Bibliography October 2000- MELIKOGLU M, OZDOGAN H, KORKMAZ C, KASAPCOPUR O, ARISOY N, AKKUS S, FRESKO Z, YAZICI H: A survey of phenotype II in familial mediterranean fever [In Process Citation]. Ann.Rheum Dis. 2000, 59:910-913.
Organism:Department of Rheumatology, Cerrahpasa Medical School, University of Istanbul, Istanbul, Turkey
Abstract: OBJECTIVE: Phenotype II in familial Mediterranean fever (FMF) is the onset of amyloidosis before the onset of FMF with its typical attacks, or as an isolated finding in a member of an FMF family. Its presence was investigated by looking for proteinuria among the asymptomatic relatives of patients with FMF complicated by amyloidosis and among the asymptomatic relatives of patients with juvenile chronic arthritis (JCA) complicated by amyloidosis, used as controls. METHODS: The relatives of the index patients (13 with FMF and amyloidosis) and controls (6 with JCA and amyloidosis) were screened for proteinuria. Rectal biopsies were performed when proteinuria was significant (>/=300 mg/d). RESULTS: 461 relatives were screened in the FMF group and 269 among the controls. Two of the FMF relatives and one JCA relative had no symptoms of FMF but had significant proteinuria. Rectal biopsy for amyloidosis was negative in all instances of significant proteinuria. CONCLUSION: Phenotype II is uncommon among the relatives of patients with FMF and amyloidosis
Internet : PM:0011053071 - PRAHALAD S, RYAN MH, SHEAR ES, THOMPSON SD, GIANNINI EH, GLASS DN: Juvenile rheumatoid arthritis: Linkage to HLA demonstrated by allele sharing in affected sibpairs. Arthritis And Rheumatism 2000, 43:2335-2338.
Organism:Dr. D.N. Glass, Division of Rheumatology, Pavilion 2-129, Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229-3039
Abstract: Objective. To test for linkage between the HLA region and juvenile rheumatoid arthritis (JRA), with stratification by onset and course types, in a cohort of affected sibling pairs (ASPs). Methods. Eighty pairs of siblings with JRA who were registered with the Research Registry for JRA ASPs (sponsored by the National Institute of Arthritis and Musculoskeletal and Skin Diseases) were typed for HLA-DR. The observed ratio of sharing of none, one, or both parental DR alleles was compared against the expected ratio of 1:2:1 by goodness-of-fit chi-square tests. A group of 265 unrelated control subjects served as a comparison population for HLA-DR allele frequencies among patients, by Fisher's exact test. Results. Overall, there was excess sharing of 2 DR alleles among ASPs with JRA. The observed ratio of sharing 0, 1, or 2 DR alleles was 8:40:32, instead of the expected ratio of 20:40:20 (P < 0.001). When stratified by JRA onset type, excess allele sharing was demonstrated among ASPs who were concordant for onset type (P = 0.002). This was true for both pauciarticular and polyarticular onset. When stratified by disease course, excess allele sharing was also demonstrated among ASPs who were concordant for disease course (P <0.001). This was true for both the pauciarticular and the polyarticular course. Among the 32 ASPs who shared two DR alleles, 5 pairs had both DR8 and DR11, which was significantly more frequent (P < 0.0001) than the incidence in the control group (n = 0). Conclusion. This study of an independent cohort of multiplex families confirms the previously reported linkage between pauciarticular JRA and the HLA-DR region that was identified using a different analytic method in a cohort of simplex families. Additionally, this study establishes evidence for linkage between polyarticular JRA and the HLA-DR region - PRIEUR A-M, QUARTIER P: Comparative tolerability of treatments for juvenile idiopathic arthritis. BioDrugs 2000, 14:159-183.
Organism:Dr. A.-M. Prieur, Unite Immunohemat./Rhumatol. Pediat., Universite Paris V, Hopital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15
Abstract: Juvenile idiopathic arthritis (JIA) includes several forms of chronic arthritis in children. Treatments are chosen according to the type and severity of the disease. Nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids remain the mainstays of therapy. Traditional slower acting anti-rheumatic drugs, such as gold therapy, penicillamine, sulfasalazine, tiopronin and hydroxychloroquine, are usually poorly active in children. In addition, adverse effects are common, including severe macrophage activation syndrome with gold therapy or sulfasalazine. Low dose, once weekly methotrexate has emerged as the therapeutic agent of choice for children who fail to respond adequately to the administration of an NSAID, especially in those with the extended oligoarticular subtype of the disease. Other immunosuppressive agents, such as cyclosporin, are sometimes combined with methotrexate. In recent years, novel treatments have been developed. Autologous hematopoietic stem cell transplantation is effective in a number of children with severe JIA, whose disease has been refractory to conventional therapy. However, only short term follow-up data are currently available for this novel therapy. In addition, severe infections complicated by macrophage activation syndrome and death have been reported. Finally, anti-tumour necrosis factor-alpha therapy has shown efficacy in more than two-thirds of children with JIA and polyarthritis, and other cytokine inhibitors may be soon available
Internet : anne-marie.prieur@nck.ap-hop-paris.fr - RERON E, MUSZYNSKI P, PRZEKLASA R, ZAJDEL K: Middle ear functional activity in juvenile chronic arthritis: initial information. New Medicine 1999, 3:59-60.
Organism:E. Reron, Audiometric Laboratory, ENT Department, Jagiellonian University Hospital, Cracow
Abstract: Intraphalangeal joints are synovial joints with a cartilaginous joint surface, and as such they can be affected by rheumatoid changes, similar to these observed in other articulations in the body. The aim of our study was a comprehensive audiometric assessment of middle ear functional activity and hearing acuity in 38 children aged 4-16 years with juvenile chronic arthritis (JCA). The evaluation of the hearing organ was performed using pure tone audiometry (PTA), verbal audiometry, tympanometry, high frequency audiometry (HFA) and evoked otoacousic emissions (EOAEs). The initial analysis of these tests revealed abnormal tympanograms in the majority of children with JCA, which indicate diminished compliance in organs transmitting acoustic energy. These pathological changes are not sufficient to cause a conductive hearing loss but they can markedly reduce the defensive mechanisms of the middle ear - SPENCER-GREEN G: Etanercept (Enbrel): update on therapeutic use [In Process Citation]. Ann.Rheum Dis. 2000, 59 Suppl 1:I46-I49
Organism:Immunex Corporation, 51 University Street, Seattle, WA 98101, USA
Abstract: Tumour necrosis factor (TNF) is an important inflammatory disease mediator in a wide spectrum of articular diseases, including adult and juvenile rheumatoid arthritis (RA, JRA). Etanercept (Enbrel), approved in the United States and in Europe for use in patients with RA and JRA, is an effective inhibitor of TNF that has been shown to provide rapid and sustained improvement in both of these diseases. Long term studies continue to show that etanercept controls signs and symptoms of RA and JRA with no change in rate or type of adverse event over time. To demonstrate that etanercept is effective as first line treatment for patients with early active RA who have not been previously treated with methotrexate, and to examine the effect of etanercept on radiographic progression, a double blind, placebo controlled study was recently conducted, comparing etanercept with methotrexate (median dose 20 mg per week). Both etanercept 25 mg twice weekly and rapidly escalated methotrexate were effective in reducing the signs and symptoms of RA, and etanercept was significantly better than methotrexate in slowing the rate of radiographic erosions. In patients with severe psoriatic arthritis (PsA), a double blind, placebo controlled study demonstrated that etanercept was also effective in reducing disease activity in PsA. Etanercept has been well tolerated in all of these clinical trials and offers an important new treatment option to patients with inflammatory articular diseases
Internet : PM:0011053088 - VERROTTI A, CIERI F, PELLICCIA P, MORGESE G, CHIARELLI F: Lack of association between antiphospholipid antibodies and migraine in children [In Process Citation]. Int.J Clin.Lab Res 2000, 30:109-111.
Organism:Department of Pediatrics, University of Chieti, Italy
Abstract: Anticardiolipin antibodies are found frequently in those suffering from migraine, but it is not clear if this association is real or coincidental. Moreover, there are no data on the prevalence of anticardiolipin antibodies in children. In this study, 40 patients were divided into two groups according to the type of migraine: group I included 22 cases (15 females and 7 males, mean age+/-SD 13.7+/-8.9 years) suffering from migraine with and without aura; group II consisted of 18 children (10 females and 8 males, age 14.7+/-6.9 years) having migraine with prolonged aura or migrainous infarction, also called complicated migraine. We studied two groups of children as controls: a group of 35 children (25 females and 10 males, mean age 13.9+/-7.1 years) with juvenile chronic arthritis (group III) and a group of 40 healthy sex- and age-matched children who did not suffer from migraine or any other neurological disease (group IV). No statistically significant differences in levels of anticardiolipin antibodies were found between group I and II and controls. Our data demonstrate that, in children with migraine, anticardiolipin antibodies are not more frequent than in healthy controls, and suggest that anticardiolipin antibodies are not implicated in the pathogenesis of migraine
Internet : PM:0011043505 - WISE CA, BENNETT LB, PASCUAL V, GILLUM JD, BOWCOCK AM: Localization of a gene for familial recurrent arthritis. Arthritis Rheum 2000, 43:2041-2045.
Organism:Research Department, Texas Scottish Rite Hospital for Children, Dallas 75219, USA
Abstract: OBJECTIVE: To localize the gene for familial recurrent arthritis via a genome-wide linkage scan in an extended kindred with the disease. METHODS: A 3-generation family in which 9 members were diagnosed with juvenile idiopathic arthritis (JIA) was ascertained. In this family the disease was of very early onset and included episodic inflammation leading to eventual destruction of joints, muscle, and skin. We treated this disorder as a distinct clinical entity that we have named "familial recurrent arthritis." A genome-wide linkage scan with polymorphic microsatellites at 10-15-cM resolution was initiated. RESULTS: The genome-wide scan generated a maximum 2-point logarithm of odds score with D15S211 (Zmax = 3.27 at thetamax = 0.0010). Haplotype reconstruction defined a candidate region of approximately 20 cM flanked proximally by D15S983 and distally by D15S127 on human chromosome 15. CONCLUSION: A gene for familial recurrent arthritis was localized to 15q22-24, as a result of a genome-wide linkage scan in a large, multiply affected kindred. Identification of the altered gene will provide insights into the pathogenesis of autoimmune joint destruction that is reminiscent of JIA
Internet : PM:0011014354 - YOTSUMOTO S, TAKAHASHI Y, TAKEI S, SHIMADA S, MIYATA K, KANZAKI T: Early onset sarcoidosis masquerading as juvenile rheumatoid arthritis. J Am.Acad.Dermatol. 2000, 43:969-971.
Organism:Departments of Dermatology and Pediatrics, Faculty of Medicine, Kagoshima University; Department of Pediatrics and Pediatric Allergy, Odate Municipal Hospital
Abstract: Symptoms of early onset sarcoidosis characterized by skin eruptions, arthritis, and uveitis mimic those of systemic type juvenile rheumatoid arthritis (JRA). We report 2 Japanese patients with early onset sarcoidosis, both of whom were initially diagnosed and treated as having JRA. Intermittent fever and synovial swelling may mask sarcoidosis in children less than 4 years old
Internet : PM:0011044836