Bibliography March 2002- AVCIN T, CIMAZ R, MERONI PL: Recent advances in antiphospholipid antibodies and antiphospholipid syndromes in pediatric populations. Lupus 2002, 11:4-10.
Organism:IRCCS Istituto Auxologico Italiano, Department of Internal Medicine, University of Milan, Via L. Arlosto 13, 20145, Milan Italy^E-Mail: pierluigi.meroni@unimi.it
Abstract: In recent years, antiphospholipid antibodies (aPL) and their associated clinical features have been recognized increasingly in various pediatric autoimmune and non-autoimmune diseases. Pathogenic mechanisms involved in pediatric antiphospholipid syndrome (APS) appear to be the same as in adults. However, since pediatric patients do not have prothrombotic risk factors present in adults, there clearly are differences in the spectrum of clinical findings. The frequency of aPL-related thrombotic events is generally low in pediatric populations. On the other hand, various commonly acquired infections are likely to be responsible for higher percentage of non-pathogenic and transient aPL in childhood. Such points have to be considered in clinical judgment of elevated aPL in children. In this review we summarize the recent data on the prevalence and clinical significance of aPL in neonates, children and adolescents - AVCIN T, AMBROZIC A, BOZIC B, ACCETTO M, KVEDER T, ROZMAN B: Estimation of anticardiolipin antibodies, anti-BETASUB2 glycoprotein I antibodies and lupus anticoagulant in a prospective longitudinal study of children with juvenile idiopathic arthritis. Clinical And Experimental Rheumatology 2002, 20:101-108.
Organism:Dr. T. Avcin, Division of Allergology, Rheumatology and Clinical Immunology, University Medical Center, Vrazov trg 1, SI-1000 Ljubljana
Abstract: Objective. Anticardiolipin antibodies (aCL) have been frequently detected in juvenile idiopathic arthritis (JIA), but have not been associated with disease activity or clinical features of the antiphospholipid syndrome (APS). Our aim was to determine aCL and anti-betaSUB2 glycoprotein I (anti-betaSUB2GPI) antibody levels and lupus anticoagulant (LA) in serial samples from children with JIA and to investigate the clinical significance of these antibodies. Methods. The values of aCL, anti-betaSUB2GPI and LA were prospectively followed in 28 children with JIA from disease onset. aCL and anti-betaSUB2GPI were assayed by an ELISA method. Two monoclonal betaSUB2GPI-dependent aCL (HCAL and EY2C9) were used as calibrators. LA was determined by a modified dilute Russell viper venom time test. Results. Thirteen (46.4%) children with JIA were already positive for aCL at their first referral to our center. During the follow-up, the frequency of aCL decreased from 46.4% to 28.6%; however, it remained significantly higher compared with healthy children. In contrast, for anti-betaSUB2GPI the difference in the frequency between the children with JIA and healthy children was not statistically significant. Serial determination of aPL levels in JIA patients revealed frequent fluctuations. Positive aCL persisted over time in 6 (21.4%) children with JIA, 6 (21.4%) children were initially positive for aCL, but became later negative, and 3 (10.7%) children were initially negative for aCL and became later positive. Persistently positive anti-betaSUB2GPI were observed during the follow-up only in one patient, while none of the patients was persistently positive for LA. No association between aCL, anti-betaSUB2GPI or LA and disease activity could be established. No patient with positive aCL, anti-betaSUB2GPI or LA showed any clinical feature of APS. Conclusion. The discrepancy between the presence of aCL and anti-betaSUB2GPI might indicate that the production of aCL in JIA is associated with an infectious trigger. Furthermore, the low frequency of anti-betaSUB2GPI and LA could explain the limited prothrombotic potential of aPL observed in JIA. However, we found a distinct group of JIA patients with persistently positive aCL, the clinical implications of which are at the present time unknown
Internet : tadej.avcin@siol.net - BULTINK IEM, LEMS WF, DIJKMANS BAC, VAN SOESBERGEN RM, LINDEMAN J: Severe aortic regurgitation in RF positive polyarticular JIA. Annals of the Rheumatic Diseases 2002, 61:282-283.
Organism:Department of Rheumatology, Slotervaart Hospital, Louwesweg 6, 1066 EC, Amsterdam Netherlands^E-Mail: iem_bultink@hotmail.com - CZAKO M, RIEGEL M, MORAVA E, SCHINZEL A, KOSZTOLANYI G: Patient with rheumatoid arthritis and MCA/MR syndrome due to unbalanced der(18) transmission of a paternal translocation t(18;20)(p11.1;p11.1). Am.J.Med.Genet. 2002, 108:226-228.
Organism:Dr. A. Schinzel, Institut fur Medizinische Genetik, Universitat Zurich, Raemistr. 74, CH-8001 Zurich
Abstract: A girl with psychomotor retardation and a pattern of minor anomalies was found to have a slightly enlarged short arm of chromosome 18 by conventional GTG-banded chromosome examination. The 18p+chromosome has also been found in the father. FISH studies using chromosome 18-and chromosome 20-specific painting probes confirmed a reciprocal whole arm translocation between chromosomes 18 and 20 in the father, resulting in monosomy of the short arm of chromosome 18 and trisomy of the short arm chromosome 20 in the patient. FISH analysis using a chromosome 18 alpha-satellite-specific probe showed a reduced signal intensity. The patient presented with a flat, oval face, upslanting palpebral fissures, periorbital fullness, and mental retardation; she also had chronic diarrhea with milk protein intolerance and juvenile rheumatoid arthritis at age 5 years. Juvenile rheumatoid arthritis, like several other immunologic disorders, has occasionally been reported in patients with deletion of 18p, and thus most likely loss of a gene or genes on 18p is responsible for various immunologic disorders occurring in these patients. (c) 2002 Wiley-Liss, Inc
Internet : schinzel@medgen.unizh.ch - CZAKO M, RIEGEL M, MORAVA E, SCHINZEL A, KOSZTOLANYI G: Patient with rheumatoid arthritis and MCA/MR syndrome due to unbalanced der(18) transmission of a paternal translocation t(18;20)(p11.1;p11.1). Am.J.Med.Genet. 2002, 108:226-228.
Organism:Institut fur Medizinische Genetik der Universitat Zurich, Raemistr. 74, CH-8001, Zurich Switzerland^E-Mail: schinzel@medgen.unizh.ch
Abstract: A girl with psychomotor retardation and a pattern of minor anomalies was found to have a slightly enlarged short arm of chromosome 18 by conventional GTG-banded chromosome examination. The 18p+chromosome has also been found in the father. FISH studies using chromosome 18-and chromosome 20-specific painting probes confirmed a reciprocal whole arm translocation between chromosomes 18 and 20 in the father, resulting in monosomy of the short arm of chromosome 18 and trisomy of the short arm chromosome 20 in the patient. FISH analysis using a chromosome 18 alpha-satellite-specific probe showed a reduced signal intensity. The patient presented with a flat, oval face, upslanting palpebral fissures, periorbital fullness, and mental retardation; she also had chronic diarrhea with milk protein intolerance and juvenile rheumatoid arthritis at age 5 years. Juvenile rheumatoid arthritis, like several other immunologic disorders, has occasionally been reported in patients with deletion of 18p, and thus most likely loss of a gene or genes on 18p is responsible for various immunologic disorders occurring in these patients - FRIEDMAN S, GRUBER MA: Ultrasonography of the hip in the evaluation of children with seronegative juvenile rheumatoid arthritis. Rinsho Ganka 2002, 29:629-632.
Organism:Dr. S. Friedman, Office of Academic Affairs, School of Medicine, L-4 Health Science Center, Stony Brook, NY 11790
Abstract: Objective. To find an objective measure of hip joint effusion with ultrasound (US) in patients with juvenile rheumatoid arthritis (JRA). Methods. The hip joints of 24 children with JRA were evaluated with US. All patients were negative for rheumatoid factor and antinuclear antibodies. Patients with unilateral or bilateral hip pain, swelling, or limitation of range of motion were included. In each hip, the distance from the femoral neck to joint capsule was measured. Values were compared to measurements in a control group of 24 children with no history of hip joint or rheumatic disease,. Statistically significant differences between the 2 groups were analyzed by t test. Two standard deviations above the control group mean was used as the standard for an effusion. Results. There was a statistically significant difference in US joint space between the children with JRA and the control subjects (p < 0.001). The mean in the control group was 0.43 cm and the mean in the JRA group was 0.60 cm. A distance of 0.59 cm from femoral neck to joint capsule was determined to be consistent with an effusion. Using this standard, 71% of the children with JRA had effusion in at least one hip, and 25% had effusion bilaterally. No control subjects had measurements above this level. Conclusion. Ultrasonography is effective in the evaluation of hip joint involvement in patients with JRA, and may be useful in facilitating the diagnosis, classification, and followup of this illness - GYLYS-MORIN VM, GRAHAM TB, BLEBEA JS, DARDZINSKI BJ, LAOR T, JOHNSON ND, OESTREICH AE, PASSO MH: Knee in early juvenile rheumatoid arthritis: MR imaging findings. Radiology 2001, 220:696-706.
Organism:Department of Radiology, Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229-3039 USA^E-Mail: gylys.morin@chmcc.org
Abstract: PURPOSE: To determine the magnetic resonance (MR) imaging findings in the knee in early juvenile rheumatoid arthritis. MATERIALS AND METHODS: MR imaging (1.5 T) was performed in the more symptomatic knee in 30 children with juvenile rheumatoid arthritis with a symptom duration 1 year or less. Conventional, fast spin-echo, three-dimensional gradient-echo, and gadolinium-enhanced T1-weighted images were assessed. Two radiologists independently read the images, and a third resolved disagreements. These images were compared with knee radiographs in 27 children. RESULTS: Mean maximal synovial thickness was 4.8 mm+-2.4 (SD). Mean synovial volume was 15.4 mL+-10.8. Suprapatellar joint effusions were seen in 26 (87%) of 30 knees, meniscal hypoplasia in 11 (37%) of 30 knees, and abnormal epiphyseal marrow in eight (27%) of 30 knees. Three knees had articular cartilage contour irregularity, fissures, and/or thinning. One knee had a bone erosion. Knee radiographs showed suprapatellar fullness in 78% of the knees, joint space narrowing in one knee, and no bone abnormalities. CONCLUSION: Synovial hypertrophy and joint effusions are the most frequent MR imaging findings of knees in early juvenile rheumatoid arthritis. Early in the disease, radiographically occult cartilage and bone erosions are uncommonly seen at MR imaging. The potential relationship of synovitis to cartilage abnormalities deserves further study - HACHULLA E: [In Process Citation]. Rev.Prat. 2002, 52:160-166.
Organism:Service de medecine interne Centre hospitalier regional et universitaire Hopital Claude Huriez 59037 Lille ehachulla@chru-lillefr
Abstract: Intermittent fever has a wide variety of causes such as infectious, cancers, or inflammatory disease. Intermittent fever is sometimes a diagnostic challenge when fever appears as the first and isolated sign of the disease. Adult onset Still disease and juvenile chronic arthritis are mainly the most common cause of intermittent inflammatory fever. Some frequent diseases gives intermittent fever in few cases like ankylosing spondylitis, pulmonary embolism, sarcoidosis or Crohn's disease. Some rare inflammatory disease gives typical intermittent fever like genetic periodic fever. Other rare diseases give sometimes intermittent fever like vasculitis, polychondritis, Castleman disease, etc. Drug fever and factitious fever are other classical causes of intermittent fever. Diagnosis of inflammatory intermittent fever is frequently based on the clinical course but some biological tests and computerized tomographic scans are worthwhile tools. Follow-up of undiagnosed cases is needed
Internet : PM:11915560 - HAEFNER R: [Juvenile idiopathic arthritis: The new nomenclature and classification of chronic childhood arthritis.]
<ORIGINAL> Juvenile idiopathische Arthritis: Die neue Nomenklatur und Klassifikation der chronischen Arthritis im Kindesalter. Aktuelle Rheumatologie 2002, 27:18-21.
Organism:Rheumakinderklinik, Gehfeldstrasse 24, 82467, Garmisch-Partenkirchen Germany^E-Mail: info.rh-kkl@rummelsberg.de
Abstract: A Classification Taskforce of the Pediatric Standing Committee of the ILAR has been commissioned to establish an internationally accepted new nomenclature and classification for chronic arthritis of childhood. The Committee's suggestions were published in 1998. The term "juvenile idiopathic arthritis" was adopted as an umbrella term, and the disease has been divided into 7 categories: 1) systemic arthritis; 2) oligoarthritis; 3) RF-negative polyarthritis; 4) RF-positive polyarthritis; 5) psoriatic arthritis; 6) enthesitis-related arthritis; 7) other types of arthritis. The new classification shall replace the diagnoses juvenile chronic and rheumatoid arthritis with their subgroups. Evaluation of the new system by patient population studies from different European Centers revealed several critical points which require further revision of the new criteria. All studies confirmed that about 20% of patients had to be grouped into the unspecific 7th category "other arthritis", because they fulfill criteria for none or for more than one of the 6 specific categories. Especially a family history of psoriasis or HLA B 27 associated disease has been overestimated, and excludes patients from one of the most frequent categories, namely, oligoarthritis. Many patients with psoriatic arthritis or enthesitis associated arthritis cannot be classified as such, because they also fulfill criteria for the categories oligo- or polyarthritis. A weak point has been adopted from the former classifications. It concerns the distinction between oligo- and polyarthritis if 5 joints are involved. This means that a large number of patients appear in the category RF-negative polyarthritis, who have more in common with the oligoarticular category, because they have an asymmetric joint involvement of mostly 5 to 8 joints, high frequency of antinuclear antibodies and the risk for chronic iridocyclitis. Last but not least patients with juvenile spondylarthropathy are not represented as a unique patient population in the ILAR classification, but appear in different categories - enthesitis related arthritis, psoriatic arthritis, even in the oligoarthritis or RF-negative polyarthritis and often in the category "other arthritis". The efforts for an internationally accepted classification are appreciated. The presented suggestions, however, require further revision to meet the demands for scientific studies as well as for practical use - HAFNER R: Juvenile idiopathic arthritis - The new nomenclature and classification of chronic childhood arthritis. Aktuelle Rheumatologie 2002, 27:18-21.
Organism:Dr. R. Hafner, Rheumakinderklinik, Gehfeldstrasse 24, 82467 Garmisch-Partenkirchen
Abstract: A Classification Taskforce of the Pediatric Standing Committee of the ILAR has been commissioned to establish an internationally accepted new nomenclature and classification for chronic arthritis of childhood. The Committee's suggestions were published in 1998. The term "juvenile idiopathic arthritis" was adopted as an umbrella term, and the disease has been divided into 7 categories: 1) systemic arthritis; 2) oligoarthritis; 3) RF-negative polyarthritis; 4) RF-positive polyarthritis; 5) psoriatic arthritis; 6) enthesitis-related arthritis; 7) other types of arthritis. The new classification shall replace the diagnoses juvenile chronic and rheumatoid arthritis with their subgroups. Evaluation of the new system by patient population studies from different European Centers revealed several critical points which require further revision of the new criteria. All studies confirmed that about 20% of patients had to be grouped into the unspecific 7th category "other arthritis", because they fulfill criteria for none or for more than one of the 6 specific categories. Especially a family history of psoriasis or HLA B 27 associated disease has been overestimated, and excludes patients from one of the most frequent categories, namely, oligoarthritis. Many patients with psoriatic arthritis or enthesitis associated arthritis cannot be classified as such, because they also fulfill criteria for the categories oligo- or polyarthritis. Aweak point has been adopted from the former classifications. It concerns the distinction between oligo- and polyarthritis if 5 joints are involved. This means that a large number of patients appear in the category RF-negative polyarthritis, who have more in common with the oligoarticular category, because they have an asymmetric joint involvement of mostly 5 to 8 joints, high frequency of antinuclear antibodies and the risk for chronic iridocyclitis. Last but not least patients with juvenile spondylarthropathy are not represented as a unique patient population in the ILAR classification, but appear in different categories - enthesitis related arthritis, psoriatic arthritis, even in the oligoarthritis or RF-negative polyarthritis and often in the category "other arthritis". The efforts for an internationally accepted classification are appreciated. The presented suggestions, however, require further revision to meet the demands for scientific studies as well as for practical use
Internet : info.rh-kkl@rummelsberg.de - KIRWAN JF, SHAH P, KHAW PT: Diode laser cyclophotocoagulation: Role in the management of refractory pediatric glaucomas. Ophthalmology 2002, 109:316-323.
Organism:Paediatric Glaucoma Clinic and Wound Healing Research Unit, Moorfields Eye Hospital and Institute of Ophthalmology, 11-43 Bath Street, London, EC1V 9EL UK
Abstract: Purpose: To report the efficacy and complications of diode laser cyclophotocoagulation (cyclodiode) in the management of refractory pediatric glaucomas. Design: Noncomparative interventional case series. Participants: Pediatric patients with uncontrolled glaucoma. Seventy-seven eyes of 61 patients underwent cyclodiode. Mean age was 7.4 years (range, 0.4-17 years). Main Outcome Measures: Intraocular pressure (IOP), visual acuity, complications. Results: Diagnoses included aphakic glaucoma, congenital glaucoma, juvenile chronic arthritis, aniridia, anterior segment dysgenesis, and Sturge-Weber syndrome. Sixty percent of eyes were aphakic, and 64% had undergone at least one previous surgical procedure for glaucoma. Patients underwent a mean of 2.3 treatment sessions per eye (maximum, 8 sessions). Mean pretreatment IOP was 32.0 mmHg. After one treatment session, 62% had a clinically useful reduction in IOP (<22 mmHg or by 30%), but this had fallen to 37% by 12 months. With repeat cyclodiode, 72% had a clinically useful reduction in IOP for a year or more (mean, 8.4-month interval between treatments). Aphakic eyes had a more sustained IOP reduction (P<0.01 log rank test). Of treatment failures, 13% had no useful IOP response, and three eyes developed subsequent retinal detachment and loss of vision. No other eyes lost vision because of cyclodiode-related complications. In 5.5% of the treatment sessions there was a significant posttreatment inflammatory episode. Cyclodiode treatment did not enable a reduction in the number of medications. Conclusions: With repeated treatment, cyclodiode can provide effective control of IOP. However, the success rate is lower than with adults, and younger eyes may recover from treatment more rapidly. Although response may be temporary, cyclodiode has a lower rate of severe adverse effects than surgical modalities and has roles as a temporizing measure, as an adjunct to surgery, or in managing selected patients in whom surgery is undesirable because of a high risk of surgical complications - KOTANIEMI K, KOTANIEMI A, SAVOLAINEN A: Uveitis as a marker of active arthritis in 372 patients with juvenile idiopathic seronegative oligoarthritis or polyarthritis. Clinical And Experimental Rheumatology 2002, 20:109-112.
Organism:Dr. K. Kotaniemi, Rheumatism Foundation Hospital, 18120 Heinola
Abstract: Objective. To compare the activity of arthritis in children with recently diagnosed sero-negative oligoarthritis or polyarthritis with or without uveitis. Methods. The study covered 372 JIA children with recently diagnosed seronegative oligo-arthritis or polyarthritis. The mean prospective follow-up period was 4.5 years. Asymptomatic anterior uveitis was found in 96 cases. The activity of arthritis in all 372 patients was assessed clinically and by laboratory parameters. Results. The erythrocyte sedimentation rate was significantly higher (p = 0.001) at the diagnosis of arthritis and at the end of the follow-up (p = 0.02) in the 96 JIA patients with uveitis than in the 276 JIA patients without uveitis. The hemoglobin value was significantly lower (p = 0.008) at the diagnosis of arthritis in patients with uveitis, but not at the end of the follow-up. The number of inflamed joints was significantly greater at the end of the follow-up in patients with persistent polyarthritis and uveitis (p = 0.01) compared to those polyarthritis patients without uveitis. Patients with uveitis were significantly more often treated with oral prednisolone (p < 0.001), glucocorticoid joint injections (p < 0.001), and with methotrexate (p = 0.003) compared to patients without uveitis. Clinical remission of arthritis was achieved significantly less frequently in patients with uveitis than in patients without uveitis (21% versus 42%, p < 0.001). Conclusion. The inflammatory activity of arthritis seems to be increased in patients with seronegative oligo- or polyarthritis and uveitis compared to those without uveitis - KURAHARA D, TOKUDA A, GRANDINETTI A, NAJITA J, HO C, YAMAMOTO K, REDDY D, V, MACPHERSON K, IWAMURO M, YAMAGA K: Ethnic differences in risk for pediatric rheumatic illness in a culturally diverse population. Rinsho Ganka 2002, 29:379-383.
Organism:734-1319 Punahou Street, Honolulu, HI, 96826 USA^E-Mail: davidk@kapiolani.org
Abstract: Objective. To analyze the differences of occurrence of pediatric rheumatic disease among various ethnic groups in a culturally diverse isolated geographic area. Methods. A retrospective study of pediatric rheumatic diseases in a multiethnic area during a 6 year period. Results. A group of 922 patients was categorized based on predominant ethnicity, and their risk of having acute rheumatic fever (ARF), juvenile rheumatoid arthritis (JRA), and systemic lupus erythematosus (SLE) was studied. Odds ratios (OR) were computed for each illness with Caucasians as the reference group. Results indicated that Polynesians were overrepresented among patients with ARF, having elevated OR that were significantly different from Caucasians (22.5-120.7, p < 0.0001). For SLE, the highest OR were obtained for Samoans, Filipinos, and Japanese. In contrast, for JRA, Filipinos and Japanese had OR less than one, and no Samoans were diagnosed with JRA, possibly indicating a protective effect against developing JRA. Conclusion. This unique retrospective study examined the ethnic variations of expression of certain rheumatic diseases in an isolated region. Results reveal that certain ethnic groups are at risk for ARF and SLE, but are protected against JRA. These findings suggest investigating possible immunogenetic similarities and differences in these illnesses - LEHMAN THOMAS JA, STRIEGEL KH, ONEL KB: Thalidomide therapy for recalcitrant systemic onset juvenile rheumatoid arthritis. Journal of Pediatrics 2002, 140:125-127.
Organism:Division of Pediatric Rheumatology, Hospital for Special Surgery, Sanford Weill Medical Center, Cornell University, 535 E 70th St, New York, NY, 10021 USA
Abstract: Systemic onset juvenile rheumatoid arthritis unresponsive to nonsteroidal anti-inflammatory drugs may be controlled with corticosteroids, but these drugs have significant side effects. We report 2 steroid-dependent children with systemic onset juvenile rheumatoid arthritis who did not respond to multiple nonsteroidal anti-inflammatory drugs, methotrexate, azathioprine, cyclosporine, and etanercept. Both children had significant improvement with thalidomide therapy - MAENPAA HM, SOINI I, LEHTO MUK, BELT EA: Insufficiency fractures in patients with chronic inflammatory joint diseases. Clinical And Experimental Rheumatology 2002, 20:77-79.
Organism:Dr. H.M. Maenpaa, Rheumatism Foundation Hospital, Department of Orthopaedics, FIN-18120 Heinola
Abstract: Objective: To describe the typical sites of stress fractures in the lower extremities and pelvis in rheumatoid patients (rheumatoid arthritis, juvenile chronic arthritis, psoriatic arthritis, ankylosing spondylitis). Methods: Thirty-three patients with 52 stress fractures [mean age 44 years (range 11-73)] were studied at the authors' institution when they were being treated for their rheumatic diseases. Fourteen patients had RA, 9 JCA, 5 PsoA, and 5 SPA. Stress fractures were detected from patient documents and from series radiographs in suspected cases. In some cases magnetic resonance imaging was also performed. Results: One patient presented with 5 fractures, 2 patients with 4 and 3 fractures, and 7 patients with 2 fractures each. Other patients (n = 19) had only one fracture each. The metatarsal (MT) bones were the most common site of involvement. Twenty-five of the 52 fractures were located on MT I-V. The second and third most common sites were the fibula (n = 13) and tibia (n = 6). All fractures of the lower tibia or fibula were associated with valgus malalignment of the ankle. Conclusion: If a patient with rheumatic disease experiences sudden and unexplained pain localised in the forefoot, above the ankle, below the knee, or in the pelvis, a stress fracture should be suspected. Patients with severe osteoporosis, high-load corticosteroid or methotrexate therapy, or marked joint deformity are at high risk of developing stress fracture
Internet : heikki.maenpaa@scanpoint.fi - MANAL K, LU X, NIEUWENHUIS MK, HELDERS PAUL JM, BUCHANAN TS: Force transmission through the juvenile idiopathic arthritic wrist: A novel approach using a sliding rigid body spring model. Journal of Biomechanics 2002, 35:125-133.
Organism:Center for Biomedical Engineering Research, University of Delaware, 126 Spencer Laboratories, Newark, DE, 19716 USA^E-Mail: buchanan@udel.edu
Abstract: Force transmission across the wrist during a grasping maneuver of the hand was simulated for three children with juvenile idiopathic arthritis (JIA) and for one healthy age-matched child. Joint reaction forces were estimated using a series of springs between articulating bones. This method (i.e., rigid body spring modeling) has proven useful for examining loading profiles for normally aligned wrists. A novel method (i.e., sliding rigid body spring modeling) designed specifically for studying joint reaction forces of the malaligned JIA wrist is presented in this paper. Loading profiles across the wrist for the unimpaired child were similar using both spring modeling methods. However, the traditional fixed-end method failed to converge to a solution for one of the JIA subjects indicating the sliding model may be more suitable for investigating loading profiles of the malaligned wrist. The results of this study suggest that a larger proportion of force is transferred through the ulno-carpal joint of the JIA wrist than for healthy subjects, with a less than normal proportion of force transferred through the radio-carpal joint. In addition, the ulnar directed forces along the shear axis defined in this study were greater for all three JIA children compared to values for the healthy child. These observations are what were hypothesized for an individual with JIA of the wrist - MANZOTTI F, ORSONI JG, ZAVOTA L, CIMINO L, ZOLA E, BONAGURI C: Autoimmune uveitis in children: Clinical correlation between antinuclear antibody positivity and ocular recurrences. Rheumatol.Int. Berlin 2002, 21:127-132.
Organism:Institute of Ophthalmology, University of Parma, Via Gramsci 14, 43100, Parma Italy^E-Mail: jorsoni@unipr.it
Abstract: Objective. The aim of this study was to identify the correlation between antinuclear antibody (ANA) titre and the onset and clinical course of uveitis in children with juvenile idiopathic arthritis (JIA) or without any other systemic autoimmune disease, i.e., idiopathic uveitis (IU). Methods. Twenty-two patients affected by uveitis were examined. Ten had JIA-associated uveitis, 12 had IU. Follow-up ranged from 7 to 101 months. The ANA were titrated three times per year and additionally in case of ocular recurrences. All patients were treated with immunosuppressive drug combination therapy (IDCT). Results. JIA-associated uveitis: ocular recurrences were noted in three ANA-positive patients and in one ANA-negative patient. IU uveitis: ocular recurrences were noted in one ANA-positive and in one ANA-negative patient. No significant rise in ANA titre was noted in either group during uveitis recurrence. Conclusions. (1) ANA had no value in predicting the recurrence of uveitis. (2) IDCT does not influence ANA production - MARTINEZ LJ: Histocompatibility results: HLA and disease. Inmunologia 1999, 18:111-117.
Organism:Inmunologia, Hospital 12 de Octubre, Ctra. Andalucia, s/n, 28047, Madrid Spain - MINDEN K, NIEWERTH M, LISTING J, ZINK A: Health care provision in pediatric rheumatology in Germany - National rheumatologic database. Rinsho Ganka 2002, 29:622-628.
Organism:Dr. K. Minden, Dt. Rheuma-Forschungzentrum Berlin, Schumannstr. 21/22, 10117 Berlin
Abstract: Objective. To describe the health care provision for children and adolescents with chronic arthritides in Germany in 1998. Methods. Data were analyzed from the German pediatric rheumatologic database of the year 1998. It contains clinical and patient questionnaire data for 2488 patients with rheumatic diseases seen at 18 pediatric rheumatology units. Results. A total of 1811 of all patients recorded in the database had chronic arthritides - 931 with juvenile chronic arthritis, 86 with juvenile spondyloarthropathy, and 65 with juvenile psoriatic arthritis were considered in the analysis. These patients seen by pediatric rheumatologists had a median age of 10 years and a median disease duration of 4 years. The majority were being treated at pediatric rheumatology disease centers and at universities. Nonsteroidal antiinflammatory drugs were the most commonly used drugs for all forms of chronic arthritides. Almost half the patients with chronic arthritides received disease modifying antirheumatic drugs, with methotrexate the most frequently prescribed agent. While the majority of patients reported having physiotherapy, low prescription rates were noted for comprehensive measures such as occupational therapy and patient education. Only a few patients showed severe functional limitation, 2% of them being rated in Steinbrocker class III or higher. While the patients' functional limitation correlated with disease activity, neither disease duration nor sex, arthritis subgroup nor time span to the first visit at the rheumatology unit had any relevant influence on functional status. Conclusion. The data reveal the spectrum of patients with chronic arthritides seen by German pediatric rheumatologists, as well as the treatment patterns of their physicians
Internet : minden@drfz.de - MYERS A, CLARK J, FOSTER H: Tuberculosis and treatment with infliximab. New England Journal of Medicine 2002, 346:625
Organism:University of Newcastle upon Tyne, Newcastle upon Tyne, NE2 4HH UK^E-Mail: andrea.myers@ncl.ac.uk - NASONOVA VA, KHALTAEV NG: The bone and joint decade 2000-2010: A multidisciplinary action. Terapevticheskii Arkhiv 2001, 73:5-7.
- OZEN S, ALIKASIFOGLU M, BAKKALOGLU A, DUZOVA A, JAROSOVA K, NEMCOVA D, BESBAS N, VENCOVSKY J, TUNCBILEK E: Tumour necrosis factor alpha Gright arrowA -238 and Gright arrowA -308 polymorphisms in juvenile idiopathic arthritis. Rheumatology (Oxford) 2002, 41:223-227.
Organism:Departments of Paediatric Nephrology and Rheumatology and Genetics, Hacettepe University Faculty of Medicine, Ankara, Turkey, Institute of Rheumatology, Prague and Paediatric Department, Charles University, Prague, Czech Republic
Abstract: OBJECTIVES: To study Gright arrowA -238 and Gright arrowA -308 polymorphisms in the promoter region of the tumour necrosis factor (TNF) alpha gene in patients with juvenile idiopathic arthritis (JIA). We analysed whether there were any associations between these polymorphisms and the type of JIA and/or the clinical course of the disease in two populations. METHODS: The first group consisted of 51 Turkish JIA patients and the second consisted of 159 JIA patients from the Czech Republic. Healthy individuals (93 and 100) from each country served as controls. Subgroups of JIA were defined according to the Durban criteria. The course of the disease was defined on the basis of the physician's global evaluation of disease activity, the swollen and tender joint count and the erythrocyte sedimentation rate. RESULTS: In both JIA cohorts, the distribution of genotypes was not significantly different among the types of JIA. The Gright arrowA -238 polymorphism did not have an effect on the patients' outcome in either group. The Gright arrowA -308 polymorphism was significantly associated with a poor outcome in the Turkish group (P=0.005) but there was no association in the Czech patients. Some features of JIA in Turkish patients differed from those in Czech patients. CONCLUSIONS: Genetic differences may accompany the phenotypic differences found in the Turkish group. Although larger numbers of patients are clearly needed to verify this, we suggest that the Gright arrowA -308 polymorphism may be operative in defining disease outcome in selected groups
Internet : PM:11886974 - PRUSEK W: Diagnostic and therapeutic standards in rheumatic diseases in children. Polski Merkuriusz Lekarski 2002, 12:152-156.
Organism:W. Prusek, Kliniczny Oddzial Pediatryczno, Reumatol. Akad. Medycznej Wroclawiu, pl. 1 Maja 8, 50-043 Wroclaw
Abstract: The rheumatic diseases constitute a significal group of chronic illnesses affecting children of all ages, including adolescens. This chapter provides an overview of rheumatic diseases and medical treatment options. The author describes in detail diagnostic and therapeutic standards proceedings in rheumatic fever, juvenile chronic arthritis, systemic lupus erythematosus and Kawasaki disease. The article contains the newest data from literature about new therapy trials in above mentioned diseases - ROMICKA AM: Classification and clinical picture of juvenile idiopathic arthritis. Polski Merkuriusz Lekarski 2002, 12:157-159.
Organism:A.M. Romicka, Klin. Reumatologii Wieku Rozwojowego, Instytutu Reumatologicznego, ul. Spartanska 1, 02-637 Warszawa
Abstract: In recent years a new nomenclature and classification childhood chronic arthritis has been introduced. The terms juvenile chronic arthritis and juvenile rheumatoid arthritis - have been replaced by juvenile idiopathic arthritis (JIA). The term JIA was adapted to indicate a childhood onset (before the 16 th. birthday) characterized primarily by arthritis lasting for at least 6 weeks, and the cause haven't been found so far. In this paper the diagnosis and clinical course systemic arthritis, polyarthritis and oligoarthritis were characterized. The courrent therapeutic approach to JIA consists a complex menage-ment - as the administration of antiinflammatory, immunosupressive and immunomodulatory drugs - as well as rehabilitation - SCOLA MP, THOMPSON SD, BRUNNER H, I, TSORAS MK, WITTE D, VAN DIJK MA, GROM AA, PASSO MH, GLASS DN: Interferon-gamma:Interleukin 4 ratios and associated type 1 cytokine expression in juvenile rheumatoid arthritis synovial tissue. Rinsho Ganka 2002, 29:369-378.
Organism:Division of Rheumatology, Children's Hospital Medical Center, 3333 Burnet Avenue, Pavilion 2-129, Cincinnati, OH, 45229-3039 USA^E-Mail: glasd0@chmcc.org
Abstract: Objective. To compare synovial tissue cytokine mRNA expression between patients with juvenile rheumatoid arthritis (JRA) and a heterogeneous group of non-autoimmune arthropathies (controls) with respect to type 1/type 2 balance. Methods. Thirty-five JRA (average 9.1 years' disease duration) and 13 control synovial tissues were studied. As a measure of the type 1/type 2 cytokine balance in a subset of the JRA and control tissues, interferon-gamma (IFN-gamma) and interleukin 4 (IL-4) mRNA levels were measured by competitive fragment reverse transcription-polymerase chain reaction. To quantitate additional cytokines relevant to this balance, multiprobe ribonuclease protection assays were employed measuring IL-5, IL-10, IL-13, IL-15, IL-18, and IL-12 (p35 and p40 subunits). Immunohistochemistry was performed on JRA tissues using antibodies specific for IL-15 and IL-18. Results. A higher IFN-gamma:IL-4 ratio (p = 0.034) was found in JRA tissues compared to controls. JRA tissues also displayed higher mRNA levels of IL-12p35 (p = 0.021), IL-15 (p = 0.002), and IL-18 (p = 0.017), but not IL-4 and IL-10. IFN-gamma expression in JRA, but not controls, correlated strongly with IL-12p35 (r = 0.63) and IL-12p40 (r = 0.73) levels. A subset of IL-15+ and IL-18+ cells was detected in JRA synovial tissues, largely within perivascular aggregates. Conclusion. JRA synovial tissue cytokine expression patterns indicate a type 1 bias, even in the later stages of disease. The strong correlation between IFN-gamma and IL-12 in JRA suggests a prominent role for IL-12 in promoting the type 1 bias, while IL-15 and IL-18 may also indirectly increase IFN-gamma expression and further bias the immune response - SLOVAK ML, BEDELL V, POPPLEWELL L, ARBER DA, SCHOCH C, SLATER R: 21q22 balanced chromosome aberrations in therapy-related hematopoietic disorders: Report from an International Workshop. Genes Chromosomes and Cancer 2002, 33:379-394.
Organism:Dr. M.L. Slovak, City of Hope National Medical Center, Department of Cytogenetics, Northwest Building, 1500 East Duarte Road, Duarre, CA 91010
Abstract: The International Workshop on the relationship between prior therapy and balanced chromosome aberrations in therapy-related myelodysplastic syndromes (t-MDS) and therapy-related acute leukemia (t-AL) identified 79 of 511 (15.5%) patients with balanced 21 q22 translocations. Patients were treated for their primary disease, including solid tumors (56%), hematologic malignancy (43%), and juvenile rheumatoid arthritis (single case), by radiation therapy (5 patients), chemotherapy (36 patients), or combined-modality therapy (38 patients). 21 q translocations involved common partner chromosomes in 81% of cases: t(8;21) (n = 44; 56%), t(3;21) (n = 16; 20%), and t(16;21) (n = 4; 5%). Translocations involving 15 other partner chromosomes were also documented with involvement of AMLI(CBFA2/RUNXI), identifying a total of 23 different 21q22/AMLI translocations. The data analysis was carried out on the basis of five subsets of 21 q22 cases, that is, t(8;21) with and without additional aberrations, t(3;21), t(16;21), and other 21 q22 translocations. Dysplastic features were present in all 21 q22 cases. Therapy-related acute myeloid leukemia (t-AML) at presentation was highest in t(8;21) (82%) and lowest in t(3;21) (37.5%) patients. Cumulative drug dose exposure scores for alkylating agents (AAs) and topoisomerase II inhibitors indicated that t(3;21) patients received the most intensive therapy among the five 21 q22 subsets, and the median AA score for patients with secondary chromosome 7 aberrations was double the AA score for the entire 21 q22 group. All five patients who received only radiation therapy had t(8;21) t-AML. The median latency and overall survival (OS) for 21 q22 patients were 39 and 14 months (mo), compared to 26 and 8 mo for 11 q23 patients, 22 and 28 mo for inv(16), 69 and 7 mo for Rare recurring aberrations, and 59 and 7 mo for Unique (nonrecurring) balanced aberration (latency P <= 0.016 for all pairwise comparisons; OS, P <= 0.018 for all pairwise comparisons). The percentages of 21 q22 patients surviving 1 year, 2 years, and 5 years were 58%, 33%, and 18%, respectively. Noticeable differences were observed in median OS between 21q22 patients (n = 7) receiving transplant (BMT) (31 mo) compared to 21 q22 patients who received intensive non-BMT therapy (n = 46) (17 mo); however, this was nonsignificant because of the small sample size (log-rank, P = 0.33). t-MDS/t-AML with balanced 21 q22 aberrations was associated with prior exposure to radiation, epipodophyllotoxins, and anthracyclines, dysplastic morphologic features, multiple partner chromosomes, and longer latency periods when compared to 11 q23 and inv(16) t-MDS/AML Workshop subgroups. In general, patients could be divided into two prognostic risk groups, those with t(8;21) (median OS, 19 mo) and those without t(8;21) (median OS, 7 mo) leukemia (log-rank, P = 0.0007). (c) 2002 Wiley-Liss, Inc
Internet : mslovak@coh.org - SLOVAK ML, BEDELL V, POPPLEWELL L, ARBER DA, SCHOCH C, SLATER R: 21q22 balanced chromosome aberrations in therapy-related hematopoietic disorders: Report from an international workshop. Genes Chromosomes & Cancer 2002, 33:379-394.
Organism:Department of Cytogenetics, City of Hope National Medical Center, 1500 East Duarte Road, Northwest Building Room 2255, Duarte, CA, 91010 USA^E-Mail: mslovak@coh.org
Abstract: The International Workshop on the relationship between prior therapy and balanced chromosome aberrations in therapy-related myelodysplastic syndromes (t-MDS) and therapy-related acute leukemia (t-AL) identified 79 of 511 (15.5%) patients with balanced 21q22 translocations. Patients were treated for their primary disease, including solid tumors (56%), hematologic malignancy (43%), and juvenile rheumatoid arthritis (single case), by radiation therapy (5 patients), chemotherapy (36 patients), or combined-modality therapy (38 patients). 21q translocations involved common partner chromosomes in 81% of cases: t(8;21) (n = 44; 56%), t(3;21) (n = 16; 20%), and t(16;21) (n = 4; 5%). Translocations involving 15 other partner chromosomes were also documented with involvement of AMLI(CBFA2/RUNXI), identifying a total of 23 different 21q22/AMLI translocations. The data analysis was carried out on the basis of five subsets of 21q22 cases, that is, t(8;21) with and without additional aberrations, t(3;21), t(16;21), and other 21q22 translocations. Dysplastic features were present in all 21q22 cases. Therapy-related acute myeloid leukemia (t-AML) at presentation was highest in t(8;21) (82%) and lowest in t(3;21) (37.5%) patients. Cumulative drug dose exposure scores for alkylating agents (AAs) and topoisomerase II inhibitors indicated that t(3;21) patients received the most intensive therapy among the five 21q22 subsets, and the median AA score for patients with secondary chromosome 7 aberrations was double the AA score for the entire 21q22 group. All five patients who received only radiation therapy had t(8;21) t-AML. The median latency and overall survival (OS) for 21q22 patients were 39 and 14 months (mo), compared to 26 and 8 mo for 11q23 patients, 22 and 28 mo for inv(16), 69 and 7 mo for Rare recurring aberrations, and 59 and 7 mo for Unique (nonrecurring) balanced aberration (latency P ltoreq 0.016 for all pairwise comparisons; OS, P ltoreq 0.018 for all pairwise comparisons). The percentages of 21q22 patients surviving 1 year, 2 years, and 5 years were 58%, 33%, and 18%, respectively. Noticeable differences were observed in median OS between 21q22 patients (n = 7) receiving transplant (BMT) (31 mo) compared to 21q22 patients who received intensive non-BMT therapy (n = 46) (17 mo); however, this was nonsignificant because of the small sample size (log-rank, P = 0.33). t-MDS/t-AML with balanced 21q22 aberrations was associated with prior exposure to radiation, epipodophyllotoxins, and anthracyclines, dysplastic morphologic features, multiple partner chromosomes, and longer latency periods when compared to 11q23 and inv(16) t-MDS/AML Workshop subgroups. In general, patients could be divided into two prognostic risk groups, those with t(8;21) (median OS, 19 mo) and those without t(8;21) (median OS, 7 mo) leukemia (log-rank, P = 0.0007) - SMERDEL A, PLOSKI R, FLATO B, MUSIEJ-NOWAKOWSKA E, THORSBY E, FORRE O: Juvenile idiopathic arthritis (JIA) is primarily associated with HLA-DR8 but not DQ4 on the DR8-DQ4 haplotype. Ann.Rheum.Dis. 2002, 61:354-357.
Organism:Institute of Immunology, Rikshospitalet University Hospital, Oslo, Norway Human Molecular Genetics Laboratory of the Department of Forensic Medicine and the Department of Diabetology and Inborn Defects, Medical Academy in Warsaw, Poland Centre for Rheumatic Diseases, Rikshospitalet University Hospital Institute of Rheumatology, Warsaw, Poland
Abstract: Background: Juvenile idiopathic arthritis (JIA) is strongly associated with the DR8-DQ4 haplotype. The genes encoding DR8 and DQ4 are in strong linkage disequilibrium (LD) and occur together on the same HLA haplotype in almost all patients and controls. Because of the strong LD it is not clear whether DR8, DQ4, or both, are primarily associated with JIA. Objective: To unveil the primary association of JIA---that is, with DR8 or DQ4. Methods: DRB1, DQA1, and DQB1 alleles of 585 Norwegian and 47 Polish unrelated patients with JIA (categorised as pauciarticular and rheumatoid factor negative polyarticular JIA), and of 3155 Norwegian and 158 Polish unrelated controls, were typed using a polymerase chain reaction or oligonucleotide hybridisation and sequence-specific primers method. Results: Several haplotypes which encoded DR8 (that is, carried DRB1*08) and which did not encode DQ4 (that is, did not carry DQA1*0401) were found. Such haplotypes were found in three Norwegian patients and two controls (p=0.029). In the Polish population such haplotypes were found among four patients with JIA and two controls (p=0.025). No haplotypes which carried DQA1*0401 and DQB1*0402 in the absence of DRB1*08 were found, either among patients with JIA (Polish and Norwegian) or among the controls (Polish). Conclusion: On the DR8-DQ4 haplotype the DRB1*08 allele is primarily associated with JIA
Internet : PM:11874841 - SRIVASTAVA A, BAXI M, YADAV S, AGARWAL A, GUPTA RK, MISRA SK, MITHAL A: Juvenile rheumatoid arthritis with amyloid goiter: report of a case with review of the literature. Endocr.Pathol. 2001, 12:437-441.
Organism:Department of Pathology, New England Medical Center, 750 Washington Street, Boston, MA 02111, USA E-mail: ASrivastava@Lifespanorg
Abstract: Clinically significant enlargement of the thyroid owing to amyloid deposition is a rare occurrence. A 23-yr-old female, a case of juvenile rheumatoid arthritis, developed rapidly increasing thyromegaly during the course of her illness with complaints of dyspnea and dysphagia. Thyroid function tests were within normal limits. Fine-needle aspiration cytology proved inconclusive. Total thyroidectomy was done for symptomatic relief with a preoperative clinical impression of malignancy. Histopathologic findings were consistent with amyloid goiter. The findings of this case are presented, to emphasize the difficulties in making a definite preoperative diagnosis, along with a brief review of the literature
Internet : PM:11914477 - TAKEI S: Mizoribine in the treatment of rheumatoid arthritis and juvenile idiopathic arthritis. Pediatr.Int. 2002, 44:205-209.
Organism:Department of Pediatrics, Faculty ofMedicine, Kagoshima University, Kagoshima, Japan
Abstract: Background: Mizoribine (MZR), isolated from culture medium of the mold, is a novel immunosuppressant developed in Japan. It has been used in patients with renal transplantation, lupus nephritis, nephrotic syndrome and rheumatoid arthritis (RA). Objectives: To review MZR in regards to mechanism of action, pharmacokinetics, efficacy and safety in the treatment of rheumatoid RA and juvenile idiopathic arthritis (JIA). Results: The drug MZR inhibits both humoral and cellular immunity in RA patients. It is completely excreted in the urine within 24 h, which contributes to the safety of MZR. A series of multicenter studies indicated that MZR was effective and safe in the treatment of RA. In JIA, however, there are only a few case reports reporting its efficacy and safety. Conclusion: A double-blinded multicenter study is needed to establish the efficacy, safety and indication of MZR in the treatment of JIA
Internet : PM:11896888 - TEN CATE R, SUIJLEKOM-SMIT LW, BRINKMAN DM, BEKKERING WP, JANSEN-VAN WIJNGAARDEN CJ, VOSSEN JM: Etanercept in four children with therapy-resistant systemic juvenile idiopathic arthritis. Rheumatology (Oxford) 2002, 41:228-229.
Organism:Departments of Paediatrics and Physical Therapy, Leiden University Medical Centre and Departments of Paediatrics and Physical Therapy, University Hospital Rotterdam/Sophia's Children's Hospital, The Netherlands
Internet : PM:11886975 - TRUCKENBRODT H, HAFNER R: A contribution to nomenclature and classification of juvenile spondylarthropathies. Aktuelle Rheumatologie 2002, 27:13-17.
Organism:Dr. H. Truckenbrodt, Rheumakinderklinik, Gehfeldstralsse 24, 82467 Garmisch-Partenkirchen
Abstract: Juvenile spondylarthropathies (JSpA) comprise a heterogeneous group of diseases which should receive further attention. They are associated with the genetic marker HLA B 27 and usually become manifest after the age of 6 to 8 years. Early and cardinal symptoms are asymmetric arthritis of the major joints of the lower limbs and/or enthesitis. All forms have a tendency to develop sacroiliitis, which often appears with only minor complaints. Ankylosing spondylitis will become manifest only in adulthood. 60 to 70% of juvenile patients suffer from undifferentiated spondylarthropathy. Differentiated forms comprise patients with sacroiliitis, psoriatic arthritis, arthritis with inflammatory bowel disease or reactive arthritis. Classification of JSpA is controversial. The EULAR classification of Oslo 1977 includes them in the diagnosis "juvenile chronic arthritis". Criteria of the ACR for juvenile rheumatoid arthritis exclude the JSpA. For the undifferentiated forms the term SEA-Syndrome is often used in USA. The new international classification of the ILAR of Durban 1997 with the term juvenile idiopathic arthritis proves unsatisfactory for classification of the JSpA. They appear mainly in the category enthesitis-related arthritis, but may also be contained in the categories oligoarthritis, RF-negative polyarthritis, psoriatic arthritis or quite often in the unspecific category "other types of arthritis". Revision of the ILAR classification is strongly recommended to do justice to the JSpA which are among the most frequent rheumatic diseases in childhood
Internet : info.rh-kkl@rummelsberg.de - TRUCKENBRODT H, HAEFNER R: [A contribution to nomenclature and classification of juvenile spondylarthropathies.]
<ORIGINAL> Zur Nomenklatur und Klassifikation der juvenilen Spondylarthropathie. Aktuelle Rheumatologie 2002, 27:13-17.
Organism:Rheumakinderklinik, Gehfeldstrasse 24, 82467, Garmisch-Partenkirchen Germany^E-Mail: info.rh-kkl@rummelsberg.de
Abstract: Juvenile spondylarthropathies (JSpA) comprise a heterogeneous group of diseases which should receive further attention. They are associated with the genetic marker HLA B 27 and usually become manifest after the age of 6 to 8 years. Early and cardinal symptoms are asymmetric arthritis of the major joints of the lower limbs and/or enthesitis. All forms have a tendency to develop sacroiliitis, which often appears with only minor complaints. Ankylosing spondylitis will become manifest only in adulthood. 60 to 70% of juvenile patients suffer from undifferentiated spondylarthropathy. Differentiated forms comprise patients with sacroiliitis, psoriatic arthritis, arthritis with inflammatory bowel disease or reactive arthritis. Classification of JSpA is controversial. The EULAR classification of Oslo 1977 includes them in the diagnosis "juvenile chronic arthritis". Criteria of the ACR for juvenile rheumatoid arthritis exclude the JSpA. For the undifferentiated forms the term SEA-Syndrome is often used in USA. The new international classification of the ILAR of Durban 1997 with the term juvenile idiopathic arthritis proves unsatisfactory for classification of the JSpA. They appear mainly in the category enthesitis-related arthritis, but may also be contained in the categories oligoarthritis, RF-negative polyarthritis, psoriatic arthritis or quite often in the unspecific category "other types of arthritis". Revision of the ILAR classification is strongly recommended to do justice to the JSpA which are among the most frequent rheumatic diseases in childhood - WYSZYNSKA E, ROMICKA AM, ZIOLKOWSKA M: Cyclosporine A in the treatment of patients with juvenile idiopathic arthritis. Reumatologia 2001, 39:335-343.
Organism:E. Wyszynska, Klinika Reumatol. Wieku Rozwojowego, Instytut Reumatologiczny, ul. Spartanska 1, 02 - 637 Warszawa
Abstract: This study presents an open, prospective trial evaluating the effectiveness, safety and tolerance of cyclosporine A (CyA) in 16 patients with juvenile idiopathic arthritis-systemic or polyarticular onset. All patients had severe course of the disease with active arthritis. In 6 children CyA was the first choice of the antirheumatic drugs, in 10 - reason for CyA treatment was resistance to previous therapy or adverse events during it. The duration of CyA treatment was from 4 to 18 months. The evaluation of clinical status and laboratory data (including several proinflammatory cytokines) was assessed before the trial, 3 and 6 months after beginning of treatment. (A good clinical control of the disease was achieved in all patients). In 11 patients a significant improvement of clinical status and laboratory data was observed with a reduction of the IL-15, and IL-6 level. In the majority of the patients steroid doses were markedly decreased. The adverse effects were observed in 6 patients (including the disturbances of tolerance in 1); in 4 of them the therapy was withdrawn. The results of this study confirmed the effectiveness of CyA in systemic and joint manifestation of juvenile idiopathic arthritis, especially in the disease of recent onset