Bibliography November 2003
- AGANNA E, HAWKINS PN, LACHMANN HJ, BYBEE A, KARENKO L, PETTERSSON T, RANKI A, HITMAN GA, WOO P, MCDERMOTT MF: The spectrum and frequency of MEFV, TNFRSF1A, and NALP3/CIAS1/PYPAF1 mutations in patients with AA amyloidosis associated with systemic inflammation. Am J Hum Genet 73:5 508, 2003
- AGLE LM, VAZQUEZ-COBIAN LB, LEHMAN TJ: Clinical trials in pediatric uveitis. Curr Rheumatol Rep 5:6 477-481, 2003
Organism: Division of Pediatric Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA goldscout@aolcomFAU - Agle, Lucila M A
Abstract: Uveitis associated with juvenile rheumatoid arthritis is the most common form of ocular inflammation in children. Prevention of permanent visual damage by this silent disease requires heightened awareness from pediatric rheumatologists and ophthalmologists. Early prediction of severity and prognosis will aid in the identification of those patients requiring more aggressive management. Generally, reports using immunosuppressives in this population are small, uncontrolled, and retrospective. Large-scale collaborative studies have been proposed and are underway. Hopefully, collaborations between pediatricians, pediatric rheumatologists, and ophthalmologists will allow the development of straightforward treatment guidelines for children suffering from chronic uveitis and its related complications - BERI A, SINGH S, GUPTA A, KHULLAR M: Comparison of serum nitric oxide levels in active juvenile rheumatoid arthritis with those of patients in remission. Rheumatol Int .: 2003
Organism: Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh-160012, India
Abstract: Thirty-four children with juvenile rheumatoid arthritis were studied for surrogate markers of increased nitric oxide (NO) production by spectrophotometry. Mean levels of reactive nitrogen intermediates and citrulline levels were higher in patients with active disease than in children with partial and complete remission. A significant correlation was also found between clinical indices of inflammation, erythrocyte sedimentation rate, modified Childhood Health Assessment Questionnaire scores, and surrogate markers of increased NO production - BEYAN E, BEYAN C, DEMIREZER A, ERTUGRUL E, UZUNER A: The relationship between serum ferritin levels and disease activity in systemic lupus erythematosus. Scand J Rheumatol 32:4 225-228, 2003
Organism: Department of Internal Medicine, Numune Education and Research Hospital, Ankara, TurkeyFAU - Beyan, E
Abstract: OBJECTIVE: The aim of this study is to investigate the relationship between serum ferritin levels and disease activity in patients with systemic lupus erythematosus (SLE). METHODS: Serum ferritin levels of 72 SLE patients were measured. The SLE patients were subdivided into two groups according to SLE disease activity index (SLEDAI) as < or = 10 and > or = 11. The results were compared with 31 patients with rheumatoid arthritis (RA). 36 patients among 72 with SLE were evaluated before and after treatment. RESULTS: Serum levels of ferritin in SLE patients were higher than RA patients (p < 0.001). There was a significant difference in ferritin levels before and after treatment. The levels of ferritin in SLE were positively correlated with SLEDAI scores. Patients with SLEDAI scores > or = 11 had significantly higher serum ferritin levels. CONCLUSION: Serum ferritin levels may be a useful marker of disease activity in SLE patients - BOUCHENAKI N, TRAN V, HERBORT CP: The role of systemic corticosteroids and immunosuppressive therapy in childhood uveitis. ARVO Annual Meeting Abstract Search and Program Planner 2003: Abstract, 2003
Abstract: Purpose: To evaluate the indications and the contribution of systemic corticosteroids and immunosuppressive therapy in childhood uveitis in a secondary referral center. Methods: A retrospective case series of children with a chronic or recurrent uveitis who were treated systemically either with corticosteroids and/or immunosuppressive treatment. Indications, efficacy and side effects were analysed. Results: From 1995 to 2002, 742 new uveitis patients were seen in the uveitis clinic at La Source Eye Center, Lausanne, Switzerland, of whom 78 (10.5%) were aged less than 18 years when uveitis started. Thirty-three children (42%) received systemic therapy. Among them, sixteen (48.5%) (7girls, 9 boys) received systemic corticosteroid therapy alone (pars planitis (6), toxoplasmosis (2), juvenile idiopathic arthritis (JIA) (2), Behcet's disease (1), inflammatory choriocapillaropathy (1), undefined diagnosis (4). Seventeen patients (51.5%) were under systemic immunosuppressive therapy and 14 patients (7 girls, 7 boys) had enough data to be included in the analysis. The diagnosis were JIA (4 patients), pars planitis (3), Behcet's disease (2), sarcoidosis (1), undetermined (4). The indications to add an other immunosuppressant were the severity of the inflammation (11 patients) and/or the need for a steroid sparing effect (6). Azathioprine was added in 10 patients, methotrexate in 5, cyclosporine in 3, entanercept in1 and colchicine in 1. Four patients had 2 and 3 patients had 3 immunosuppressants in addition to corticosteroids. After introduction of immunosuppressive therapy disease was controlled in all patients with sufficient follow-up data (n=11) and all were able to reduce their prednisone dose, with 3 patients able to discontinue it. One patient had to discontinue azathioprine (replaced by mycophenolate mofetyl) because of liver toxicity. No other severe side-effects were reported. Conclusions: A relatively large proportion of childhood uveitis needed systemic corticosteroids and immunosuppressive therapy. Azathioprine was effective in all cases used, was well tolerated and is safe as long as blood and liver tests are performed regularly. The use of steroid sparing immunosuppressive agents in the treatment of children with uveitis contributes to control inflammation and helps to avoid the adverse effects especially on growth of systemic steroids - CHAN Y-C, TAY Y-K, TAN LKS, HAPPLE R, GIAM Y-C: Harlequin Ichthyosis in Association with Hypothyroidism and Juvenile Rheumatoid Arthritis. Pediatric Dermatology (United States ) 20:5 421-426, 2003
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 - DAVIS JC, JR., VAN DER HD, BRAUN J, DOUGADOS M, CUSH J, CLEGG DO, KIVITZ A, FLEISCHMANN R, INMAN R, TSUJI W: Recombinant human tumor necrosis factor receptor (etanercept) for treating ankylosing spondylitis: a randomized, controlled trial. Arthritis Rheum 48:11 3230-3236, 2003
Organism: University of California, San Francisco, CA 94143, USA jdavis@medicineucsfeduFAU - Davis, John C Jr
Abstract: OBJECTIVE: To determine the safety and efficacy of etanercept in a multicenter, randomized, placebo-controlled, double-blind trial of adults with moderate to severe active ankylosing spondylitis (AS). METHODS: Patients (n = 277) were treated with either etanercept 25 mg (n = 138) or placebo (n = 139) subcutaneously twice weekly for 24 weeks. The primary outcome measures were the percentages of patients achieving the Assessments in Ankylosing Spondylitis 20% response (ASAS20) at weeks 12 and 24. Other outcome measures included the percentage of patients achieving higher ASAS responses, and the safety of etanercept in patients with AS. All outcome measures were assessed at 2, 4, 8, 12, and 24 weeks. RESULTS: Treatment with etanercept resulted in dramatic improvement. The ASAS20 was achieved by 59% of patients in the etanercept group and by 28% of patients in the placebo group (P < 0.0001) at week 12, and by 57% and 22% of patients, respectively, at week 24 (P < 0.0001). All individual ASAS components, acute-phase reactant levels, and spinal mobility measures were also significantly improved. The safety profile of etanercept was similar to that reported in studies of patients with rheumatoid arthritis or psoriatic arthritis. The only adverse events that occurred significantly more often in the etanercept group were injection-site reactions, accidental injuries, and upper respiratory tract infections. CONCLUSION: Etanercept is a highly effective and well tolerated treatment in patients with active AS - DEIXLER E, HELMKE K: [Adult still's disease as a manifestation of severe hypophosphatemia Still's disease: A disturbance of energy metabolism?]
<ORIGINAL> Adultes Still-Syndrom als Manifestation einer schweren Hypophosphataemie: Morbus Still - eine Stoerung des Energiestoffwechsels? Zeitschrift fuer Rheumatologie 62:3 287-293, /6
Abstract: In this case report, a 56-year-old woman with a reversible multiple organ disease with septic fever, arthritis, rash, weight loss, thrombocytopenia, severe disturbance of liver function, renal tubular dysfunction, general muscular weakness, pleural and pericardial effusions and elevated CRP, leukocytosis and a striking hyperferritinemia is presented. An autoimmune disease and a septic process were excluded. Because of the significantly decreased plasma phosphorus concentration (0.2 mmol/l), caused by malabsorption after Whipple's operation with lowered vitamin D and secondary hyperparathyroidism and by chronic alcoholism, severe phosphate deficiency was diagnosed. By substitution of phosphorus, all symptoms disappeared and laboratory findings normalized. Except for the patient's age, all criteria for the diagnosis of Still's disease were fulfilled. So we reflected upon Still's disease to be rather a disturbance of energy metabolism with secondary immunologic changes than an inflammatory rheumatic disease. Significant weight loss in Still's disease and osteopenia might be signs of decreased energy supply. Besides hypophosphatemia, deficiency of magnesium, which is involved in all ATP-dependent processes, and mitochondrial diseases cause disturbances of energy metabolism - DOLEZALOVA P, TELEKESOVA P, NEMCOVA D, BELOROVA J, KVASNICKA J, HOZA J: [In Process Citation]. Cas Lek Cesk 142:10 615-619, 2003
Organism: Klinika detskeho a dorostoveho lekarstvi 1 LF UK a VFN, Praha detske@lf1cuniczFAU - Dolezalova, P
Abstract: BACKGROUND: Endothelial activation is an important etiopathogenetic factor in a group of disorders characterised by primary or secondary vasculitis. The aim of our study was to determine blood concentrations of von Willebrand factor (vWF), vypusteno soluble intercellular adhesion molecule-1 (ICAM-1) and E-selectin (E-sel) in children with various rheumatic diseases and in paediatric controls and to correlate them with clinical and laboratory variables. METHODS AND RESULTS: Total of 28 healthy children (ZD) and 48 patients were evaluated: 6 with systemic lupus erythematosus (SLE), 7 with other diffuse connective tissue diseases (SSD), 11 with Henoch-Schonlein purpura (HSP), 14 with oligoarticular juvenile idiopathic arthritis (JIA) and 10 febrile controls (FC). Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and full blood count were recorded. ICAM-1, E-sel and vWF concentrations were measured by sandwich ELISA kits. In SLE patients' concentrations of vWF and ICAM-1 were significantly higher than in healthy (p < 0.05), but not febrile controls. ICAM-1 was significantly increased also in SSD group when compared to healthy children (p < 0.01). Differences in other groups did not reach statistical significance. Significant negative correlation with age was observed for the group as a whole, E-sel correlated with leukocyte and thrombocyte counts (p < 0.01), both molecules with CRP (p < 0.05) and with each other (p < 0.01). CONCLUSIONS: Combined measurement of vWF, ICAM-1 and E-sel as possible markers of endothelial activation in such vypusteno wide spectrum of paediatric patients and controls is unique vypusteno. Our finding of increased concentrations of vWF and/or ICAM-1 in children with systemic autoimmune diseases underlines the importance of endothelial involvement in these disorders, but their predictive value in the disease monitoring needs to be further studied - DOLEZALOVA P, TELEKESOVA P, NEMCOVA D, BELOROVA J, KVASNICKA J, HOZA J: Laboratory Markers of Endothelial Involvement in Rheumatic Diseases with Vasculitis in Childhood
LABORATORNI UKAZATELE POSTIZENI ENDOTELU U REVMATICKYCH ONEMOCNENI PROVAZENYCH VASKULITIDOU U DETI. Casopis Lekaru Ceskych (Czech Republic ) 142:10 615-619, 2003
Abstract: Background. Endothelial activation is an important etiopathogenetic factor in a group of disorders characterised by primary or secondary vasculitis. The aim of our study was to determine blood concentrations of von Willebrand factor (vWF), vypus(caron)te(caron)no soluble intercellular adhesion molecule-1 (ICAM-1) and E-selectin (E-sel) in children with various rheumatic diseases and in paediatric controls and to correlate them with clinical and laboratory variables. Methods and Results. Total of 28 healthy children (ZD) and 48 patients were evaluated: 6 with systemic lupus erythematosus (SLE), 7 with other diffuse connective tissue diseases (SSD), 11 with Henoch-Schonlein purpura (HSP), 14 with oligoarticular juvenile idiopathic arthritis (JIA) and 10 febrile controls (FC). Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and full blood count were recorded. ICAM-1, E-sel and vWF concentrations were measured by sandwich ELISA kits. In SLE patients' concentrations of vWF and ICAM-1 were significantly higher than in healthy (p<0.05), but not febrile controls. ICAM-1 was significantly increased also in SSD group when compared to healthy children (p<0.01). Differences in other groups did not reach statistical significance. Significant negative correlation with age was observed for the group as a whole, E-sel correlated with leukocyte and thrombocyte counts (p<0.01), both molecules with CRP (p<0.05) and with each other (p<0.01). Conclusions. Combined measurement of vWF, ICAM-1 and E-sel as possible markers of endothelial activation in such vypus(caron)te(caron)no wide 'spectrum of paediatric patients and controls is unique vypus(caron)te(caron)no. Our finding of increased concentrations of vWF and/or ICAM-1 in children with systemic autoimmune diseases underlines the importance of endothelial involvement in these disorders, but their predictive value in the disease monitoring needs to be further studied - DUZGUN N, MORRIS Y, YILDIZ HI, OZTURK S, AYVA SK, ENSARI A, EREKUL S, KUZU I, ERDOGAN N, DUMAN M: Amyloid goiter in juvenile onset rheumatoid arthritis. Scand J Rheumatol 32:4 253-254, 2003
- FOSTER CS: Diagnosis and treatment of juvenile idiopathic arthritis-associated uveitis. Curr Opin Ophthalmol 14:6 395-398, 2003
Abstract: SUMMARY: PURPOSE OF REVIEW This review surveys publications from peer-reviewed literature published since 2001on the matter of uveitis associated with juvenile idiopathic arthritis.RECENT FINDINGS Uveitis associated with juvenile idiopathic arthritis (JIA) continues to blind substantial numbers of children every year, even in the most developed of societies. This is so because of delayed diagnosis (as a consequence of the silent, asymptomatic nature of ocular inflammation in some patients), and because of delayed referral by general ophthalmologists of children with JIA-associated uveitis to ocular immunologists prepared to move on to systemic immunomodulatory therapy in those instances where the child's uveitis is chronic or continues to recur with each attempt at tapering corticosteroid. The evidence for this is vast.SUMMARY Progress in the matter of reducing the prevalence of blindness secondary to this disease will require the increasing willingness of the world's ophthalmologists to refer patients with JIA-associated uveitis much earlier in the course of his or her disease for consideration of immunomodulatory therapy, and will require legislation mandating visual acuity testing in children attending day care centers - HATAKKA K, MARTIO J, KORPELA M, HERRANEN M, POUSSA T, LAASANEN T, SAXELIN M, VAPAATALO H, MOILANEN E, KORPELA R: Effects of probiotic therapy on the activity and activation of mild rheumatoid arthritis--a pilot study. Scand J Rheumatol 32:4 211-215, 2003
Organism: Rheumatism Foundation Hospital, HeinolaFAU - Hatakka, K
Abstract: OBJECTIVE: To study the effects of Lactobacillus rhamnosus GG (LGG) on rheumatoid arthritis (RA). METHODS: Twenty-one RA patients were randomised to receive 2 capsules of LGG or a placebo twice daily in double-blind fashion for 12 months. Arthritis activity was evaluated by clinical examination, HAQ index, and laboratory tests (e.g. ESR, CRP, pro- and anti-inflammatory cytokines). RESULTS: There were no statistical differences in the clinical parameters, biochemical variables and HAQ index between the study groups over the intervention period. The mean number of tender and swollen joints decreased from 8.3 to 4.6 in the Lactobacillus group and from 5.5 to 4.8 in the placebo group (p = 0.41). According to the global assessment the RA activity was reduced in 71% (LGG group) vs. 30% (controls) (p = 0.15). Serum IL-1 beta increased slightly in the LGG group (p = 0.07), but no differences were seen in IL-6, TNF-alpha, MPO, IL-10 or 1L-12. CONCLUSIONS: Although there were no statistical significant differences in the activity of RA, more subjects in the LGG group reported subjective well being. More studies on the effects of probiotic bacteria in RA are needed - HEILIGENHAUS A, MINGELS A, NEUDORF U, GANSER G: [Juvenile idiopathic arthritis and uveitis: screening and anti-inflammatory therapy]. Klin Monatsbl Augenheilkd 220:11 738-753, 2003
Organism: Augenabteilung am St-Franziskus-Hospital, Munster arndheiligenhaus@t-onlinedeFAU - Heiligenhaus, Arnd
Abstract: This article provides an overview of the rheumatic diseases in childhood, their pathogenesis, epidemiology, diagnosis and clinical course. EULAR, ACR and the current ILAR-classification are compared. The antiinflammatory medical treatment is described. The characteristics of uveitis in the various forms of arthritis are reviewed. The prognostic factors for uveitis manifestation, the clinical course, complications, the prognostic factors for visual loss, and the diverse antiinflammatory medical regimens are evaluated. The suggestions of the "uveitis in childhood study group" concerning screening and treatment of uveitis in patients with juvenile idiopathic arthritis are provided, and a nation-wide documentation of these patients is described - HEINZMANN A, JERKIC S, GANTER KERSTIN, KURZ T, BLATTMANN S, SCHUCHMANN L, GERHOLD KERSTIN, BERNER R, DEICHMANN KA: Association study of the IL13 variant Arg110Gln in atopic diseases and juvenile idiopathic arthritis. Journal of Allergy and Clinical Immunology 112:4 735-739, 2003
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 - HELDERS PJ, NIEUWENHUIS MK, VAN DER NJ, KRAMER PP, KUIS W, BUCHANAN TS: Displacement response of juvenile arthritic wrists during grasp. Arthritis Care Res 13:6 375-381, 2000
Organism: University Medical Center and Children's Hospital, Dept Pediatric Physical Therapy, Room # KB 020560, PO Box 85090, 3508 AB Utrecht, The NetherlandsFAU - Helders, P J
Abstract: OBJECTIVE: To analyze the displacement response of juvenile arthritic wrists during grasp in order to diagnose early ligamental laxity and facilitate early splinting. METHODS: X-rays of the wrists, made under standardized conditions, of 30 children with juvenile chronic arthritis (mean age 10.4 years, range 4.5-16.9) were analyzed after being digitalized. Osseous landmarks were identified, and coordinates were calculated from measured angles and lengths with an accuracy of 0.01'. Lunate and carpal-ulnar distance were obtained according to Youm, and ulnar variance according to Hafner. RESULTS: Overall, an increase in ulnar-lunate displacement and carpal narrowing and a decrease in ulnar variance were found. However, not all wrists responded to the same extent. Radial displacement of the lunate, though slight, was found in 2 wrists and the amount of ulnar displacement varied substantially (3.1% to 22.5%). The variance in amount of displacement could suggest that juvenile wrists do not respond to increased compressive forces to the same extent. CONCLUSION: The changes found are similar to those found in the healthy wrist. Furthermore, our findings suggest that the juvenile wrist acts in accordance with the generally accepted explanation for the development of malalignment in adult wrists. It seems that laxity of ligaments can be diagnosed early by the force grip maneuver during x-ray. It would have a significant impact on the moment of orthotic intervention as well as the design of the orthotic device. Further study along this line seems justified - HO C, WALTON DS: Goniosurgery for glaucoma complicating chronic childhood uveitis. ARVO Annual Meeting Abstract Search and Program Planner 2003: Abstract, 2003
Abstract: Purpose: To evaluate the efficacy of goniotomy in medically uncontrolled glaucoma secondary to chronic childhood uveitis. Methods: A retrospective review of goniosurgery performed by a single surgeon (DW) for childhood uveitic glaucoma refractory to medical therapy was done. 35 eyes of 27 patients treated over 30 years were included, forming the largest known reported series and longest follow-up. Success was defined as final intraocular pressure(IOP) less than 22mmHg without medications and qualified success as IOP less than 22mmHg with medications. Results: 49 goniotomies were performed in 35 eyes of 27 patients. 23 eyes had 1 goniotomy and 12 had 2 or 3 goniotomies. All except 6 patients were girls. Juvenile rheumatoid arthritis associated uveitis was the diagnosis in 77% of eyes and the others had idiopathic uveitis. 71% of eyes were phakic and 29% aphakic. The mean follow-up was 107.6 months (range, 2-324) and the mean age at first goniotomy was 10.8 years (range, 4.5-22). The mean pre-operative IOP was 36.6 +/- 7.0mmHg on a mean of 2.7 +/- 1.1 medications. Surgical success was achieved in 20(57%) eyes and qualified success in 5(14%) eyes on 1.8 +/- 1.3 medications, giving an overall success rate of 71%. This was achieved with a single goniotomy in 64%. The mean post-operative IOP in eyes with successful control was 14.5 +/- 3.1mmHg. Eyes with surgical failure showed 6.9 clock hours of synechial closure of the trabecular meshwork compared to 2.2 clock hours and 1.5 clock hours in eyes with qualified success and complete success respectively. Phakic eyes had an overall success of 84% compared to 40% in aphakic eyes. Hyphema occurred post-operatively in 82% of eyes which in most instances was mild and transient. Only 2 eyes with moderate hyphema associated with elevated IOP required washout. Deterioration of pre-exisitng cataracts in the early post-operative months occurred in 2 eyes of a single patient. There was no exacerbation of uveitis in the early post-operative period. No other significant complications occured. Conclusions: Goniosurgery is a safe and efficacious conjunctiva-sparing first surgical option for the treatment of refractory childhood uveitic glaucoma. The majority of patients do not require glaucoma medication after the procedure. The surgical success is adversely affected by greater amounts of synechial angle closure and aphakia. Aphakic patients have a success rate less than half that of phakic patients - JOHNSTON CA, STEELE RG, HERRERA EA, PHIPPS S: Parent and Child Reporting of Negative Life Events: Discrepancy and Agreement across Pediatric Samples. J Pediatr Psychol 28:8 579-588, 2003
Organism: University of Kansas, Memphis, Tennessee St Jude Children's Research Hospital, Memphis, TennesseeFAU - Johnston, Craig A
Abstract: OBJECTIVE: To examine the consistency in child and parent reporting of child's negative life events across child/pediatric samples. METHODS: A total of 613 child-parent dyads provided independent reports of negative life events. The pairs included three groups consisting of children who were healthy (n = 362), diagnosed with cancer (n = 130), and diagnosed with a chronic illness (juvenile rheumatoid arthritis, diabetes, or cystic fibrosis; n = 121). RESULTS: Children reported significantly more negative life events than their parents reported for them. Additionally, children in the chronically ill group self-reported significantly fewer negative life events than the other groups. However, parents of children with cancer reported significantly more negative life events than the other groups. Although discrepancies exist in all three samples, parents and children in the healthy group were significantly more discrepant than the other groups. CONCLUSIONS: These results suggest that communication of children's life events between parent and child may increase during children's experience of cancer or a chronic illness. However, significant discrepancies remain in child and parent report of negative life events. Because of this, clinicians are encouraged to recognize the strengths and limitations of using multiple reporters in assessing negative life events in children - KOTANIEMI K, SAVOLAINEN A, KARMA A, AHO KIMMO: Recent advances in uveitis of juvenile idiopathic arthritis. Survey Of Ophthalmology 48:5 489-502, 2003
- LAIHO K, SAVOLAINEN A, KAUTIAINEN H, KEKKI P, KAUPPI M: The cervical spine in juvenile chronic arthritis. Spine J 2:2 89-94, 2002
Organism: Rheumatism Foundation Hospital, FIN-18120 Heinola, Finland kari@laihoasFAU - Laiho, Kari
Abstract: BACKGROUND CONTEXT: In patients with juvenile chronic arthritis (JCA) the cervical spine is often affected, leading to pain and functional limitations. PURPOSE: To describe the frequency of the radiographic abnormalities in the cervical spine of a large series of patients with JCA, examined after skeletal maturity. STUDY DESIGN: Consecutive patients with JCA, who had cervical spine radiographs available taken at adult age (>18 years) were included in the study from one outpatient clinic and one rheumatology ward in the Rheumatism Foundation Hospital, Heinola, Finland. PATIENT SAMPLE: The series consisted of 159 patients fulfilling the diagnostic criteria of the European League Against Rheumatism for JCA. OUTCOME MEASURES: Evaluation of cervical spine radiographs for inflammatory changes. METHODS: Inflammatory changes in the cervical spine radiographs were measured as well as the size of the fourth cervical vertebra. Patient records were studied. The statistical analysis was calculated by Student's t-test or Mann-Whitney U test. RESULTS: In 98 cases (62%) some inflammatory changes were detected in the cervical spine. Apophyseal joint ankylosis was noted in 65 patients (41%), anterior atlantoaxial subluxation in 27 (17 %) and atlantoaxial impaction in 39 (25 %). The fourth cervical vertebra was abnormally small in 41 patients (26%). CONCLUSIONS: Radiographically, the most frequent inflammatory change in the cervical spine of patients with JCA was apophyseal joint ankylosis at multiple levels. Atlantoaxial impaction and anterior atlantoaxial subluxation were typical of the upper cervical spine. Clinically, these changes tend to limit neck movements. A small C4 vertebral body was seen in patients with early disease onset and short body stature - LEE YH, KIM HJ, RHO YH, CHOI SJ, JI JD, SONG GG: Functional polymorphisms in matrix metalloproteinase-1 and monocyte chemoattractant protein-1 and rheumatoid arthritis. Scand J Rheumatol 32:4 235-239, 2003
Organism: Division of Rheumatology, Department of Internal Medicine, College of Medicine, Korea University, Seoul, KoreaFAU - Lee, Y H
Abstract: The aim of this study is to investigate whether the functional polymorphisms in the promoter of matrix metalloproteinase-1 (MMP-1) and in the regulatory region of the monocyte chemoattractant protein-1 (MCP-1) gene are associated with susceptibility to rheumatoid arthritis (RA) and its clinical features. The MMP-1 1G/2G polymorphism and the MCP-1 promoter A/G polymorphism were determined by polymerase chain reaction-restriction fragment length polymorphism in 117 RA patients and 97 healthy controls. The genotype distribution of the MMP-1 promoter did not differ between RA patients and control subjects. However, in the 2G/2G genotype, ESR and Plat were higher than the 1G/1G genotype. The genotype distribution of the MCP-1 promoter did not differ between the RA and control groups. Clinically there was no significant difference among RA patients according to the MCP-1 promoter genotypes. Our data show that the functional promoter polymorphism in the MMP-1 promoter may not play an important role in the susceptibility of RA, but the polymorphism may be related to clinical phenotypes - LO R, V, FISHMAN NO, NACHAMKIN I: Recurrent catheter-related Rhodotorula rubra infection. Clin Microbiol Infect 9:8 897-900, 2003
Organism: Department of Medicine, Division of Infectious Diseases, University of Pennsylvania School of Medicine, University of Pennsylvania, Philadelphia, PA 19104-4283, USAFAU - Lo Re, V
Abstract: A 34-year-old male receiving chronic parenteral nutrition for treatment of short bowel syndrome and intermittent immunosuppressive agents for juvenile rheumatoid arthritis developed recurrent, catheter-associated Rhodotorula rubra fungemia over a one-year period. Infection with this yeast is associated with insertion of central venous catheters. Recurrence of R. rubra infection is an unusual event that presumably occurred because of chronic skin colonization by the organism - MANNERS P, LESSLIE J, SPELDEWINDE DEIRDRE, TUNBRIDGE D: Classification of juvenile idiopathic arthritis: Should family history be included in the criteria? Rinsho Ganka 30:8 1857-1863, 2003
Abstract: Objective: (i) To determine the efficacy of the Durban classification for children with juvenile idiopathic arthritis (JIA) where <5 joints were involved at onset (with systemic arthritis excluded) by determining the proportion of the cohort that proved to be "unclassifiable"; (ii) to define reasons for cases being "unclassifiable," particularly regarding family history; and (iii) to compare the efficacy of a proposed hierarchical system (an unofficial modification of the Durban classification) with the Durban classification, where family history details are included as descriptors, rather than as classification criteria. Methods: Charts were reviewed of 50 children with fewer than 5 joints involved at presentation for JIA, followed for at least 12 months, with systemic arthritis excluded. Cases were classified according to the EULAR criteria, the Durban criteria, and by a proposed "modified Durban" classification subject to hierarchy, with exclusions in the following order: systemic arthritis, rheumatoid factor (RF) positive arthritis, psoriasis or a combination of dactylitis and psoriatic nail changes (psoriatic arthritis), and HLA-B27 positive arthritis (enthesitis related arthritis), with the remainder of children being classified as having either RF negative polyarthritis or RF negative oligoarthritis, depending on number of joints involved, with additional information noted as descriptors. The "modified Durban" classification was proposed only to stimulate discussion among clinicians. Results: Of 50 children, 56% were "unclassifiable" by the Durban classification, mainly because of inadequate family history despite appropriate questioning. Using the proposed "modified Durban" classification, 2% were "unclassifiable." Family history was classified as inadequate for the following reasons: The parents did not know family history; the child or parent was adopted; the father was unknown or parent died early; parents never attended; extended family had lost communication with parents; or a relative was considered to have psoriasis, but not confirmed by dermatologists. Other reasons for "unclassifiable" included: dermatologists unable to confirm psoriasis; family history of inflammatory bowel disease and sacroiliitis but B27 status unknown; proband B27 negative but family history of B27-related disease; family history of psoriasis, but patient had insufficient criteria for psoriatic arthritis and therefore excluded from oligoarthritis, psoriatic arthritis and other groups. Conclusion: (i) The Durban classification showed poor efficacy for JIA where <5 joints were involved at onset, with more than half the cases being "unclassifiable". (ii) The most common reason was that appropriate family history was not available despite being sought by the clinician. (iii) A proposed hierarchical system, an unofficial modification of the Durban classification, showed good efficacy, with only one of 50 cases being "unclassifiable." - MUEHLHAEUSER U, MUENZEL F, ESCHENHAGEN T_(REPRINT): Etanercept
<Original> Etanercept. DMW Deutsche Medizinische Wochenschrift 128:34-35 1775-1778, 2003 - OGILVIE EM, FIFE MS, THOMPSON SD, TWINE N, TSORAS M, MOROLDO M, FISHER SA, LEWIS CM, PRIEUR AM, GLASS DN, WOO P: The -174G allele of the interleukin-6 gene confers susceptibility to systemic arthritis in children: a multicenter study using simplex and multiplex juvenile idiopathic arthritis families. Arthritis Rheum 48:11 3202-3206, 2003
Organism: University College London, London, UKFAU - Ogilvie, Emma M
Abstract: OBJECTIVE: Levels of interleukin-6 (IL-6) have been shown to correlate with the fever and disease activity of systemic juvenile idiopathic arthritis (JIA). In a previous case-control study, a significant association between the IL-6 -174 nucleotide variant and systemic JIA was noted, and HeLa cell transfection assays show functional differences in levels of transcription of the IL-6 -174 alleles. The present study was undertaken to confirm the previous findings and to assess possible association with variations of the A(n)T(n) tract in the promoter. METHODS: We studied a cohort of JIA families from 3 countries, using transmission disequilibrium testing. Genotyping of the -174 nucleotide variant was done by restriction fragment length polymorphism, heteroduplex analysis, or allelic discrimination. The A(n)T(n) tract at -392 to -373 was typed using DNA sequencing. Statistical analysis was performed using the programs Transmit and EHplus. RESULTS: There was a significant excess transmission of the -174G allele in the systemic JIA families (P = 0.041). The excess transmission was only to systemic JIA patients with age at onset >5 years (P = 0.007). No significant association with the other subtypes was found. No A(n)T(n) alleles or -174/A(n)T(n) haplotypes were significantly associated with systemic JIA. CONCLUSION: This study confirms that the IL-6 -174 nucleotide variant is significantly associated with systemic JIA. The significant excess transmission to patients with age at onset >5 years but not to those with age at onset < or =5 years suggests that there may be genetic heterogeneity between the 2 groups - QUINN MA, GREEN MJ, MARZO-ORTEGA H, PROUDMAN S, KARIM Z, WAKEFIELD RJ, CONAGHAN PG, EMERY P: Prognostic factors in a large cohort of patients with early undifferentiated inflammatory arthritis after application of a structured management protocol. Arthritis Rheum 48:11 3039-3045, 2003
Organism: Royal Adelaide Hospital, Adelaide, AustraliaFAU - Quinn, Mark A
Abstract: OBJECTIVE: Inflammatory arthritis of the hands is a frequent clinical presentation with a variable outcome. Patients not satisfying the classification criteria for recognized arthritides are described as having undifferentiated inflammatory arthritis, for which there are no accepted therapeutic algorithms. This study assessed the clinical outcome of patients with undifferentiated arthritis of the hands after use of a treatment algorithm, and evaluated the prognostic features in these patients. METHODS: One hundred consecutive patients with undifferentiated arthritis of the hands were assessed following use of a pragmatic treatment algorithm that was based on clinical presentation and response to treatment. The following standard step-up treatment protocol was used: 1) nonsteroidal antiinflammatory drugs (NSAIDs), 2) a single dose of corticosteroid administered by either intramuscular or intraarticular injection, and 3) disease-modifying antirheumatic drugs (DMARDs). Patients with specific rheumatologic diagnoses were excluded. The primary outcome was persistence of synovitis at 12 months. RESULTS: Seventy-eight percent of patients received NSAIDs, 72% received corticosteroids, and 30% received DMARD therapy. Among patients who had synovitis at 12 months, the prevalence of rheumatoid factor (RF) seropositivity, swollen joints, and synovitis at baseline was greater than in those without persistent synovitis. Logistic regression analysis showed baseline investigations to be poor predictors of subsequent DMARD use, with the best predictor being persistence of synovitis at 12 weeks. Rheumatoid arthritis (RA) developed in 14 patients. Logistic regression analysis showed that significant predictors of RA were RF seropositivity and the painful joint count at baseline. No patient who experienced resolution of synovitis by 12 weeks had persistent synovitis that subsequently required DMARD therapy. Only 13% of patients entered remission. Early resolution of synovitis was associated with an excellent prognosis. CONCLUSION: Undifferentiated arthritis of the hands is not a benign condition, with 30% of patients receiving DMARD therapy by 12 months and low remission rates. Results of the clinical assessment at 12 weeks is the single best predictor of future therapy. This study provides background data for use in determining future therapeutic interventions - RAVELLI A, MARTINI A: Early predictors of outcome in juvenile idiopathic arthritis. Clinical and Experimental Rheumatology (Italy ) 21:5 SUPPL. 31 S89-S93, 2003
Abstract: The definition and management of "early arthritis" in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. Over the past 3 decades a number of studies have evaluated the long-term outcome of cohorts of patients with JIA and some of them have also attempted to identify early prognostic factors. In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed - REINOSO BF: [In Process Citation]. An R Acad Nac Med (Madr) 120:2 297-305, 2003
Abstract: It is described the experience of the first 130 patients specifically treated by the first Spanish Unit of Chronic Pain in Children. The mean age of the patients was 7.6 years (SD 5.1). Sex distribution was 6:4 for male:female. The more frequents causes of pain were: Hematologic cancer (35%), neuropathic pain (15%) and juvenile chronic arthritis (12%). The mean duration of pain prior to pain treatment was 295 days. The main therapeutic option was treatment with opiate agents (79%) during a mean period of time of 111 days and mean decrease of 88.1% in pain intensity - ROBINSON RF, NAHATA MC, HAYES J, RENNEBOHM R, HIGGINS G: Quality-of-life measurements in juvenile rheumatoid arthritis patients treated with etanercept. Clinical Drug Investigation 23:8 511-518, 2003
Abstract: Objective: The aims of this study were: (1) to assess functional status, emotional well-being and quality of life in patients with polyarticular and systemic juvenile rheumatoid arthritis (JRA) treated with etanercept, and (2) to determine the prevalence and significance of adverse events associated with etanercept therapy. Patients and methods: All JRA patients (n=21) who received etanercept in our rheumatology clinic over a 14-month period were evaluated. Patient demographics, type of arthritis, dosing regimens, family history, measures of joint function and laboratory parameters were obtained for each patient. A questionnaire that comprised validated functional assessment and quality-of-life measures (the Childhood Health Assessment Questionnaire (CHAQTM), the Juvenile Arthritis Function Assessment Report (JAFAR 5TM) and the Pediatric Quality of Life Inventory Version 4 (PedsQL Generic ScaleTM) scales) was administered to patients and parents to assess physical and emotional function, pain, adverse drug events and quality of life at each clinic visit. Results: Functional status and quality of life improved in patients with polyarticular and systemic disease. A significant difference between pre- and post-etanercept functional assessment (JAFARTM and CHAQTM) and quality-of-life assessment by parents and patients was found (p=0.009, p=0.002, pltoreq0.001, pltoreq0.001, respectively). The JAFARTM results concurred with those of the CHAQTM test, and did not distinguish between patients with polyarticular and systemic disease. Laboratory parameters indicative of toxicity did not differ between patients with polyarticular and systemic JRA and the number of adverse events reported was low. Underlying disease did not appear to predict improvement. Conclusion: Etanercept appeared to improve functional status, emotional wellbeing, quality of life and activity level with minimal toxicity in patients with polyarticular and systemic JRA - ROSE AJ: Bone and joint diseases around the world. France: Rheumatoid polyarthritis, chronic juvenile idiopathic arthritis, and ankylosing spondylitis. A public health priority. Rinsho Ganka 30:Supplement 67 45-46, 2003
Abstract: Since its creation, the Association francaise des Polyarthrites is doing everything possible to come to the aid of people with polyarthritic diseases and to help medical research make advances against this illness. Each year, with the help of sponsors, we organize a national information campaign with first-hand accounts and presentations by sufferers on radio and television stations, so that polyarthritis should be better understood by the general public. Last year, together with other associations, we asked the Minister of Health about the barriers to receiving innovative treatment for polyarthritis because of the cost. Because of this action, the Minister has made additional funds available so that more sufferers can benefit from new treatment. Recently, several associations dealing with chronic and serious inflammatory rheumatic illnesses who came together as an action group presented a text to the Minister of Health about the urgent need to make these illnesses a public health priority. Working sessions between the Minister, patient associations, and rheumatologists to consider how to implement a public health plan are in progress - SMOLEWSKA E, BROZIK H, SMOLEWSKI P, BIERNACKA-ZIELINSKA M, DARZYNKIEWICZ Z, STANCZYK J: Erratum: Apoptosis of peripheral blood lymphocytes in patients with juvenile idiopathic arthritis (Annals of the Rheumatic Diseases (2003) 62 (761-763)). Annals of the Rheumatic Diseases (United Kingdom ) 62:11 1126, 2003
- SWALE VJ, PERRETT CM, DENTON CP, BLACK CM, RUSTIN MH: Etanercept-induced systemic lupus erythematosus. Clin Exp Dermatol 28:6 604-607, 2003
Organism: Department of Dermatology, Royal Free Hospital, London, UK vjswale@doctorsorgukFAU - Swale, V J
Abstract: Tumour necrosis factor (TNF) is a pro-inflammatory cytokine with a role in the pathogenesis of a number of conditions including rheumatoid arthritis, psoriasis, psoriatic arthritis, ankylosing spondylitis and Crohn's disease. Etanercept (Enbrel; Immunex Corp., Seattle, WA, USA) is a recombinant soluble fusion protein of TNF-alpha type II receptor and IgG which acts by blocking the action of TNF-alpha. It is licensed for use in rheumatoid arthritis and juvenile chronic arthritis. A number of studies report the development of antinuclear and anti-double-stranded DNA antibodies in patients treated with TNF antagonists for rheumatoid arthritis. There are few reports of the development of clinical features of discoid, subacute or systemic lupus erythematosus. We present one of the first reported cases of etenercept-induced systemic lupus erythematosus and review the literature of lupus and TNF antagonists - UMARJI SIM, LEE MB, GARGAN MF, PORTINARO NMA, LEARMONTH ID: Total hip arthroplasty in skeletal dysplasia. HIP International (Italy ) 13:3 177-183, 2003
Abstract: This study presents the results of 38 hip prostheses in 24 people of short stature (under 152 cm). A retrospective clinical and radiological study recording the diagnosis, age at reconstruction, height, weight, type of prostheses, length of follow-up, radiological appearances and patient satisfaction was performed. Harris hip scores were used to assess activities of daily living. All patients were under 152cm and their diagnoses included achondroplasia, spondyloepiphyseal dysplasia, multiple epiphyseal dysplasia, developmental dysplasia of the hip and juvenile chronic arthritis. The mean height of these patients was 135 cm (range: 109cm to 150cm). The mean age was 38 years (range: 19 to 75 years) with mean follow-up 67 months (range: 12 to 406 months). Only one patient, who is now aged 69 years (though 65 years at revision surgery), has required revision surgery to date. The results were excellent for 33 hips (Harris hip score between 80-100), good in three (Harris hip score between 70-80), satisfactory in one (score 60-70) and poor for one (Harris hip score <50). The mean Harris hip score to date is 89. Hip replacement surgery is difficult in this challenging group of patients but can nonetheless yield gratifying results in over 90% of cases - YAMAMOTO JH, BEZERRA EG, ABDALLA M_(REPRINT), HIRATA CE, DAMICO FM, KUBO P_(REPRINT), OLIVALVES E: Causes of uveitis in a tertiary center in sao paulo city, brazil. ARVO Annual Meeting Abstract Search and Program Planner 2003: Abstract, 2003
Abstract: Purpose: To analyze retrospectively the diagnosis of patients being followed up at the uveitis service of Hospital das Clinicas, University of Sao Paulo Medical School, during a 6 month-period, from February to August, 2002. Methods: 262 patients (P), who attended the uveitis service during that period, have their charts reviewed and diagnosis confirmed during the appointment. The following data were analyzed: age, gender, anatomic and etiological diagnosis, disease activity and ocular complications. Results: 90% of the patients had a follow-up longer than 1 year. 61% were female and 39% male. The mean age was 41 years (range 4-88); 10% were 0-16 years old , 42% were 17-40 years old and 48% were 41 or more. Distribution according to anatomic diagnosis was: anterior 20%, intermediate 4.5%, posterior 38 %, diffuse 31 %, and others 6.5%. Uveitis etiology was determined in 78% of the cases, and the most common causes were toxoplasmosis 22%, Vogt-Koyanagi-Harada syndrome (SVKH) 13%, Behcet's disease (BD) 10%, toxocariasis 4.5% and juvenile rheumatoid arthritis 4.5%. 40 patients had active uveitis. The most frequent ocular complications were cataract (41%), glaucoma (12%), posterior synechiaes (11%) and retinal detachment (6%). Infectious causes were present in 81% of posterior uveitis, whereas non-infectious systemic diseases were observed in 61% of anterior and 73% of diffuse uveitis. Uncommon diagnosis was observed such as vasculitis associated with antiphospholipid syndrome, HTLV-I/II related-uveitis, autoimmune retinopathy (CAR-like retinopathy). Conclusions: Epidemiological studies may reflect uveitis etiology change patterns in different places and population; nevertheless they also reflect the particular characteristics of each center (tertiary, diagnosis criteria, special interests, etc). The present study demonstrate the importance of endogenous uveitis such as SVKH and BD, as well as of infectious uveitis such as toxoplasmosis and toxocariasis - YELIN E, TRUPIN L, KATZ P, LUBECK D, RUSH S, WANKE L: Association between etanercept use and employment outcomes among patients with rheumatoid arthritis. Arthritis Rheum 48:11 3046-3054, 2003
Organism: Rosalind Russell Medical Research Center for Arthritis, University of California, San Francisco, California 94143-0920, USA yelin2@itsaucsfeduFAU - Yelin, Edward
Abstract: OBJECTIVE: To assess the association between use of etanercept and employment outcomes among patients with rheumatoid arthritis (RA). METHODS: In 1999, 497 RA patients of working ages (18-64 years) reported their employment status in the year of diagnosis and as of the study year, in structured telephone interviews. Of these, 238 had been in clinical trials of etanercept and were currently taking that medication, while 259 were members of an observational study and were not taking etanercept. We used regression techniques to estimate whether employment outcomes in 1999 (employed versus not and, among the employed, hours of work per week, weeks of work per year, and hours of work per year) among the 379 of the 497 patients who were employed at the time of diagnosis were associated with etanercept use, with and without adjustment for demographic characteristics, RA status, overall health status, and the nature of the job held at the time of diagnosis. RESULTS: At the time of diagnosis, 75% of RA patients from the observational study who did not take etanercept and 77% of those who did take the medication were employed. By 1999, among those employed at diagnosis, 55% of the former group and 71% of the latter were employed (difference 16 percentage points). After adjustment for demographics, overall health status, duration of RA, RA status, and occupation and industry, the difference widened to 20 percentage points. Among all who were employed at the time of diagnosis, those from the etanercept clinical trials worked an average of 5.4 more hours per week in 1999; after adjustment, the etanercept group worked 7.4 more hours per week. CONCLUSION: Among all persons who were employed at the time of RA diagnosis, having been in the etanercept clinical trials was associated with higher employment rates in 1999 and a greater number of hours per week of work in that year, suggesting that a randomized trial to establish the relationship between treatment and employment outcomes is now warranted