Bibliography October 2003

1. ANGELINI F, CANCRINI C, COLAVITA M, PANEI P, CONCATO C, ROMITI ML, CHINI L: Role of parvovirus B19 infection in juvenile chronic arthritis. Is more investigation needed? [13]. Clinical and Experimental Rheumatology (Italy ) 21:5 684, 2003

2. ARAKAWA H, YAMASAKI M, KURIHARA Y, YAMADA H, NAKAJIMA Y: Methotrexate-induced pulmonary injury: serial CT findings. J Thorac Imaging 18:4 231-236, 2003
   Organism: Department of Radiology, St Marianna University School of Medicine, Kawasaki City, JapanFAU - Arakawa, Hiroaki
   Abstract: We describe serial computed tomographic (CT) findings of methotrexate (MTX)-induced pulmonary injury. MATERIALS AND METHODS: The cases of 8 patients (3 men and 5 women; mean age 58.6 years, range 16 to 75 years) of clinically diagnosed MTX-induced pulmonary injury were reviewed. Six patients had rheumatoid arthritis, 1 had lupus erythematosus profundus, and 1 had juvenile rheumatoid arthritis. CT findings on admission and at follow-up were evaluated. RESULTS: The most common CT features were diffuse and patchy bilateral ground-glass opacity with (n = 3) or without reticulation (n = 4) and consolidation (n = 1). These opacities showed no predilection for any particular lung zone in 6 patients but did show dependent predilection in 1 patient and upper lobe predilection in 1. Diffuse centrilobular ill-defined nodules were noted in 1 patient, which disappeared on follow-up. During the average post-treatment follow-up period of 31.0 days (range 3 to 76 days), the opacities quickly improved after treatment in 6 patients; however, in 2 patients with pre-existing interstitial pneumonitis the opacities were refractory. CONCLUSION: CT features of MTX-induced pulmonary injury were variable and included diffuse parenchymal opacification, reticular opacities, and centrilobular nodules. These opacities usually responded quickly to treatment; however, those patients with lung fibrosis at presentation may have worse prognosis

3. BARLOW JH, CULLEN LA, FOSTER NE, HARRISON K, WADE M: Does arthritis influence perceived ability to fulfill a parenting role? Perceptions of mothers, fathers and grandparents. Patient Educ Couns 37:2 141-151, 1999
   Organism: Psychosocial Rheumatology Research Centre, School of Health and Social Sciences, Coventry University, Priory Street, Coventry CV1 5FB, UKFAU - Barlow, J H
   Abstract: The presence of a painful, disabling chronic disease may have implications for perceived ability to fulfill a parenting role. The purpose of this research was to examine the realities of parenting from the perspectives of mothers, fathers and grandparents with arthritis using a combination of methods: a cross sectional survey and in-depth focus group discussions. There was consensus that pain, fatigue and restricted physical functioning combined to interfere with the parenting role. Overall, approximately 35% of the sample had experienced difficulties attributed to arthritis. A gender difference emerged with women reporting more difficulties in relation to caring for babies and toddlers, whereas men reported more problems as children grew older. Key themes concerned: physical limitations; practical and caring issues; social factors; emotional response; hereditary risks and safety issues. Perceived inability to fulfill parenting roles resulted in feelings of frustration, guilt, anger and depression. A number of positive outcomes were mentioned including children's increased awareness of the needs of others. Limitations of the methodological approach adopted are discussed

4. BARTON A, LAMB R, SYMMONS D, SILMAN A, THOMSON W, WORTHINGTON J, DONN R: Macrophage migration inhibitory factor (MIF) gene polymorphism is associated with susceptibility to but not severity of inflammatory polyarthritis. Genes Immun 4:7 487-491, 2003
   Organism: Arthritis Rheumatism Campaign Epidemiology Research Unit, University of Manchester, Manchester, UK ABzarton@fslsermanacukFAU - Barton, A
   Abstract: The aim of the study was to investigate whether polymorphisms of macrophage migration inhibitory factor (MIF) determine susceptibility to or severity of inflammatory polyarthritis (IP). Genotypes for a single-nucleotide polymorphism (MIF-173*G/C) and a tetranucleotide (CATT)(n) repeat mapping to the promoter region of the MIF gene were compared between UK Caucasian IP cases (n=438) and controls (n=343). Both polymorphisms were also investigated for association with features of disease activity and severity at baseline and by 5 years. The MIF-173*C allele (OR 1.7, 95% CI 1.3-2.4, P=1.8 x 10(-4)) and the CATT(7) allele (OR 1.5, 95% CI 1.0-2.1, P=0.02) were found to be associated with increased susceptibility to IP. Furthermore, presence of the haplotype containing both associated polymorphisms was associated with a three-fold increase risk of developing IP. No association with disease severity or activity either at baseline or by 5 years was detected for either of the promoter polymorphisms studied. In conclusion, MIF is a susceptibility gene for the development of IP. The same alleles previously reported to be associated with susceptibility to juvenile idiopathic arthritis account for the increased risk. The promoter polymorphisms of MIF, investigated in this study, do not influence the severity of disease outcome by 5 years

5. BECHTOLD S, RIPPERGER P, HAFNER R, SAID E, SCHWARZ HP: Growth hormone improves height in patients with juvenile idiopathic arthritis: 4-year data of a controlled study. J Pediatr 143:4 512-519, 2003
   Organism: Endocrine Division, University Children's Hospital Munich, Lindwurmstrasse 4, D-80337 Munich, Germany SusanneBechtold@heliosmeduni-muenchendeFAU - Bechtold, S
   Abstract: OBJECTIVE: To evaluate the efficacy and safety of growth hormone treatment in severely growth retarded children with juvenile idiopathic arthritis (JIA) receiving glucocorticoids. STUDY DESIGN: Children with systemic and polyarticular idiopathic arthritis (22 F, 16 M) with a mean age of 10.1 years were enrolled in this controlled study. Eighteen patients (9 F, 9M; mean age, 10.5 years) received growth hormone in a dose of 0.20 to 0.33 mg/kg body weight per week for 4 years. Twenty patients (13 F, 7 M; mean age, 9.6 years) served as an untreated control group. RESULTS: Mean improvement in height in the treated group was 1 SD, whereas the patients of the control group lost 0.7 SD. Disease activity markers correlated significantly with the mean growth velocity standard deviation score. In general, children with mild or moderate disease and lower comedication grew and responded better to growth hormone therapy than those with active disease. No adverse events were noted. CONCLUSION: Our data suggest that long-term growth hormone therapy has a beneficial effect in children with severe forms of JIA. Further data are needed to confirm the efficacy and safety of growth hormone and its effect on final height

6. BERNTSON L, ANDERSSON GB, FASTH A, HERLIN T, KRISTINSSON J, LAHDENNE P, MARHAUG G, NIELSEN S, PELKONEN P, RYGG M: Incidence of juvenile idiopathic arthritis in the Nordic countries. A population based study with special reference to the validity of the ILAR and EULAR criteria. J Rheumatol 30:10 2275-2282, 2003
   Organism: Department of Pediatrics, Falun Hospital, SE-791 82 Falun, Sweden lillemorberntson@falunmailteliacomFAU - Berntson, Lillemor
   Abstract: OBJECTIVE: To find the incidence of juvenile arthritis according to the ILAR and EULAR criteria within defined areas in the Nordic countries, and to study the validity of the ILAR and EULAR criteria from this perspective. METHOD: A longitudinal, prospective, population based study with patients enrolled according to the ILAR and EULAR criteria. Twenty doctors in Iceland, Norway, Sweden, Denmark, and Finland collected data from the incidence cases within their catchment areas over a period of 1.5 years, beginning July 1, 1997. Clinical and serological data from the first year of the disease were collected. RESULTS: In the whole group of 315 patients, the incidence rate was 15 per 100,000 children/year (95% CI 13-17) according to the ILAR criteria, varying from 7 (1-13) in Iceland, 19 (7-31) and 23 (10-36) from 2 different regions in Norway, and 9 (5-12) and 16 (9-23) from 2 different areas in Denmark, to 15 (12-18) in Sweden and 21/100,000/year (15-26) in the Helsinki region in Finland. An early peak in distribution for age of onset was found in girls but not in boys. The number of antinuclear antibody (ANA) positive children in the whole group, made up of children who had undergone at least one analyzed ANA test, was 123/315 (39%). Girls were ANA positive in 83/197 (42%) and boys in 40/118 (34%). Uveitis developed in 27/315 (8.6%) children during the first 6 months of the disease. CONCLUSION: Incidence rates of juvenile arthritis for areas within the Nordic countries were in accord with previous data. The ILAR criteria present slightly higher incidence rates, with a shorter disease duration for inclusion, compared to the EULAR criteria. Patients in one subgroup in either of the criteria sets do not necessarily belong to the expected subgroup in the other set of criteria; e.g., for juvenile ankylosing spondylitis (EULAR) and enthesitis related arthritis (ILAR). Our epidemiological findings are a reminder to be aware of possible new subgroups in children with juvenile arthritis

7. CHAN YC, TAY YK, TAN LK, HAPPLE R, GIAM YC: Harlequin ichthyosis in association with hypothyroidism and juvenile rheumatoid arthritis. Pediatr Dermatol 20:5 421-426, 2003
   Organism: National Skin Center, Singapore ycchan@nscgovsgFAU - Chan, Yuin-Chew
   Abstract: Harlequin ichthyosis is a rare and severe congenital erythrodermic ichthyosis characterized at birth by hyperkeratotic plates covering the entire body, ectropion, eclabium, poorly developed ears, and contractures of the hands and feet. Two Chinese children, a 2-year-old boy and an 11-year-old girl, presented with these classic features as well as alopecia and loss of eyebrows and eyelashes. The boy was small for his age and was found to have hypothyroidism at the age of 18 months; he is currently on thyroxine replacement therapy. At 6 years of age, the girl developed symmetrical polyarthritis associated with positive rheumatoid factor and radiologic evidence of erosive arthritis, suggestive of juvenile rheumatoid arthritis. She received prednisolone, nonsteroidal anti-inflammatory drugs (NSAIDs), and subsequently methotrexate for her arthritis, with clinical and radiologic improvement. Early therapy with oral retinoids in both children accelerated shedding of the hyperkeratotic plates as well as improved ectropion and eclabium. There was no major adverse reaction to oral retinoids. The development of juvenile rheumatoid arthritis in survivors with harlequin ichthyosis has not been previously described. The use of prednisolone and NSAIDs in the girl did not affect the skin condition, but the addition of methotrexate led to a decrease in erythema. The association with autoimmune disease is probably coincidental. The psychosocial impact of this severe lifelong disease on the two families was enormous. Early retinoid therapy may improve the disorder and help increase survival rates. A multidisciplinary approach, including psychosocial support of the affected families, is vital in the management of this lifelong disease

8. GARAVITO G, MALAGON C, RAMIREZ LA, DE LA CRUZ OF, URIBE O, NAVARRO E, IGLESIAS A, MARTINEZ P, JARAQUEMADA D, EGEA E: [In Process Citation]. Biomedica 23:3 254-262, 2003
   Organism: Universidad Autonoma de Barcelona, Barcelona, Espana ggaravito@uninorteeducoFAU - Garavito, Gloria
   Abstract: Oligotypes of the human leukocyte antigen HLA Class II, DRB1 alleles were characterized at the molecular level in a group of Colombian children suffering juvenile rheumatoid arthritis (JRA). The distribution of these alleles was examined in a group of Colombian mestizo children (genetic admixture of Amerindians, Europeans and Africans) suffering from clinically distinct JRA subsets in order to detect HLA allele frequency differences in patients with different JRA subsets. A group of 65 patients with JRA and 65 controls were characterized for the subtypes of the HLA-DRB1 alleles using polymerase chain reaction with sequence-specific oligonucleotide probes (PCR-SSOP). The oligotyping protocol recommended by the 12th International Histocompatibility Workshop held in St. Malo, Paris, in 1996, was used. Subtype HLA-DRB1*1104 was the allele most strongly associated with susceptibility to JRA (Fisher's p = 0.013, odds ratio (OR) = 16.79, etiologic fraction (EF) = 0.93). HLA-DRB1*1602 was also associated with susceptibility to a lesser degree (Fisher's p = 0.016, OR = 8.98, EF = 0.88). HLA-DRB1 alleles participating in JRA protection were HLA-DRB1*1501 (preventive fraction (PF) = 0.466, p = 0.005) and HLA DRB1*1402 (PF = 0.49, p = 0.009). The relationship between some HLA-DRB1 alleles and clinical features was also compared. The presence of rheumatic factor was associated with the alleles HLA-DRB1*0407 (p = 0.05, OR = 11.2, EF = 0.45) and HLA-DRB1*1302 (p = 0.02, OR = 22.8, EF = 0.63). There was also an association between HLA-DRB1*0701 (p = 0.001, OR = 58, EF = 0.73) with expressing ANA +. We found that in the oligoarticular subset, the allele HLA-DRB1*1104 (p = 0.0034, OR = 41.53, EF = 0.97) was the one expressed most commonly. In the poliarticular group, the alleles most frequently expressed were HLA-DRB1*0404 (Fisher's p = 0.012, OR = 8.75, EF = 0.88). In patients with systemic JRA, the HLA-DRB1*1602 allele (p = 0.005, OR = 21.33, EF = 0.95) was most frequent. These results suggested that the MHC genes of mestizo children influence not only the clinical expression of the disease, but also the susceptibility to its development

9. GROM AA: Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: The same entities? Current Opinion in Rheumatology 15:5 587-590, 2003

10. HASHKES PJ, SHAJRAWI I: Sarcoid-related uveitis occuring during etanercept therapy. Clinical and Experimental Rheumatology (Italy ) 21:5 645-646, 2003
    Abstract: We report the case of a 7-year-old boy who was initially diagnosed as having polyarticular juvenile idiopathic arthritis. Clinical and laboratory features of overt sarcoidosis became evident early during etanercept therapy when he developed acute panuveitis, papular skin rash and elevated levels of angiotensin-converting enzyme. Non-caseating granulomas were present in the liver. Uveitis resolved upon discontinuation of etanercept and systemic administration of corticosteroids. In rare cases expression of autoimmune disorders or expanded clinical features of these disorders may occur during etanercept treatment

11. HEINZMANN A, JERKIC SP, GANTER K, KURZ T, BLATTMANN S, SCHUCHMANN L, GERHOLD K, BERNER R, DEICHMANN KA: Association study of the IL13 variant Arg110Gln in atopic diseases and juvenile idiopathic arthritis. J Allergy Clin Immunol 112:4 735-739, 2003
    Organism: University Children's Hospital, University of Freiburg, Freiburg, GermanyFAU - Heinzmann, Andrea
    Abstract: BACKGROUND: It has previously been shown that various inflammatory diseases, such as diabetes mellitus, bronchial asthma, chronic inflammatory bowel diseases, and rheumatoid arthritis, are in some circumstances genetically linked to the same chromosomal regions. Consequently, common genes underlying the pathogenetics of these diseases have been proposed. Chronic inflammatory disorders can be subdivided by their predominant immune response, either TH1 or TH2. For example, juvenile idiopathic arthritis (JIA) is a TH1 disease, and bronchial asthma is a TH2 disease. OBJECTIVES: The present study investigated the polymorphism Arg110Gln within the IL13 gene, a strong TH2 cytokine. We attempted to determine whether it is associated with these 2 diseases and whether this would reflect the TH1/TH2 paradigm. METHODS: Arg110Gln was typed in 4 different populations: asthmatic children, atopic children, children with JIA, and a control population. Statistical analysis was performed by using logistic and linear regression analysis of serum IgE levels and the Armitage trend test. RESULTS: The variant Gln110 was shown to be associated with increased total serum IgE levels in our atopic population (P =.006) and was weakly associated with bronchial asthma (P =.04). There was no association of the variant with JIA when compared with the control population. However, the variant Gln110 was significantly less frequent in children with JIA compared with its presence in children with bronchial asthma (P =.007). CONCLUSION: This is the first study to compare the same gene variant in TH1 and TH2 chronic inflammatory diseases. The results suggest that the same gene variant might protect from one disease and make an individual susceptible to the other

12. JOHNSTONE R: A small study, but the results ring true. BMJ 327:7419 844-845, 2003
    Organism: Arthritis and Rheumatism International, 14 Hazel Lane, Skelmersdale WN9 6UN robertjohnstone@onetelnetukFAU - Johnstone, Robert

13. KLEPPE S, METRY D, BACINO C: Blau syndrome and the complexities of proper diagnosis and treatment. Am J Hum Genet 73:5 263, 2003

14. KORKMAZ C, AKAY OM, KASIFOGLU T: Spondyloepiphyseal dysplasia tarda simulating juvenile chronic arthritis [6]. Clinical and Experimental Rheumatology (Italy ) 21:5 677, 2003

15. LANGER HE, EHLEBRACHT-KONIG I, MATTUSSEK S: [In Process Citation]. Z Arztl Fortbild Qualitatssich 97:6 357-363, 2003
    Organism: Rheumatologische Schwerpunktpraxis am Evangelischen Krankenhaus Dusseldorf DrLanger@rheuma-onlinedeFAU - Langer, Hans-Eckhard
    Abstract: Since 1989 patient education programmes for patients with rheumatoid arthritis, ankylosing spondylitis and other spondylarthropathies, systemic lupus erythematosus, vasculitis, fibromyalgia, and juvenile chronic arthritis and their parents have been developed by an interdisciplinary team of the German Society of Rheumatology (Deutsche Gesellschaft fur Rheumatologie). Up to date, about 500 people were trained to be group leaders or specialised trainers with an associated train-the-trainer program. In 1999, the Society discharged preliminary guidelines for patient education in rheumatology. Prospective randomised studies demonstrated that patient education led both to an improvement of knowledge, self-efficacy and self-help activities and to an improvement of arthritis-related helplessness and pain, and a reduction of both temporary and permanent disability

16. LIEM JJ, ROSENBERG AM: Growth patterns in juvenile rheumatoid arthritis. Clinical and Experimental Rheumatology (Italy ) 21:5 663-668, 2003
    Abstract: Objective. To define patterns of growth in juvenile rheumatoid arthritis (JRA) and to evaluate possible associated clinical and laboratory correlates. Methods. The study population comprised 67 children with JRA who had been followed for 5 years or longer and whose follow-up period did not extend beyond 18 years of age. Height and weight z scores were calculated with reference to age-related standards for each of the annual follow-up intervals and correlated with JRA subtype, the presence of rheumatoid factor (RF), the erythrocyte sedimentation rate (ESR), alkaline phosphatase level (ALP) and medication history. Results. Initial height-for-age (HAZ) scores for pauciarticular, polyarticular and systemic JRA onset groups (PaJRA, PoJRA and SJRA respectively) were +0.27, -0.07 and +0.40 respectively. A significantly lower HAZ score in the SJRA population compared to the PaJIA population first became apparent at year 2 and the difference was maintained throughout the 9-year follow-up period. A significantly lower HAZ score in the SJRA population compared to the PoJRA population first became apparent at year 6 and the difference was maintained until the ninth year. During the 9-year follow-up period, RF-positive children tended to have negative HAZ scores whereas RF-negative children tended to have positive HAZ scores. The SJRA onset group displayed significantly lower HAZ scores, as compared to the HAZ score at onset, for 7 of the 9 subsequent follow-up intervals. Only 2 patients had heights < 2SD below the mean at final determination. Delay in generalized linear growth occurred predominantly in the SJRA population and to a lesser degree in those with PoJRA associated with RF positivity. Conclusions. Delay in linear growth occurs in some children with JRA. Patients with pauciarticular and RF-negative polyarticular disease can have growth patterns similar to normal children. Children with RF-positive polyarticular and systemic JRA have more significant growth retardation that occasionally can be sustained and extreme

17. MEURER M: Childhood discoid lupus erythematosus and antimalarials. Dermatology (Basel) 207:2 133, 2003

18. PAROLI MP, SPERANZA S, MARINO M, PIRRAGLIA MP, PIVETTI-PEZZI P: Prognosis of juvenile rheumatoid arthritis-associated uveitis. Eur J Ophthalmol 13:7 616-621, 2003
    Organism: Immunovirology Ocular Service, Department of Ophthalmology, University of Roma La Sapienza, Roma, Italy uveitipiv@iolitFAU - Paroli, M P
    Abstract: PURPOSE: To evaluate the clinical characteristics and the visual prognosis of uveitis in juvenile rheumatoid arthritis (JRA). METHODS: The authors examined 63 patients with uveitis and JRA observed from January 1985 to December 2000. The following characteristics of each patient were considered: age at first visit, age at onset of uveitis and arthritis, sex, laterality and localization of uveitis, ocular complications, antinuclear antibody (ANA) and human leukocyte antigen (HLA) DR11 positivity, and follow-up. A retrospective study on mid-time visual outcome and ocular complications was performed on 42 patients with more than 12 months of follow-up. RESULTS: A total of 76.2% of the patients were female, with a mean age of 8.1 years. Chronic anterior uveitis was bilateral in 77.8% of the cases and unilateral in 22.2%. Arthritis was oligoarticular at onset in 87.3% of cases, and polyarticular in 12.7%. Mean age at arthritis onset was 4.5 years and mean age at uveitis onset was 5.4 years. ANA were positive in 92% of cases and HLA DR11 was present in 36 of the 43 patients tested (83.7%). Among the 42 patients with more than 12 months of follow-up, ocular complications occurred in 90.5% of cases and the most frequent were cataract (64.4% of eyes) and band keratopathy (59.2% of eyes). Secondary glaucoma (25% of eyes) was associated with the worst visual prognosis. A total of 64.5% of eyes maintained a visual acuity between 20/33 and 20/20 at the end of the follow-up. CONCLUSIONS: Visual prognosis of uveitis associated with JRA is improving, owing to earlier diagnosis and intensive treatment. Ocular complications occurred frequently in patients with uveitis and JRA but they did not seem to seriously affect the final visual outcome. The authors did not observe any correlation between prognosis and sex, age at the onset of uveitis or arthritis, pattern of arthritis, or positivity for ANA or HLA DR11. In a percentage of cases, uveitis may develop before arthritis or years after the onset of arthritis; therefore, continuous ophthalmologic examinations are needed in young people with JRA

19. PRAHALAD S, FRASER AM, O'BRIEN E, KERBER RA, MINEAU GP, BOHNSACK JF: Lack of association between birth order and juvenile idiopathic arthritis. Arthritis Rheum 48:10 2989-2990, 2003
    Organism: University of Utah, Salt Lake City, UT, USAFAU - Prahalad, Sampath

20. PRAKKEN BJ, ROORD S, RONAGHY A, WAUBEN MARCA, 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

21. QUINN MA, GREEN MJ, GOUGH AK: Low dose methotrexate osteopathy in a patient with polyarticular juvenile idiopathic arthritis. Ann Rheum Dis 62:11 1123-1124, 2003

22. ROSEN P, THOMPSON S, GLASS D: Non-HLA gene polymorphisms in juvenile rheumatoid arthritis. Clinical and Experimental Rheumatology (Italy ) 21:5 650-656, 2003
    Abstract: A substantial amount of work has gone into elucidating the non-HLA genetic associations in JRA. In this paper, we attempt to provide an overview of this body of knowledge. Direct comparison of the different studies is difficult. Different ethnic populations, different JRA/JIA subgroups, and different systems of nomenclature and classification all impose various limitations. Adding to the complexity is the polygenic nature of chronic childhood arthritis. Family based studies will be necessary to overcome ethnicity related issues. A candidate gene approach complemented by genome wide screen data will hopefully advance our knowledge of the genetics of JRA

23. ROSENBAUM JT, PLANCK SR, DAVEY MP, IWANAGA Y, KURZ DE, MARTIN TM: With a mere nod, uveitis enters a new era. Am J Ophthalmol 136:4 729-732, 2003
    Organism: Casey Eye Institute, Oregon Health and Science University, Portland, Oregon 97239, USA rosenbaj@ohsueduFAU - Rosenbaum, James T
    Abstract: PURPOSE: To advance the knowledge of the ophthalmologist with regard to new developments in the genetics and pathologic mechanisms of uveitis. DESIGN: A review of recently published literature exploring the relationship between the nucleotide oligomerization domain (NOD2) gene and uveitis. RESULTS: Mutations in the nucleotide-binding region of NOD2 were found to be responsible for familial juvenile systemic granulomatosis (Blau syndrome or Jabs disease), a rare form of uveitis, arthritis, and dermatitis. The NOD2 gene is thought to be involved in the innate immune response to pathogens. Currently, the pathologic mechanisms behind Blau syndrome in familial juvenile systemic granulomatosis are unknown, but the interactions of NOD2 with caspases, nuclear factor kappaB, and other pathways are slowly being revealed. CONCLUSIONS: A single amino acid change in NOD2 can lead to a chronic granulomatous uveitis. By studying NOD2 and the proteins that interact with NOD2, we should gain a better understanding of the pathogenic mechanisms of uveitis and identify novel ways to halt its destructive consequences

24. SAWYER MG, WHITHAM JN, ROBERTON DM, TAPLIN JE, VARNI JW, BAGHURST PA: The relationship between health-related quality of life, pain and coping strategies in juvenile idiopathic arthritis. Rheumatology (Oxford) .: 2003
    Organism: Research and Evaluation Unit, Women's and Children's Hospital and Department of Paediatrics, University of Adelaide, South Australia
    Abstract: OBJECTIVES: To investigate the relationship between health-related quality of life (HRQL), experience of pain and pain coping strategies in children with juvenile idiopathic arthritis (JIA). To compare reports describing these variables obtained from children and their parents. METHODS: Participants were 59 children aged 8 to 18 yr with JIA and their parents. Parents and children completed the PedsQL(TM) generic core scales and arthritis module, the visual analogue scale of the Varni-Thompson Pediatric Pain Questionnaire, and the Waldron/Varni Pediatric Pain Coping Inventory. Parents rated children's functional disability using the Childhood Health Assessment Questionnaire. RESULTS: Parents reported significantly lower scores (indicating worse HRQL) than children on five of the eight PedsQL(TM) scales rating children's HRQL. Parents and children reported a significant negative relationship between pain levels and the PedsQL(TM) scores assessing children's physical, emotional and social functioning. They also reported a significant negative relationship between scores on several pain coping scales and scores on the PedsQL(TM) scales. However, the pattern of these relationships varied for reports from parents and children. CONCLUSIONS: Pain intensity and pain coping strategies have a significant and independent relationship with several domains that comprise the HRQL of children with JIA. However, parents and children have differing perceptions of the nature of these relationships. The differences emphasize the importance of clinicians obtaining information about children's HRQL, pain levels and pain coping strategies from both parents and children

25. SHAW KL, SOUTHWOOD TR, MCDONAGH ON BEHALF OF THE BRITISH PAEDIATRIC RHEUMATOLOGY GROUP JE: Developing a programme of transitional care for adolescents with juvenile idiopathic arthritis: results of a postal survey. Rheumatology (Oxford) .: 2003
    Organism: Institute of Child Health, Division of Reproductive and Child Health, University of Birmingham, Birmingham B15 2TT, UK
    Abstract: Objectives. To explore the transitional needs of adolescents with juvenile idiopathic arthritis (JIA), as perceived by a range of professionals, and to examine how these needs may be addressed within a structured programme of transitional care. Methods. Postal surveys (n = 1670) were distributed to key professionals employed in health, social support, education and vocation. Results. Surveys were completed by 478 individuals. The majority of respondents (91%) were currently active in the care of adolescents with JIA. Planning for transitional care was perceived to be important for both adolescents and parents and to require multidisciplinary involvement. Respondents rated a wide range of resources to be important in supporting adolescents, including self-medication teaching packages and social skills training. A number of barriers to providing transitional care were identified, including inadequate resources, coordination and training. Conclusion. Transitional care in the context of JIA is perceived as necessary by a wide variety of professionals

26. SINGH S: Juvenile chronic/idiopathic arthritis-JCA/JIA: An overview. Journal International Medical Sciences Academy (India ) 15:1 49-52, 2002

27. SKYTTA ET, SAVOLAINEN HA, KAUTIAINEN HJ, BELT EA: Long-term results of leg length discrepancy treated with temporary epiphyseal stapling in children with juvenile chronic arthritis. Clinical and Experimental Rheumatology (Italy ) 21:5 669-671, 2003
    Abstract: Objective. The aim of the present study was to evaluate retrospectively the long-term efficacy of temporary stapling of the knee epiphyses over four decades of use in children with JCA. Methods. Medical data of the patients with temporary epiphyseodesis due to leg length discrepancy (LLD) were studied. Seventeen knees in 17 patients were found with sufficient follow-up data for evaluation. Patient documents and radiographs of these patients were evaluated. Results. The mean age at the time of the operation was 11 years (range: 6-15) in 3 males and 14 females. The preoperative mean LLD was 21 mm (SD 8) and at staple removal 4 mm (SD 10). The difference was -17 mm (95 % CI: -10 to -23). Statistically the result remained the same during the follow-up. Two reversible complications were documented among the 17 stapled knees. In five (29%) cases the correction was affected by re-occurrence of LLD quickly after removal of the staples. Conclusion. In this study with 17 patients and a wide range of follow-up times we found that the good correction of LLD achieved by stapling is usually permanent

28. VELA JI, GALAN A, FERNANDEZ E, ROMERA M, TORRES JJ: [Anterior uveitis and juvenile idiopathic arthritis]. Arch Soc Esp Oftalmol 78:10 561-566, 2003
    Organism: Hospital Universitario Vall D'Hebron, Barcelona, EspanaFAU - Vela, J I
    Abstract: PURPOSE: Anterior uveitis is one of the most important extraarticular manifestations of juvenile idiopathic arthritis (JIA). The aim of this study was to describe the frequency of uveitis, complications and ocular surgical procedures, to corroborate risk factors for the development of uveitis and to analyze its evolutive characteristics. METHODS: Retrospective review of 132 children diagnosed with JIA in our hospital from 1985 to 2000. Patients presenting anterior uveitis (Tyndall + or recent keratic precipitates) were studied. RESULTS: Uveitis was detected in 26 of 132 children (19%). All of them presented pauciarticular JIA. The patients received corticosteroid therapy for a mean time of 19.2 months, developing a mean of 4.5 episodes of uveitis. Patients diagnosed with uveitis before or within 1 year from the onset of arthritis required longer treatment (295 months versus 206) and suffered more episodes (73 versus 44) than those with uveitis found later on. Complications (cataract, band keratopathy, glaucoma, macular edema) developed in 27.9% of the affected eyes. Surgery was required in 10 eyes. CONCLUSIONS: The incidence of uveitis in our study is similar to recently reported rates. Female sex, pauciarticular onset, serum antinuclear antibodies (ANA) and early onset of uveitis seem to increase the development of chronic uveitis. Periodic slit-lamp ophthalmologic screenings in high risk patients are recommended (Arch Soc Esp Oftalmol 2003; 78: 561-566)

29. WEBER P, BRUNE T, GANSER G, ZIMMER K-P: Gastrointestinal symptoms and permeability in patients with juvenile idiopathic arthritis. Clinical and Experimental Rheumatology (Italy ) 21:5 657-662, 2003
    Abstract: Objective. Examining for gastrointestinal involvement in juvenile idiopathic arthritis is an important part of diagnostic and therapeutic procedures. Only few scientific data are available. Methods. In a prospective study, 41 patients with juvenile idiopathic arthritis were examined for clinical and laboratory data of gastrointestinal involvement. Sugar absorption tests with lactulose, mannitol, and sucrose were applied to assess gastric and intestinal mucosal lesions. Faecal albumin and alpha1-antitrypsin levels were measured to examine gastrointestinal protein loss, a test for occult blood in stool was administered and Helicobacter pylori serology was performed. Results. 39% of our study population complained of chronic abdominal pain. The patient group showed increased sucrose excretion (p = 0.002), but a normal lactulose/mannitol ratio compared with healthy controls (p = 0.472). 21% of the patients had an elevated faecal alpha1-antitrypsin level, but only one patient showed occult blood loss. There was no correlation between risk factors and clinical or laboratory signs of gastrointestinal involvement. Conclusion. We conclude that a high percentage of children and adolescents with juvenile idiopathic arthritis treated with non-steroidal antiinflammatory drugs show clinical or laboratory signs of gastrointestinal involvement

30. YOKOTA S: Interleukin 6 as a therapeutic target in systemic-onset juvenile idiopathic arthritis. Current Opinion in Rheumatology 15:5 581-586, 2003