in early juvenile idiopathic arthritis: Determinants and responsiveness of the Child Health Questionnaire. Rinsho Ganka 30:7 1602-1610, 2003
Abstract: Objective: To assess the determinants and responsiveness of the Norwegian version of the Child Health Questionnaire (CHQ) in patients with early juvenile idiopathic arthritis (JIA) and to compare health status in patients and controls. Methods: A total of 116 children (median age 8.4 yrs) with JIA and <2.5 years of disease duration (median 11.0 mo) were examined by a pediatric rheumatologist and reassessed after a median of 10.0 months. Physical and psychosocial health were assessed by means of the CHQ, which provides summary scores for physical and psychosocial health, the Childhood Health Assessment Questionnaire (CHAQ), and the Child Behavior Checklist (CBCL, n=32). Matched controls (n=116), randomly selected from the general population, completed the CHQ at baseline. Results: The patients with JIA had poorer physical health and slightly impaired psychosocial health compared with the controls (41.2+-13.6 vs 55.2+-7.3 (p<0.001) and 51.0+-7.5 vs 54.1+-5.7 (p=0.002), respectively). The most important determinants of the CHQ physical summary score were the child's pain, morning stiffness, the CRAQ disability index, erythrocyte sedimentation rate (ESR), overall well-being, and physician's global assessment of disease activity. The psychosocial summary score correlated with the CBCL level of internalizing, externalizing, and total behavior problems. The standardized response mean for the physical summary score was large (0.96) for those who improved, and moderate (-0.60) for those who became worse. Conclusion: The CHQ discriminated between patients with early, JIA and controls. The most important determinants of the CHQ physical summary score were the child's pain, morning stiffness, CHAQ, ESR, overall well-being, and physician's global assessment of disease activity. The CHQ was sensitive to clinical changes in children with JIA - SITWALA K, V, MOR-VAKNIN N, MARKOVITZ DM: Minireview: DEK and gene regulation, oncogenesis and AIDS. Anticancer Research 23:3A 2155-2158, 2003
Abstract: A brief review summarizing the relationships that exist between the DEK protein and gene regulation, oncogenesis and AIDS - SKOUMAL M, KOLARZ G, KLINGLER A: Serum levels of cartilage oligomeric matrix protein. A predicting factor and a valuable parameter for disease management in rheumatoid arthritis. Scand J Rheumatol 32:3 156-161, 2003
Organism: Institute for Rheumatology of the Kurstadt Baden, Austria martinskoumal@a1netFAU - Skoumal, M
Abstract: OBJECTIVE: To examine whether cartilage oligomeric matrix protein (COMP) correlates with inflammation and/or joint destruction of patients with rheumatoid arthritis (RA) and to test COMP as predicting factor for the outcome of patients with established RA. METHODS: Serum levels of COMP were measured in sera of 62 patients, suffering from RA according to the ACR criteria and treated in intervals in our department, over a period of 5 years. A commercially available sandwich--type ELISA-kit developed by AnaMar Medical AB, Sweden, was used. The results of serum COMP were compared with the Disease Activity Score (DAS), the Larsen Score, and clinical and laboratory parameters. RESULTS: We found a positive correlation between serum levels of COMP at baseline and deterioration of Larsen score even after 5 years (p < 0.007; r = 0.34). To confirm serum COMP as an independent predicting factor for patients with RA we looked at a subgroup of patients (n = 17) with elevated serum levels of COMP (mean 11,7 U/l) and low clinical prognostic factors. In this subgroup we also found a significant correlation with delta Larsen score (p < 0.01; r = 0.59) after 5 years. CONCLUSION: Serum levels of COMP is known to reflect increased cartilage turnover. The results indicate that serum COMP may be used as a prognostic marker of cartilage degradation in a patient group with established RA - SUSS GB: Illness concepts of primary school children - An educational training program for children with juvenile chronic arthritis
KRANKHEITSKONZEPTE UND PATIENTENSCHULUNG BEI JUVENILER CHRONISCHER ARTHRITIS. Monatsschrift fur Kinderheilkunde (Germany ) 151:7 738-744, 2003
Abstract: Background. The aim of the study was to examine illness concepts of children with juvenile chronic arthritis and to evaluate a new educational training program. Methods. The illness concepts were drawn up in a short semi-structured interview. The answers of 33 children aged 6 to 10 were clustered by content. The program that followed for 16 of these children used video demonstration, role play, transparencies and worksheets. The evaluation was based on the comparison with a unspecifically treated control-group of 17 children. Findings. A descriptive presentation of the results shows that these children mostly have no or very vague ideas about cause, processes and treatment of their illness. With the help of the program the knowledge in these areas grew significantly. Interpretation. The effect of the education considering further the assumed impact on well-being and compliance - is to be seen as extremely helpfull for these children beyond their increase of knowledge - TAKKEN T, VAN DER NJ, KUIS W, HELDERS PJ: Physical activity and health related physical fitness in children with juvenile idiopathic arthritis. Ann Rheum Dis 62:9 885-889, 2003
Organism: Department of Paediatric Physical Therapy, University Hospital for Children and Youth Het Wilhelmina Kinderziekenhuis, University Medical Centre Utrecht, Utrecht, The NetherlandsFAU - Takken, T
Abstract: OBJECTIVE: To obtain insight into the interaction between daily physical activity and components of health related physical fitness in children with juvenile idiopathic arthritis. METHODS: Forty five patients (10 male/35 female; mean (SD) age 8.9 (2.2) years) participated in the study. Body mass, height, skinfold thickness, number of swollen joints, and joint range of motion were determined. The maximal oxygen consumption (VO(2peak)) was assessed during a graded maximal bicycle exercise test. Daily physical activity levels were measured with a Caltrac activity monitor and a parental physical activity rating (PAL) on a five point Likert scale. RESULTS: Partial correlation coefficients (to control for age) between physical activity and indices of health related physical fitness showed significant relationships between Caltrac motion counts and absolute VO(2peak) (r=0.31) and relative VO(2peak) (r=0.34), but not with the indices of body composition. There was also a significant correlation between PAL and relative VO(2peak) (r=0.33). CONCLUSIONS: Physical activity was significantly related to cardiorespiratory fitness but not to body composition in children with juvenile idiopathic arthritis. A longitudinal follow up should show whether an active lifestyle protects for loss of aerobic fitness in this patient group - TRISTANO AG, CASANOVA-ESCALONA L, TORRES A, RODRIGUEZ MA: Macrophage activation syndrome in a patient with systemic onset rheumatoid arthritis: Rescue with intravenous immunoglobulin therapy. Journal of Clinical Rheumatology (United States ) 9:4 253-258, 2003
Abstract: We report the case of a 7-year-old boy with systemic onset juvenile rheumatoid arthritis (JRA) who developed macrophage activation syndrome during a period of relative remission. He presented with high-grade fever, enlarged lymph nodes, acute hepatitis, severe pancytopenia, and evidence of disseminated intravascular coagulopathy. He became severely ill, with persistent mucosal bleeding, hypovolemia, and vascular instability, and he was admitted to the intensive care unit. No response was observed to treatment with high-dose intravenous corticosteroids and blood product replacements, but the patient fully recovered after the completion of two pulses of intravenous immunoglobulin. This treatment modality may be an alternative to anti-TNF-alpha and cyclosporine A for a prompt response in JRA patients with this potentially fatal condition - TSITSAMI E, BOZZOLA E, MAGNI-MANZONI S, VIOLA S, PISTORIO A, RUPERTO N, MARTINI A, RAVELLI A: Positive family history of psoriasis does not affect the clinical expression and course of juvenile idiopathic arthritis patients with oligoarthritis. Arthritis Rheum 49:4 488-493, 2003
Organism: IRCCS Policlinico S Matteo, Universita di Pavia, Pavia, ItalyFAU - Tsitsami, Elena
Abstract: OBJECTIVE: In the 1997 revision of the International League of Associations for Rheumatology (ILAR) criteria for juvenile idiopathic arthritis (JIA), a family history of psoriasis is an exclusion for the oligoarthritis category. We investigated whether psoriasis in a first or second degree relative influences the clinical expression and course of JIA patients with oligoarthritis. METHODS: In a cross-sectional study, consecutive oligoarticular-onset JIA patients were investigated. Clinical evaluations included confirmation of a family history of psoriasis and assessment of nail abnormalities, dactylitis, psoriatic rash, variables of JIA activity, and laboratory indicators of inflammation. Retrospective assessments included sex, onset age, disease duration, antinuclear antibodies, HLA-B27, uveitis, ocular complications, second-line therapies, intraarticular corticosteroid injections, radiographic joint lesions, joint involvement over time, and laboratory investigations at disease presentation and first observation. RESULTS: A total of 185 patients were included. Thirty-three had a positive family history of psoriasis (group 2) and 139 did not (group 1). Thirteen patients fulfilled the ILAR criteria for juvenile psoriatic arthritis (group 3). Patients in groups 1 and 2 were comparable for all parameters, except for a higher frequency of females in group 1 (P = 0.04). As compared with group 2, patients in group 3 were less frequently antinuclear antibody positive and had a more severe arthritis and a different distribution of joint involvement. CONCLUSION: We found close similarities in the clinical features and course among patients with oligoarthritis who had a positive family history for psoriasis and those who did not. These findings argue against the exclusion of the former patients from the oligoarthritis category of JIA - WULFFRAAT NM, BRINKMAN D, FERSTER A, OPPERMAN J, TEN CATE R, WEDDERBURN L, FOSTER H, ABINUN M, PRIEUR AM, HORNEFF G, ZINTL F, DE K, I, KUIS W: Long-term follow-up of autologous stem cell transplantation for refractory juvenile idiopathic arthritis. Bone Marrow Transplant 32 Suppl 1:S61-4.: S61-S64, 2003
Organism: Department of Pediatrics, University Medical Center Utrecht, AB, Utrecht, The NetherlandsFAU - Wulffraat, N M
Abstract: Since 1997, autologous stem cell transplantation (ASCT) had been applied to more than 40 children with polyarticular or systemic juvenile idiopathic arthritis (JIA). For this review, results of the follow-up are available from 25 children with systemic JIA and six with polyarticular JIA that were reported in detail from eight different pediatric European transplant centers. Before ASCT all children had progressive disease despite the use of corticosteroids, methotrexate (MTX) up to 1 mg/kg/week, cyclosporin (2.5 mg/kg/day) and/or anti-TNFalpha therapy. The clinical follow-up of these children ranges from 8 to 60 months (median 33 months) - ZINTL F, SCHILLER I, MUELLER A, AUMANN VOLKER_(A), KENTOUCHE K, FUCHS D, SAUERBREY AXEL_(A), GRUHN B, HAEFER R, HERMANN J, OPPERMANN JOACHIM_(A): Treatment of Children and Adolescents with Juvenile Rheumatoid Arthritis (JRA) and Severe Systemic Lupus Erythematodes (SLE) with High Dose Chemotherapy and Autologous Stem Cell Transplantation (ASCT). Blood 100:11 Abstract, 2002
Abstract: ASCT has been proposed as a new therapeutic option for patients with severe autoimmune disease refractory to conventional treatment. Here, we report three children with a severe form of systemic JRA and two patients with severe systemic lupus erythematodes treated with ASCT in a phase I study. Patients: Three patients (age: 5, 9, 14 yrs) who developed severe systemic JRA with high spiking fever, rashes, hepatomegaly, polyarthritis, morning stiffness, ESR > 100 mm/h, CRP > 100 mg/l were refractory to NSAIDs, MTX, cyclophosphamide, steroids, etanercept after 2.5, 13 and 6 yrs. 2 patients (age: 16, 20 yrs) with SLE had a disease duration of 2.5 / 5.5 yrs with arthritis, carditis, pericarditis, hypertonus, reduced pulmonary capacity, increased Anti-ds DNA titre. SLE was refractory to steroids, MTX, IVIG, CsA and cyclophosphamide (total doses: TPN 340: 14,2 g/m2; TPN 373: 6,2 g/m2). TPN 373 had a WHO class IV glomerulonephritis with a creatinine clearance of 52 ml/min nonresponsive to i.v. cyclophosphamide. Stem cell harvest: After a priming dose of cyclophosphamide (2-3 g/m2) and mobilization with G-CSF (10 mug/kg/day) peripheral blood stem cells were collected using of a Cobe separator. Using a Clinimacs device, CD34-positive selection was performed yielding a final CD34+ -cell amount of 4.2 - 11.9 x 106/kg contaminated with zero to 3.2 x 104/kg CD3+ lymphocytes, respectively. Stem cells were stored in liquid nitrogen. Conditioning regimen: Fludarabine (30 mg/m2): days -7 and -6; cyclophosphamide (50 mg/kg): days -5 to -2; ATG (5 -10 mg/kg): days -6 to -2; methylprednisolone (1g/m2): days -4 to -2. On day 0, the frozen CD34+ cells were thawed and infused. Results: All drugs but prednisolone were stopped before ASCT. Prednisolone was tapered and stopped 2 months after transplant. The conditioning of the patients with cyclophosphamide and G-CSF for CD34+ mobilisation was well tolerated without symptoms of reactivation of rheumatic arthritis and SLE. Rapid engraftment of neutrophils > 1.0 GPT/l: days +10 to +13; platelets > 20 GPT/l: days +6 to +19. Lymphocytes showed a tendency of normalisation during 5 months posttransplant in patients with JRA. One patient with SLE acquired on day + 45 EBV infection with LPD which was treated successfully with ganciclovir, cidofovir and rituximab. Patients were discharged from hospital on day + 24 to + 53 and remained free from active JRA and SLE with no immunosuppressive medication for 4, 17, 19, 29 and 29 months, respectively. CHAQ score showed a clear improvement at evaluation 6-12 months after ASCT. The SLEDAI scores decreased continuously (TPN 340: day +365: 0; TPN 373: day +115: 4). After a traumatic injury one patient with JRA developed a gonarthritis 17 months after ASCT without symptoms of her initial disease as spiking fever, rash, morning stiffness. Conclusion: ASCT is a possible new approach that offers hope to such patients