Bibliography November 05

1. AL BRAIKI AS: Efficacy of anti-TNF drugs in sever uveitis. IOVS 45:Suppl. 1 U983, 2004
   Abstract: Purpose: To determine the effectiveness of anti-TNF drugs in the management ofsevere uveitis.Methods: Patients with uveitis were recruited who had failed to control their intraocular inflammation or complications such as macular oedema, with a maintenance dose of corticosteroids of 10mg/day or less, plus immumosuppressive agents such as cyclosporin (5mg/kg/day), mycophenolate (1g bd) or methotrexate (10-15mg/week). Patients with a history of or who were considered high risk of previous exposure to TB were excluded. Either infliximab (5mg/kg in an infusion) or etanercept (25 mg s/c twice weekly) were given. The aim was to control the inflammation and allow steroid dose reduction to 10mg/day or less. Where possible other agents such as cyclosporin and mycophenolate were also reduced and/or discontinued. The length of time taken for disease relapse if it occurred was identified.Results: Eight patients met the inclusion criteria- 3 females, 5 males all Caucasian. 3 patients had an associated systemic disease - one each of juvenile idiopathic arthritis, ankylosing spondylitis and Reiter's disease. 4 patients were given a single dose of infliximab, I bad two doses of Infliximab (within 10 month interval when the inflammation relapsed). 3 had etanercept (one for 11months and two for 21 months). Six months after initiating medication with anti-TNF the mean dose of prednisolone was reduced in all patients (16+/-2.9SE vs 9.3+/-7.2SE)p=0.042.5 patients were able to discontinue second line immunosuppressive at 3 months. Recurrence of inflammation (AC/VC 2+) occurred in 3 patients; one with posterior uveitis when steroid dose was 5mg and Cellcept 250mgOD 10 months after infliximab. He had a second Infliximab dose and the inflammation settled. The second patient with Reiters disease had Etanercept for 11 months and steroids were discontinued. He developed a severe disease relapse and high dose steroids was restarted. The third patient has a mild relapse whensteroid dose was below 15g and is awaiting his 2(nd)dose of Infliximab. All the other patients have remained stable for a mean follow-up 12.8 months (5-22 months) with controlled inflammation at a steroid dose of 10mg or less (two patients on 10mg,two patients on 5mg and one patient is off steroids).Conclusions: Anti-TNF therapy is a useful addition to central inflammation in situations where other agents may not have been effective. Regular infusions of infliximab may not be necessary, as with FA and still allows control of the inflammation for 4-5 months with significant reduction of the steroid dose to 10 mg or less without the need for second line immunosuppressive agents

2. BAARS RM, ATHERTON CI, KOOPMAN HM, BULLINGER M, POWER M: The European DISABKIDS project: development of seven condition-specific modules to measure health related quality of life in children and adolescents. Health Qual Life Outcomes 3:1 70, 2005
   Abstract: Backgroud: The European DISABKIDS project aims to enhance the Health Related Quality of Life (HRQoL) of children and adolescents with chronic medical conditions and their families. We describe the development of the seven cross-nationally tested condition-specific modules of the European DISABKIDS HRQoL instrument in a population of children and adolescents. The condition-specific modules are intended for use in conjunction with the DISABKIDS chronic generic module. METHODS: Focus groups were used to construct the pilot version of the DISABKIDS condition-specific HRQoL modules for asthma, juvenile idiopathic arthritis, atopic dermatitis, cerebral palsy, cystic fibrosis, diabetes and epilepsy. Analyses were conducted on pilot test data in order to construct field test versions of the modules. A series of factor analyses were run, first, to determine potential structures for each condition-specific module, and, secondly, to select a reduced number of items from the pilot test to be included in the field test. Post-field test analyses were conducted to retest the domain structure for the final DISABKIDS condition-specific modules. RESULTS: The DISABKIDS condition-specific modules were tested in a pilot study of 360 respondents, and subsequently in a field test of 1152 respondents in 7 European countries. The final condition-specific modules consist of an 'Impact' domain and an additional domain (e.g. Worry, Stigma, Treatment) with between 10 to 12 items in total. The Cronbach's alpha of the final domains was found to vary from 0.71 to 0.90. CONCLUSIONS: The condition-specific modules of the DISABKIDS instrument were developed through a step-by-step process including cognitive interview, clinical expertise, factor analysis, correlations and reliabilities. A cross-national pilot and field test were necessary to collect these data. In general, the internal consistency of the domains was satisfactory to high. In future, the DISABKIDS instrument may serve as a useful tool with which to assess HRQoL in children and adolescents with a chronic condition. The condition-specific modules can be used in conjunction with the DISABKIDS chronic generic module

3. BELLINTANI C, GHIRINGHELLI P, GERLONI V, GATTINARA M, FARRONATO G, FANTINI F: [Temporomandibular joint involvement in juvenile idiopathic arthritis: treatment with an orthodontic appliance]. Reumatismo 57:3 201-207, 2005
   Organism: Cattedra e Reparto di Ortognatodonzia, Clinica Odontoiatrica, Universita degli Studi di Milano bellintanicla@liberoitFAU - Bellintani, C
   Abstract: OBJECTIVE: About 65% of children suffering from juvenile idiopathic arthritis (JIA) shows a more or less marked involvement of temporo-mandibular joint (TMJ) with altered mandibular growth, resorption of the condyles, occlusary instability, reduced chewing ability and facial dysmorphia. The purpose of our study is to prevent and to treat the progressive evolution of JIA on craniofacial growth and morphology with a functional appliance; surgery should be considered only in so far as the adequacy of TMJ movement is concerned. METHODS: From 1992 until now 72 children with proved JIA and TMJ involvement have been treated (50 females, 22 males, aged 6 to 16 years old). TMJ involvement was bilateral in 61% and unilateral in 39% of patients. A diagnostic workup was carried out involving tomograms of TMJ and cephalometric radiograph and analysis. The authors used a bimaxillary activator in the attempt to modify the unfavourable growth pattern and provide a gradual ante-rotation of the jaw. RESULTS: Almost all JIA patients showed satisfactory long term results, easing of pain, reduced skeletal discrepancy, increased function and good facial profile. CONCLUSIONS: The long term results of this study indicate that orthopaedic therapy might control the vicious circle of the malocclusion in children with JIA, preventing exacerbation of mandibular clockwise rotation. Surgical intervention for the improvement of TMJ function should be considered only if a severe restricted state is imminent

4. BIESTER S: Etanercept in the treatment of chronic anterior uveitis in children. IOVS 45:Suppl. 1 U982, 2004
   Abstract: Purpose: Chronic anterior uveitis in children often takes a serious course. Despite various immunosuppressive drugs some children do not sufficiently respond with a high risk of becoming severely disabled. Until now anti TNF alpha therapy for uveitis has been used in a few children, but the results were not ambiguous. Hem we report of 19 patients treated with Etanercept for uveitis, mostly associated with juvenile onset arthritis.Methods: In a retrospective study 19 patients were treated with Etanercept (0.4 mg/kg body weight, twice a week, subcutaneously); 17 also had juvenile idiopathic arthritis, in 2 children no underlying disease was found. The age variedfrom 13-25 years (mean age 14.6 years). Patients were included when the previous antiinflammatory therapy was not effective. This consisted of systemic steroids (n=19), Cyclosporin A (n=18), Methotrexate (n= 19), Azathioprine (n= 15), Cyclophosphamide (n= 2), Immunoglobulins (n= 1) and Mycophenolat mofetil (n=2). The grading for uveitis was effective: no relapse or more than 2 relapsesless than before treatment, mild: one relapse less than before treatment, no response: no change in relapse rate, worsening: more relapses under treatment than before. The grading for arthritis (depending on the clinical findings) was: very effective, effective, mild, no response, worsening.Results: For arthritis the response to Etanercept was very effective in 6 patients, effective in 7 patients, mildly effective in 2 patients, not respond ing in I patient and became worse in I patient. For uveitis the response to this treatment was mild in 7 patients, 9 patients did not respond, and 3 patients even worsened their course of uveitis (new relapses after years).Conclusions: Our results show that the anti TNF alpha drug Etanercept was veryeffective or at least mildly effective in 15 of 17 (88%) patients for their rheumatoid arthritis. In contrast for uveitis our study shows that only a mild response was found in 37%, not resulting in a completely quiet eye for a longer time, but 47 % of patients did not respond at all. Even worse was, that 3 patients (16 %) experienced new exacerbations of their uveitis after along period of quiet disease. So our study suggests that for patients with juvenile uveitis Etanercept may not be very helpful. While highly effe ctive for arthritis, this study confirms previous results, suggesting that Etanercept can even provoke uveltis

5. CHAN AY, LIU DT: Uveitis in young adults with juvenile idiopathic arthritis. Ann Rheum Dis 64:12 1808, 2005

6. DAVIS PJ, HACKETT J, JOHNSON K, MCDONAGH JE: Joint restriction in an unhappy teenager. Ann Rheum Dis 64:12 1786-1787, 2005

7. DE JAGER WK: High levels of IL18 in systemic juvenile idiopathic arthritis. Immunobiology 209:4-6 300, 2004

8. DE K, I, VASTERT B, KLEIN M, TEKLENBURG G, ARKESTEIJN G, PUGA YG, ALBANI S, KUIS W, WULFFRAAT N, PRAKKEN B: Autologous stem cell transplantation for autoimmunity induces immunologic self-tolerance by reprogramming autoreactive T-cells and restoring the CD4+CD25+ immune regulatory network. Blood .: 2005
   Organism: Department of Pediatric Immunology, University Medical Center Utrecht, 'Wilhelmina Children's Hospital', Utrecht, The Netherlands
   Abstract: Despite a rapidly accumulating clinical experience with autologous stem cell transplantation (ASCT) as a treatment for severe refractory autoimmune disease, data on the mechanisms by which ASCT induces immune tolerance are still very scarce. In this study it is shown that ASCT restores immunologic self-tolerance in Juvenile Idiopathic Arthritis (JIA) via two mechanisms. First, ASCT induces a restoration of the frequency of FoxP3 expressing CD4+CD25(bright) regulatory T-cells (Tregs) from severely reduced numbers before ASCT to normal levels after ASCT. This recovery is due to a preferential homeostatic expansion of CD4+CD25+ Tregs during the lymphopenic phase of immunereconstitution, as measured by Ki67 and CD44 expression, and to a renewed thymopoiesis of naive mRNA FoxP3 expressing CD4+CD25+ Tregs after ASCT. Second, using artificial-Antigen-Presenting-Cells to specifically isolate self-reactive T-cells we demonstrate that ASCT induces autoimmune cells to deviate from a proinflammatory phenotype (mRNA IFN-gamma and T-bet high) to a tolerant phenotype (mRNA IL-10 and GATA-3 high). These data are the first to demonstrate the qualitative immunological changes that are responsible for the induction of immune tolerance by ASCT for JIA; the restoration of the CD4+CD25+ immune regulatory network and reprogramming of autoreactive T-cells

9. DUARTE C, GOMES C, CORREIA AJ, SALGADO M: Renal amyloidosis: an uncommon complication of juvenile idiopathic arthritis. Clin Rheumatol :1-2.: 1-2, 2005
   Organism: Unidade de Nefrologia do Hospital Pediatrico de Coimbra, Avenida Bissaya Barreto, 3000, Coimbra, Portugal, carolduarte@netcabopt
   Abstract: A 9-year-old girl presented with systemic-onset juvenile idiopathic arthritis, diagnosed at 3.5 of age and which was difficult to control despite several therapeutic trials. Five years after diagnosis of juvenile idiopathic arthritis, nephrotic proteinuria was noticed. Renal biopsy confirmed the diagnosis of amyloidosis, and chlorambucil was initiated, with general improvement of the disease and reduction of proteinuria

10. FALL NDATE, BOVE KEVIN E, STRINGER KEITH, LOVELL DANIEL: Association between lack of angiogenic response in muscle tissue and high expression of angiostatic ELR-negative CXC chemokines in patients with juvenile dermatomyositis. Arthritis & Rheumatism 52:10 3175-3180, 2005
    Abstract: Objective. To investigate the relationship between the vasculopathy of juvenile dermatomyositis (juvenile DM) and the balance between the angiostatic ELR- and angiogenic ELR+ CXC chemokines in the muscle of patients with the disease.Methods. The expression of 3 ELR- CXC chemokines (interferon-inducible protein 10 [IP-10], monokine induced by interferon-gamma, and interferon-inducible T cell a-chemoattractant) and 2 ELR+ CXC chemokines was quantitated in muscle biopsy samples from 7 patients with juvenile DM and 7 healthy children, by real-time polymerase chain reaction. The findings were correlated with various histopathologic features, with particular emphasis on the degree of vasculopathy. Synovial biopsy specimens from patients with juvenile rheumatoid arthritis (JRA) were used for additional comparison.Results. The angiostatic ELR- chemokines were expressed at high levels, while the angiogenic ELR+ chemokines were barely detectable, in most juvenile DM samples. This contrasted sharply with the findings in both normal muscle biopsy specimens and JRA synovial tissue specimens. The expression of the ELR- chemokines in juvenile DM samples correlated with the intensity of mononuclear cell infiltration. Furthermore, the juvenile DM samples with the highest degree of capillary loss had the highest levels of ELR- CXC chemokines. The presence of IP-10 in juvenile DM muscle specimens was confirmed by immunohistochemistry analysis. In addition, immunohistochernical staining of muscle tissue revealed that CXCR3, a receptor utilized by ELR- CXC chemokines, was expressed in vascular endothelial cells.Conclusion. Increased expression of the interferon-induced angiostatic ELR- CXC chemokines is a feature of juvenile DM that parallels the degree of vasculopathy in patients with the disease. Collectively, these findings are consistent with a model in which a subset of inflammatory cells secrete angiostatic ligands, which then contribute to a local atrophying effect on the muscle's vasculature via a receptor-mediated process

11. GRAHAM TB: Imaging in juvenile arthritis. Current Opinion in Rheumatology (United States ) 17:5 574-578, 2005
    Abstract: Purpose of review: The purpose of this review is to highlight recent developments in imaging in juvenile arthritis. Recent findings: The developments in imaging in juvenile arthritis are primarily focused on evaluation of destructive changes and inflammatory changes in joints. Plain radiography can demonstrate destructive changes in juvenile arthritis. The most validated instrument for assessing destructive changes juvenile arthritis is the Poznanski index, and this index is being used more in studies to understand the natural history and clinical correlates of destructive disease. Magnetic resonance imaging has been shown to be superior to plain radiography in demonstrating destructive changes. Further work is proceeding to detect earlier, biochemical changes in articular cartilage prior to the development of thinning or erosion. Magnetic resonance imaging and ultrasound can demonstrate both inflammatory and destructive changes. Utilization of these techniques to show inflammatory changes can provide information about joints that can supplement physical examination, particularly in difficult joints to examine, such as the hips, temporomandibular joints, small joints of the feet, and tenosynovial locations. This information may help to guide therapy. Summary: Imaging provides useful information to supplement clinical and laboratory examination in the optimal treatment of patients with juvenile arthritis. (c) 2005 Lippincott Williams & Wilkins

12. GRAINGER R, HARRISON A: TNF inhibitors for inflammatory arthritis in New Zealand. N Z Med J 118:1224 U1706, 2005
    Organism: Wellington Regional Rheumatology Unit, Hutt Hospital, Lower Hutt, New Zealand RebeccaGrainger@huttvalleydhborgnzFAU - Grainger, Rebecca
    Abstract: For the vast majority of the estimated 100,000 New Zealanders who suffer from rheumatoid arthritis (RA), relatively inexpensive disease-modifying antirheumatic drugs (DMARD) regimens are sufficient to control inflammatory disease and maintain long-term function. Some DMARDs have been shown to slow, but not arrest, the progression of erosions. All but a few of those who suffer from ankylosing spondylitis (AS) can manage full social participation with non-steroidal anti-inflammatory drugs (NSAIDs) and an exercise regimen. For the small subset of arthritis sufferers who have disabling pain and progressive damage from uncontrolled inflammatory disease, the advent of the biological era offered great promise. In most of the developed world, this promise is being delivered to patients with an expanding range of diseases including RA, AS, and psoriatic arthritis, but central government (PHARMAC) funding for TNF inhibitors in New Zealand has until recently been limited to etanercept for approximately 40 patients with juvenile inflammatory arthritis

13. HAMMERSCHMIDT Y, MULLER H: [Nursing in juvenile idiopathic arthritis: not making the illness the center of care]. Pflege Z 58:10 626-627, 2005
    Organism: Kinderkrankenschwestern im Klinikum Stuttgart, OlgahospitalFAU - Hammerschmidt, Yvonne

14. HOUGHTON K, MALLESON P, CABRAL D, PETTY R, TUCKER L: Primary Sjogren's syndrome in children and adolescents: are proposed diagnostic criteria applicable? J Rheumatol 32:11 2225-2232, 2005
    Organism: Division of Rheumatology, Department of Pediatrics, University of British Columbia, British Columbia's Children's Hospital, Vancouver, Canada khoughton@cwbccaFAU - Houghton, Kristin
    Abstract: OBJECTIVE: To compare the proposed criteria for the diagnosis of primary Sjogren's syndrome (pSS) in childhood to the validated American-European Consensus Group (AECG) classification criteria for pSS in adults. METHODS: Charts of 7 children with pSS seen at British Columbia's Children's Hospital (BCCH) and data on 128 children identified through Medline in the English language literature between 1963 and 2003 were reviewed for pediatric and AECG criteria for pSS. The presence of > or = 4 criteria was required to satisfy the respective classification criteria. The expert clinical opinion of pediatric rheumatologists was considered the gold standard for diagnosis. RESULTS: A total of 24/62 (39%) cases satisfied the AECG criteria; 47/62 (76%) satisfied the proposed pediatric criteria. Inclusion of recurrent parotitis increased the sensitivity of the pediatric clinical criteria. From the cases, 78/133 (59%) satisfied the pediatric oral symptom criteria; only 6/78 (8%) had xerostomia in the absence of recurrent parotitis. There was no reported case of recurrent conjunctivitis in the absence of keratoconjunctivitis sicca. We found 101/130 (78%) cases had at least one positive autoantibody test result [antinuclear antibodies (ANA), rheumatoid factor (RF), SSA, SSB]; 78/123 (63%) had autoantibodies to SSA or SSB. CONCLUSION: The AECG adult criteria for pSS should not be applied to children as the sensitivity is unacceptably low. The inclusion of recurrent parotitis increases the sensitivity of the pediatric criteria, and recurrent parotitis should alert the clinician to the possibility of pSS. The inclusion of recurrent conjunctivitis did not improve the sensitivity over the AECG ocular criteria. The addition of ANA and RF to the AECG criteria did not change the number of patients satisfying the criteria for pediatric pSS

15. HRAFNKELSDOTTIR K, .KAMPHUIS: Identification of novel pan HLA DR binding MMP-3 epitopes in children with uveitis and juvenile idiopathic arthritis. Immunobiology 209:4-6 364, 2004

16. HUR MINA, KIM YOUNG CHUL, LEE KYU MAN, KIM KWANG NAM: Macrophage activation syndrome in a child with systemic juvenile rheumatoid arthritis. Journal of Korean Medical Science 20:4 695-698, 2005
    Abstract: Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumatic disorders in children. We describe a 13-month-old boy in whom MAS developed as a complication of systemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, coagulopathies resembling disseminated intravascular coagulation, anemia and thrombocytopenia. Hyperplasia of hemophagocytic macrophages was remarkable in his bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvements. This is the third case of MAS associated with S-JRA in Koreans, and the first one, in which hemophagocytic macrophages were proven in bone marrow

17. KELES I, AYDIN G, TOSUN A, INAL E, KELES H, ORKUN S: Familial Mediterranean fever and ankylosing spondylitis in a patient with juvenile idiopathic arthritis: a case report and review of the literature. Rheumatol Int :1-6.: 1-6, 2005
    Organism: Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Kirikkale University, Kirikkale, Turkey
    Abstract: The association of familial Mediterranean fever (FMF) with juvenile idiopathic arthritis (JIA) or ankylosing spondylitis (AS), most commonly with negative HLA-B27 antigen, was described in several previous reports, although the pathogenic mechanism of this association still remains unknown. Herein we report an uncommon association of FMF with HLA-B27 positive AS as an occasional coincidence in a patient who had been diagnosed as having JIA 23 years previously

18. KIM EC: A retrospective review of infliximab for ocular inflammation. IOVS 45:Suppl. 1 U983, 2004
    Abstract: Purpose: To evaluate the clinical usefulness of infliximab (Remicade (R)), an anti-TNF-alpha inhibitor, in patients with inflammatory eye disease that was found to be resistant to conventional immunosuppressive medications.Methods: Fifteen patients (10 males and 5 females aged 16-66 years) who received infliximab primarily for either inflammatory eye disease or joint disease were studied retrospectively to determine the effects of infliximab on their ocular inflammation. Main outcome measures included degree of inflammation and symptoms and visual acuity before and after treatment and side eff ects of remicade treatment.Results: Nine patients with uveitis (including anterior and panuveitis), 5 patients with scleritis, and 1 patient with ocular cicatricial pemphigoid(OCP) were treated with infliximab. Systemic diagnoses included adult and juvenile rheumatoid arthritis (n=4), ankylosing spondylitis (n=2), Behcet's disease (n=4),and OCP (n=1). Four patients had ocular inflammation without associated systemic disease. Indications for infliximab treatment were systemic and/or joint inflammation unresponsive to other anti-rheumatic drugs in 4 patients (infusionevery 5-8 weeks) and ocular inflammation resistant to conventional immunomodulatory agents including methotrexate, cyclosporin A, mycophenolate mofetil, and prednisone in 11 patients (infusions every 2-4 weeks). The mean number of months of follow-up per patient was 10.4 months. Eight of 14 patients with ocular inflammation experienced improvement in ocular inflammation. Two of 14 had stable or no change in inflammation. Two of 14 had worsened inflammation over the course of treatment. Three of 14 patients received only one recent dose of infliximab therefore evaluation of inflammation was not available. Visual acuity was stable in 11 patients, slightly worse in 2 patients, and significantly im proved in 2 patients. There were no adverse side effects due to infliximab. Two patients discontinued use of inflixiamb due to insurance noncoverage issues.Conclusions: Infliximab is a well-tolerated and possibly effective alternativefor managing certain types of recalcitrant ocular inflammatory diseases including uveitis and scleritis, as well as their associated inflammatory arthrtides

19. KIM JW, KIM JS: A case of cutaneous lupus erythematosus presenting with facial annular erythemas preceded by juvenile rheumatoid arthritis. Korean Journal of Dermatology (South Korea ) 43:8 1089-1093, 2005
    Abstract: Annular erythema associated with anti-Ro and/or anti-La antibodies in patients with Sjogren syndrome (SS)/lupus erythematosus (LE) overlap syndrome or SS, has recently been described as a distinct clinical entity in Orientals. It may be a counterpart of annular subacute cutaneous LE, (SCLE) seen in Caucasians. We report a 12-year-old girl with facial annular erythemas in a setting of anti-Ro/La positive cutaneous LE, preceded by juvenile rheumatoid arthritis. Through the pertinent laboratory workup, we were able to exclude the possibility of SS/LE overlap syndrome, SS or SCLE. Another clinical variant might exist in the disease spectrum of annular erythema which is associated with anti-Ro/La antibodies

20. KVIEN TK, HEIBERG, LIE E, KAUFMANN C, MIKKELSEN K, NORDVAG BY, RODEVAND E: A Norwegian DMARD register: prescriptions of DMARDs and biological agents to patients with inflammatory rheumatic diseases. Clin Exp Rheumatol 23:5 Suppl 39 S188-S194, 2005
    Organism: Department of Rheumatology, Diakonhjemmet Hospital, Box 23 Vinderen, N-0319 Oslo, Norway tkkvien@medisinuionoFAU - Kvien, T K
    Abstract: Information concerning the effectiveness of drug therapy cannot be obtained only from randomized controlled clinical trials, due to limitations such as a short time frame and narrow inclusion and exclusion criteria. Therefore, complementary longitudinal observational studies performed in a real life setting are required. NOR-DMARD, a Norwegian 5-center register, was established in December 2000. All DMARD prescriptions to patients with inflammatory arthropathies are included, and patients are followed longitudinally with a variety of assessments. As of 2005, 4683 DMARD regimens have been included. Methotrexate is the most commonly used DMARD in rheumatoid arthritis and psoriatic arthritis. The proportions of patients who have received anti-TNF drugs in rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, juvenile arthritis and other diseases have been 22.5, 21.6, 53.8, 36.9 and 9.7%, respectively. The proportion of patients receiving anti-TNF drugs is considerably higher in 2004 than earlier, and criteria for prescribing anti-TNF drugs appear to be trending toward patients with less severe and active disease. Confounding by indication or channeling bias represents a challenge for the group comparisons of longitudinal effectiveness data, but can be addressed by modern statistical techniques. The NOR-DMARD register may in the future provide comparative real life effectiveness data that may also be used in cost-effectiveness analyses

21. LOPES MC: Sleep cyclic alternating pattern in children with juvenile idiopathic arthritis. Sleep (Rochester) 28:Suppl. S A102-A103, 2005

22. LUKACZER D, DARLAND G, TRIPP M, LISKA D, LERMAN RH, SCHILTZ B, BLAND JS: A pilot trial evaluating Meta050, a proprietary combination of reduced iso-alpha acids, rosemary extract and oleanolic acid in patients with arthritis and fibromyalgia. Phytother Res 19:10 864-869, 2005
    Organism: Clinical Research at the Functional Medicine Research Center, Gig Harbor, WA 98332, USA DanLukaczer@metagenicscomFAU - Lukaczer, Daniel
    Abstract: The aim of this open-label, 8-week observational trial was to investigate the efficacy of Meta050 (a proprietary, standardized combination of reduced iso-alpha-acids from hops, rosemary extract and oleanolic acid) on pain in patients with rheumatic disease. Osteoarthritis, rheumatoid arthritis and fibromyalgia patients were given 440 mg Meta050 three times a day for 4 weeks, which was changed to 880 mg twice a day for the subsequent 4 weeks in the majority of patients. Pain and condition-specific symptoms were assessed using a standard visual analog scale (VAS), an abridged arthritis impact measurement scale (AIMS2) and the fibromyalgia impact questionnaire. Fifty-four subjects with rheumatic disease completed the trial. Following treatment, a statistically significant decrease in pain of 50% and 40% was observed in arthritis subjects using the VAS (p < 0.0001; Wilcoxon-ranked sums) and AIMS2 (p < 0.0001), respectively. Fibromyalgia subject scores did not significantly improve. A decreasing trend of C-reactive protein, a marker for inflammation, was also observed in those subjects who presented with elevated C-reactive protein. No serious side effects were observed. These observations suggest that Meta050 at a dosage of 440 mg three times a day has a beneficial effect on pain in arthritis subjects

23. MAHESH A, PANCHAPAKESA RC, PORKODI R, VASANTHY S, BALA MS: Camptodactyly - A case report. Indian Journal of Practical Pediatrics (India ) 7:3 261-263, 2005
    Abstract: Camptodactyly is a noninflammatory condition characterized by soft tissue tightening without limitation of flexion. This rare condition can be mistaken for juvenile idiopathic arthritis. Non tender swelling and minimal flexion contracture of proximal inter phalangial joint of fingers, commonly involving fifth digit but also other fingers except thumb. It may be associated with familial arthritis and other disease states

24. MEHOLJIC-FETAHOVIC A: [Complex functional test in juvenile rheumatoid arthritis]. Med Arh 59:6 373-375, 2005
    Organism: Pedijatrijska Klinika, Klinicki Centar Univerziteta u Sarajevu MEHOLJICAJSA@HOTMAILCOMFAU - Meholjic-Fetahovic, Ajsa
    Abstract: The Juvenile rheumatoid arthritis is a special form of rheumatoid arthritis which occurs in childhood and adolescence (before the age of sixteen). The disease primarily affects the joints, but can also cause heavy damages to organs and systems such as the heart, blood vessels, skin, epidermis, eyes, peripheral nerves etc. The disease occurs more often with girls. In the therapeutic sense, viewed from the physical medicine and rehabilitation aspect, lying in bed is needed during the acute phase of the disease, and when the inflammation ceases, normal body activities should be encouraged. Rehabilitation with standard methods of physical therapy includes enhancement of daily activities and quality of life, adaptation of patient to the new conditions and prevention of complications. At the Physical medicine and rehabilitation department of the Pediatric clinic CCU Sarajevo, during year 2001 and 2002, fourteen children were observed. All children were treated with kinezitherapy, after which everyday life activities were observed as well as the mobility. In these everyday life activities we were observing whether child can use spoon, fork and knife, whether or not a child can dress his/her upper or lower part of the body with or without buttoning, whether or not it can bathe without someone's help. While observing mobility we evaluated general mobility with changing the body's position from lying to sitting and also from sitting to standing position, climbing onto and getting of a bed, ascending and descending stairs, ability to crouch down or not. These methods are used to prevent inability or decrease it as much as possible. It is accomplished by preventing the forming of muscular atrophies, preventing the development of contractions of joints, as well as preventing the development of osteoporosis and bone destruction due to the inactivity. The imperative of a good rehabilitation is "active movement"

25. MILLER-HOOVER S: Juvenile Idiopathic Arthritis: Why Do I Have to Hurt So Much? J Infus Nurs 28:6 385-391, 2005
    Organism: Suzan Miller-Hoover is a pediatric clinical nurse specialist at Banner Children's Hospital, Mesa, Arizona
    Abstract: In the current world of high-tech medicine, some individuals nevertheless experience immeasurable chronic pain daily. Among these are 60,000 to 70,000 children in the United States who have juvenile idiopathic arthritis. The children affected by this disease experience a decreased quality of life and sometimes blindness. Outcome severity in these cases is related directly to the amount of injury to bones, joints, soft tissue, and eyes at the time of diagnosis. There are not enough specialists to ensure timely diagnosis and medications, and although the symptoms of juvenile idiopathic arthritis are treatable, there is no cure. Until timely diagnosis and treatment can be ensured, pain and blindness will continue to be the daily experience of these children

26. MRSIC M: Diagnosis and treatment of Gaucher disease
    DIJAGNOZA ILIJECENJE GAUCHEROVE BOLESTI. Paediatria Croatica (Croatia ) 49:3 167-173, 2005
    Abstract: Gaucher disease (Morbus Gaucher) is the most common lysosomal storage disorder. Incidence of the disease is around 1:40-60 000 individuals and it is assumed that in Croatia we have 20-30 patients with Morbus Gaucher. The cause of this storage disorder is an autosomal recessive inherited glucocerebrosidase deficiency, which, depending upon the residual activity of the enzyme, results in a more or less decreased breakdown of the sphingolipids. The disease is extremely serious, disabling and can be mortal in the near or distant future without treatment. Morbus Gaucher is recognized in three types. Type I or what is known as the non-neuronopathic form is most common in Europe while the neuronopathic forms (type II and III) are much more rare (5-10%). Types II and III are characterized by central nervous system involve ment and are usually diagnosed in childhood. Disease manifestations are observed in various human organs. The most common disease features involve the liver, spleen, bones, lungs and brain. Morbus Gaucher is a lysosomal storage disorder and disease manifestation can be observed in almost all human organs. Diagnosis of Morbus Gaucher is easily established by measuring enzyme glucocerebrosidase activity in leukocytes or fibroblasts. Disease activity can be predicted by measuring the chitotriosidase level in sera. Enzyme replacement therapy with imiglucerase (Cerezyme(R)) is now the therapeutic gold standard. Imiglucerase prevents progressive manifestation of the disease and patients have a normal life. The cost of the treatment is high due to the cost of the recombinant technology, which is used to produce imiglucerase. The cost of the enzyme replacement therapy is 150-200,000 EUR per year for imiglucerase in a typical adult patient. Due to the high cost of patient treatment the Ministry of Health of Republic of Croatia, and the Croatian Health Insurance Company established a special program in 2002 for what they called "Expensive drug treatment". This program covers treatment costs for patients with inherited metabolic disorders, adenosine deaminase deficiency, chronic myeloid leukemia, AIDS, multiple sclerosis, juvenile arthritis and ovarian cancer. So far 11 adult patients with Gaucher disease have been diagnosed in Croatia, a 7 of them are on enzyme replacement therapy with imiglucerase. According to our experience administration of imiglucerase has decreased spleen and liver size and the number of bone pain crises, as well as normalization of platelet and red blood cells. Administration of the imiglucerase does no reverse bone changes e.g. avascular hip necrosis or vertebra collapses, but prevents further bone deterioration. Accordingly, treatment with imiglucerase should be started immediately after establishing diagnosis to prevent irreversible changes to human organs

27. RAMANAN AV, GROM AA: Does systemic-onset juvenile idiopathic arthritis belong under juvenile idiopathic arthritis? Rheumatology (United Kingdom ) 44:11 1350-1353, 2005
    Abstract: 'Science is the systematic classification of experience' George Henry Lewes (1817-78), English philosopher, critic, dramatist, scientist. Juvenile idiopathic arthritis (JIA) is prevalent in about 1 in 1000 children. The earliest formal description of this disease was by Sir George Frederick Still in 1897 [1]. This work was done when he was a registrar at the Hospital for Sick Children, Great Ormond Street, London [2]. In this initial description of 19 patients he identified three patterns of arthritis, one of which came to be known later as Still's disease [now known as systemic-onset juvenile idiopathic arthritis (SoJIA)]. Over the next few decades it came to be app reciated that one form of arthritis in children is very different and dominated by the presence of systemic manifestations. Over the last two decades several paediatric rheumatologists have come together to classify juvenile arthritis for purposes of better disease identification and research. All along, the systemic form of juvenile arthritis was always recognized as belonging to a distinct group; in fact for several decades (and even now in some countries) the systemic form of juvenile arthritis was referred to as Still's disease. In this article we will attempt to highlight the reasons why we feel that SoJIA is perhaps not best retained in the company of JIA. (c) The Author 2005 Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved

28. RONAGHY A, .ZONNEVELD-HUIJSSOON: Meningococcal C vaccination does not aggravate Juvenile Idiopathic Arthritis and induces specific antibody titers. Immunobiology 209:4-6 359, 2004

29. SAHIN O, KARABAY O, PARLAK AH, TAMER A: A case with adult still's disease who was treated with indomethacin and review of treatment approaches
    INDOMETAZINLE TEDAVI EDILEBILEN ERISKIN STILL OLGUSU VE TEDAVI YAKLASIMLARININ LITERATUR ISIGINDA DEGERLENDIRI LMESI. Journal of Rheumatology and Medical Rehabilitation (Turkey ) 16:1 64-69, 2005
    Abstract: Adult Still's disease (ADS) is a rare condition, usually presenting with high fever accompanied by systemic manifestations. Non steroid anti inflammatory drugs (NSAID) and steroids are used for first-line therapy. In patients that are nonresponsive to this therapy or those that require high dose steroid, disease-modifying antirheumatic drugs such as methotrexate (MTX), gold salts, cyclophosphamide, cyclosporine- A and azathioprine (AZA) can be used. The efficacy of interleukin 1 (IL -1) inhibitors and tumor necrosis factor-alpha (TNF alpha) blockers in chronic inflammatory disease like rheumatoid arthritis, ankylosing spondylitis and juvenile chronic arthritis is pointed. Recently studies show that these agents can be used successfully also in the patients who are resistent for conventional therapy. Here, it is aimed to investigate the new and conventional treatment approaches of ASD in the light of literature via presentation of a 51 year old female case with ASD that was treated with indomethacin

30. SAVAGE MO, CIANFARANI S: IGFs and IGFBPs in GH insensitivity
    <BOOK> Endocrine Development
    <Series> Endocrine development. <SERIES> 9: 100-106,
    Abstract: IGF-I, IGFBP-3 and ALS are GH-dependent peptides and their production is disturbed in states of GH insensitivity. This chapter explores the relative degrees of IGF-I, IGFBP-3 and ALS deficiency across the spectrum of GH insensitivity. In classical GH insensitivity syndrome (GHIS), known as Laron syndrome, due to GH receptor (GHR) deficiency, serum IGF-I, IGFBP-3 and ALS are severely reduced with inability to produce these peptides during an IGF-I generation test. Across the spectrum of severity of GHR defects, some patients have short stature and normal facial appearance, so-called partial or non-classical GH insensitivity. In these cases the IGF-I, IGFBP-3 deficiency is less severe. A positive relationship exists between height SDS and IGFBP-3 SDS (r(2) = 0.45, p < 0.001) in patients from the European series with GHIS. In a new series of GHIS cases (n = 36) there was a significant difference in IGFBP-3 and ALS (p < 0.05) between classical (n = 25) and non-classical cases (n = 11). IGF-I, IGFBP-3 and ALS were significantly higher (p < 0.05) in pubertal compared with pre-pubertal subjects in the same series. In idiopathic short stature (ISS), heterozygous mutations of the GHR may have a dominant negative effect. ISS patients have lower IGF-I levels than the normal population. In 21 cases, mean IGF-I SDS was -1.39 (-2.4 to -1.16) and IGFBP-3; -0.45 (-1.13 to 0.38). However, IGF-I and IGFBP-3 responses in the IGF-I generation test were generally normal. In acquired GHI due to chronic illness such as Crohn's disease, juvenile arthritis and cystic fibros is, IGF-I deficiency is present, although IGFBP-3 is usually normal. In summary, assessment of IGF-I, IGFBP-3 and ALS contributes to diagnosis in GH insensitivity states. In our experience, IGF-I is more sensitive to disturbance of GH action that IGFBP-3, however in severe GHIS cases, IGF-I is usually undetectable and measurement of IGFBP-3 is valuable as a guide to the severity of the biological defect. Copyright (c) 2005 S. Karger AG, Basel

31. SCHEPP CPH: Effect of TGF-beta 1 on T cells in juvenile idiopathic arthritis. Immunobiology 209:4-6 432, 2004

32. SHI J, KOVACS SJ, WANG Y, LUDDEN TM, BHARGAVA VO: Population pharmacokinetics of the active metabolite of leflunomide in pediatric subjects with polyarticular course juvenile rheumatoid arthritis. J Pharmacokinet Pharmacodyn 32:3-4 419-439, 2005
    Organism: Global Biopharmaceutics, Drug Metabolism and Pharmacokinetics, Aventis Pharmaceuticals, Bridgewater, NJ, USA, junshi@FRXcomFAU - Shi, Jun
    Abstract: Leflunomide is a pyrimidine synthesis inhibitor used in the treatment of rheumatoid arthritis. Data from two clinical studies were used to establish a population pharmacokinetic (PPK) model for the active metabolite (M1) of leflunomide in patients with juvenile rheumatoid arthritis (JRA) and determine appropriate pediatric doses. Seventy-three subjects 3-17 years of age provided 674 M1 concentrations. The PPK model was derived from nonlinear mixed-effects modeling and qualified by cross-study evaluation and predictive check. A one-compartment model with first-order input described M1 PPK well. Body weight (WT) correlated weakly with oral clearance (CL/F = 0.020.[WT/40](0.430)) and strongly with volume of distribution (V/F = 5.8.[WT/40](0.769)). Steady-state concentrations (C(ss)) of M1 in JRA were compared for a variety of leflunomide dose regimens using Monte-Carlo simulation. To achieve comparable C(ss) values in pediatric patients with JRA to that in adult patients, doses of leflunomide should be adjusted modestly: 10 mg/d for 10-20 kg, 15 mg/d for 20-40 kg, and 20 mg/d for > 40 kg

33. SMITH SD: Trabeculectomy in children with Juvenile Idiopathic Arthritis (JIA)-associatcd uveitic glaucoma. IOVS 45:Suppl. 1 U985, 2004
    Abstract: Purpose: To describe outcomes of trabeculectomy with anti-metabolites in children with Juvenile Idiopathic Arthritis (JIA)-associated uveitis.Methods: Retrospective review of patients 14 years or younger with JIA-associated uveitic glaucoma who underwent trabeculectomy with or without phacoemulsification cataract surgery with posterior chamber intraocular lens implantation (CE/PC-IOL) between January 1996 and June 2002. Patients underwent trabeculectomy when the intraocular pressure (IOP) was greater than 28 mm Hg on maximal medical therapy and cataract surgery when the best corrected vision was 20/100 or less.Results: Eight children (7 female, 1 male) with a median age of 10 years at the time of surgery were included in this study. Ten eyes underwent trabeculectomy with antimetabolite (2 children had bilateral surgery and 3 eyes had CE/PC-IOL at the time of trabeculectomy surgery). Median duration of uveitis before glaucoma surgery was 3.1 years. Seven of eight children were on systemic immunosuppression for an average of 2.4 years before surgery. All patients receivedintensive and prolonged post-operative topical corticosteraid and immunosuppressive therapy. Seven of 10 eyes had surgical success; five of 10 eyes had complete success defined as IOP < 22 turn Hg without medications. Three eyes failed (failure is defined as IOP > 21 turn Hg with topical medications). Pre-operative median IOP was 34 mm Hg compared to the post-operative median IOP of 15.5 mm. All 3 eyes that underwent combined trabeculectomy and CE/PC-IOL had final vision >= 20/25.Conclusions: Trabeculectomy surgery can be successful in patients with JIA-associated uveitis with appropriate patient selection and adequate pre- and post-operative control of inflammation

34. TRUSZKOWSKI K, NIWALD M, NIEWIADOMSKA-JAROSIK K, SMOLEWSKA E, BROZIK H, STANCZYK J: Atrial ejection force (AEF) - Early parameter of the left-verticle dysfunction of in children with systemic autoimmune connective tissue disorders
    SIl(stroke)A WYRZUTU LEWEGO PRZEDSIONKA (AEF) JAKO WCZESNY WSKAZNIK DYSFUNKCJI LEWEJ KOMORY W PRZEBIEGU CHOROB TKANKI l(stroke)ACZNEJ U DZIECI. Pediatria Polska (Poland ) 80:9 740-744, 2005
    Abstract: Systemic autoimmune connective tissue disorders such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), dermatomyositis often also affect circulatory system. In adult patients suffering from collagenosis, many changes in the cardiovascular system have been recorded. Occult dysfunction of the left ventricle has often been described in patients in the early stage of the disease without evident manifestation of the heart damage. In the majority of patients, left ventricular diastolic filling abnormalities have been found to precede the impairment in left ventricular systolic function. AEF (atrial ejection force) correlates with the systolic function of left atrium and may be a good indirect parameter defining the left ventricular diastolic function. All of the data that has been presented so far concerned exclusively adults. The aim of the study was to determine if AEF is considerably increased in children suffering from various forms of connective tissue disorders. Patients and methods. A group of 36 children suffering from SLE (n = 7), RA (n = 4), JRA (juvenile rheumatoid arthritis) (n = 23), dermatomyositis (n = 2) was examined. The mean age in this group was 13.25 (+/- 1.4). The control group consisted of 20 healthy children. The mean age in this gro-up was 14.9 (+/- 1.1). Results. The AEF mean value in each group of patients was: for SLE 12.89 (+/- 2.74), for RA 4.39 (+/- 3.35), for JRA 7.5 (+/- 1.54). In the control group the mean AEF value was 6.75 (+/- 0.76) which in comparison with patients with LE and RA was a statistically significant difference. The difference between the AEF values in the control and JRA groups was not significant. Furthermore, in the patient, group of a reverse wave was recorded in the mitral valve in 10 patients, and liquid in the pericardium was found in 4. Conclusions. AEF is significantly higher in children with SLE, which might be due to compensation of left ventricle dysfunction. In the group of patients suffering from JRA, considerable changes in the examined parameters were not recorded. This maybe result from a m ilder and slower natural history of the disease. AEF may be a good, reliable parameter of left ventricle dysfunction in children with connective tissue diseases

35. VASTERT SJH: Drug-induced reduction of perforin in cd16brightcd56dim natural killer cells. Immunobiology 209:4-6 294, 2004

36. WALSH NC, CROTTI TN, GOLDRING SR, GRAVALLESE EM: Rheumatic diseases: the effects of inflammation on bone. Immunol Rev 208:228-51.: 228-251, 2005
    Organism: Beth Israel Deaconess Medical Center, New England Baptist Bone and Joint Institute, Harvard Institutes of Medicine, Boston, MA 02115, USAFAU - Walsh, Nicole C
    Abstract: Rheumatoid arthritis, juvenile idiopathic arthritis, the seronegative spondyloarthropathies including psoriatic arthritis, and systemic lupus erythematosus are all examples of rheumatic diseases in which inflammation is associated with skeletal pathology. Although some of the mechanisms of skeletal remodeling are shared among these diseases, each disease has a unique impact on articular bone or on the axial or appendicular skeleton. Studies in human disease and in animal models of arthritis have identified the osteoclast as the predominant cell type mediating bone loss in arthritis. Many of the cytokines and growth factors implicated in the inflammatory processes in rheumatic diseases have also been demonstrated to impact osteoclast differentiation and function either directly, by acting on cells of the osteoclast-lineage, or indirectly, by acting on other cell types to modulate expression of the key osteoclastogenic factor receptor activator of nuclear factor (NF) kappaB ligand (RANKL) and/or its inhibitor osteoprotegerin (OPG). Further elucidation of the mechanisms responsible for inflammation-induced bone loss will potentially lead to the identification of novel therapeutic strategies for the prevention of bone loss in these diseases. In this review, we provide an overview of the cell types, inflammatory mediators, and mechanisms that are implicated in bone loss and new bone formation in inflammatory joint diseases

37. WERTHEIM MS: Heterogeneity of keratic precipitates revealed by in vivo confocal imaging. A new diagnostic tool? IOVS 45:Suppl. 1 U987, 2004
    Abstract: Purpose: Formation of keratic precipitates (KP) is a characteristic finding invarious forms of intraocular inflammation, including uveitis and corneal transplant rejection. Clinically KP may take a limited number of forms distinguished by size and distribution across the corneal endothelium. We evaluated the heterogeneity of KP in subtypes of uveitis by scanning confocal microscopy, a technique providing 500-fold magnification.Methods: Cornea and KP were viewed with an ASL-1000 Scanning Confocal Microscope (Advanced Scanning Ltd, New Orleans, LO) in patients (n=35) presenting to atertiary referral uveitis service with immune-mediated and infectious forms of uveitis, including HLA B27-associated, sarcoidosis, Vogt-Koyanagi-Harada syndrome, juvenile idiopathic arthritis, Fuchs' heterochromic iridocyclitis, CMV retinitis, herpetic anterior uveitis, ocular toxoplasmosis and idiopathic uveitis. Images were captured and digitalized in real time.Results: Keratic precipitates were successfully imaged by this non-invasive confocal microscopy system. Three-dimensional views were rendered from z-stacks of certain images. The KP could be classified into distinct morphological patterns including: smooth rounded, globular, dendritiform, cruciform and stellate. In general, in any given patient, the morphology of KP was consistent acrossthe endothelium. Likewise, patients with a given disease tended to have KPs with similar morphologies. Patients with infections demonstrated KP architecture usually distinct from a non-infectious process.Conclusions: Scanning confocal microscopy has been used for the first time to describe the anatomy of KP in uveitis. Such observations reveal a heterogeneity that could not be ap preciated by conventional slit lamp microscopy and may have diagnostic relevance

38. WOO P, WILKINSON N, PRIEUR AM, SOUTHWOOD T, LEONE V, LIVERMORE P, WYTHE H, THOMSON D, KISHIMOTO T: Open label phase II trial of single, ascending doses of MRA in Caucasian children with severe systemic juvenile idiopathic arthritis: proof of principle of the efficacy of IL-6 receptor blockade in this type of arthritis and demonstration of prolonged clinical improvement. Arthritis Res Ther 7:6 R1281-R1288, 2005
    Organism: Great Ormond Street Hospital, NHS Trust, London, UK patriciawoo@uclacukFAU - Woo, Patricia
    Abstract: Eighteen Caucasian (white, Middle East and Asian) children diagnosed by paediatric rheumatologists in the UK and France as having systemic juvenile idiopathic arthritis (sJIA) were enrolled in this open label, single dose trial. All patients had evidence of continued symptoms and disease activity for at least three months while receiving >0.2 mg/kg/day of prednisolone, or its equivalent, prior to recruitment. Twelve patients also received methotrexate (< or =20 mg/m2/week). The patients were divided into three groups receiving 2, 4 or 8 mg/kg of MRA (tocilizumab) by intravenous infusion. No evidence of dose-limiting toxicity was observed and there were no dose-limiting safety issues. MRA appeared to be dramatically effective, with clinical and laboratory responses observed by 48 h post infusion, and these improvements continued well after serum MRA was undetectable. Eleven patients achieved the JIA definition of improvement (at least 3 of 6 core set criteria with a 30% improvement and no more than one worsened by 30%) and eight achieved > or =50% improvement. There were no observable differences with age. Clinical improvement in these children was observed for up to eight weeks, supporting the hypothesis that IL-6 is a key cytokine in the upregulation of genes crucial in the inflammation processes of sJIA, and the possibility of sequestration of MRA in the extra-vascular compartment needs to be considered

39. YAMAGISHI F: [Chemoprophylaxis for tuberculosis]. Kekkaku 80:10 647-653, 2005
    Organism: Department of Respiratory Diseases, National Hospital Organization Chiba-East National Hospital, 673 Nitona-cho, Chuo-ku, Chiba-shi, Chiba 260-8712, Japan yamagisf@chibaehospgojpFAU - Yamagishi, Fumio
    Abstract: In Japan, a person who is 29 or younger, contacted a tuberculosis patient recently and judged to have received infection is the object of the chemoprophylaxis by the public expenditure. On the other hand, in ATS/CDC, they call chemoprophylaxis as the treatment of latent tuberculosis infection, and if there is a strong possibility that a person may appear tuberculosis, they start chemoprophylaxis, regardless of age. We usually prescribe isoniazid for latent tubercular infection. The period of prescription is at least 6 months, which is effective, but 9 months is more desirable. Recently, in the West, tuberculosis are frequent among patients who are administered Infliximab, anti-tumor necrosis factor-alpha. In Japan, after 6 months' evaluation period for Infliximab medication to rheumatoid arthritis, 11 out of 2,000 became tuberculosis (100,000 to 550). As for the tuberculin reaction before Infliximab medication, 2 were un-carrying out and 4 were negative. Moreover, even in positive examples, many of them were small redness. From this result, the Japanese Society for Tuberculosis and the Japan College of Rheumatology advised jointly. Those who are using immunosuppressant drugs and a doctor judged that they need chemoprophylaxis are supposed to the start medication if they are positive in tuberculin reaction, or there are some proof of tubercular infection on chest X-ray film, or there are high possibility of having received tubercular infection. By carrying out active chemoprophylaxis regardless of age, tuberculosis from high risk group is expected to decrease

40. ZANNIN ME: Early methotrexate treatment in JIA-related uveitis. IOVS 45:Suppl. 1 U982, 2004
    Abstract: Purpose: The treatment of chronic uveitis in Juvenile Idiopathic Arthritis (JIA) is still a challenge for ophthalmologists. Methotrexate (MTX), widely used in JIA, has been recently introduced as a second-line agent for the treatment of sight-threatening uveitis. We report the preliminary results of a prospective study on the early MTX treatment of uveitis in children with oligoarticularJIA.Methods: Patients with severe course uveitis lasting less than 6 months have been treated with MTX at a standard dose of 10 mg/m(2)/week orally (group 1). The control group consisted in patients with severe long-standing eye involvement treated with MTX at the same dosage (group 2). Data on demographics, number of relapses, ocular complications and drug-related side effects werereported.Results: Nine patients (6F, 3M) entered the study. In all but one uveitis was detected before or at onset of arthritis. The age at onset of uveitis was comparable in both groups and ranged between 21 and 93 months. The follow-up period ranged between 10 and 128 months. In Group 1, including 6 patients, mean disease duration was 1.5 months, mean age was 49 months and mean follow-up was 16.8 months. Group 2, including 3 patients, received MTX treatment between 72 and 108 months after the onset of uveitis, mean age 120-192 months and follow-up38.6 months. In Group 1, only one patient experienced no relapse during the study period; the remaining patients had 1 to 4 relapses. In the second group, 2 patients presented 1 and 2 relapses, respectively and one patient had 5 relapses. Two cases in each group developed synechiae. One case, included in Group2, progressed towards a panuveitis despite of treatment. No major side effects during the MTX treatment have been reported in both groups.Conclusions: To our knowledge this represents the first prospective study on the early MTX treatment for JIA-related uveitis. Our preliminary data suggest that MTX does not significantly influence the course of uveitis in JIA even if introduced earlier. Further randomized studies on larger series are needed to confirm this observation