Bibliography February 2008

1. AARNTZEN EHet BARRERA P: Short digits...what's up?, Arthritis Rheum., Vol. 57(8), 1568-1571., 2007
   Organism:St Radboud University Medical Centre, Nijmegen, The NetherlandsFAU - Aarntzen, E H J G
   Abstract:

2. ABRAHAMYAN L, JOHNSON SR, BEYENE J, SHAH PSet FELDMAN BM: Quality of randomized clinical trials in juvenile idiopathic arthritis, Rheumatology (Oxford)., Vol. ., 2008
   Organism:The Hospital for Sick Children, Department of Health Policy Management & Evaluation, Department of Child Health Evaluative Sciences, Department of Public Health Sciences and Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada
   Abstract:Objectives. We evaluated the quality of randomized clinical trials (RCTs) of therapy for juvenile idiopathic arthritis (JIA) using an individual component approach and assessed temporal changes. Methods. A systematic review of the literature was performed to identify all RCTs involving exclusively JIA patients. Two investigators independently assessed the identified articles for six quality indicators: generation of allocation sequence, allocation concealment, masking, intention-to-treat (ITT) analysis, dropout rates and clearly stated primary outcome. Results. Fifty-two RCTs involving JIA patients were assessed. Generation of allocation sequence was unclear in 79% of the studies. Reporting of allocation concealment was adequate in only one-third of the studies. Masking was adequate in 73%, inadequate in 19% and unclear in 8% of the reports. ITT analysis was employed in 37% of the reports. Per-protocol analysis was used in 40% and in 23% the method was unclear. Most of the reports (67%) had dropout rates </=20%. About half of the reports (n = 25) failed to show a significant effect of the experimental treatment. No significant associations were found between the study results and quality indicators. With the exception of adequate masking and dropout rate, all quality indicators showed a trend of improvement over the decades. Conclusions. The quality of RCTs in JIA based on the selected indicators was poor. Although there were some positive changes over time, the reporting and methodological quality of trials should be improved. New, more powerful and acceptable RCT designs should be developed in this patient population

    

3. ALLANTAZ F, CHAUSSABEL D, BANCHEREAU Jet PASCUAL V: Microarray-based identification of novel biomarkers in IL-1-mediated diseases, Curr.Opin.Immunol., Vol. 19(6), 623-632., 2007
   Organism:Baylor Institute for Immunology Research and Baylor Research Institute, Dallas, TX, USAFAU - Allantaz, Florence
   Abstract:Interleukin 1b (IL-1b) is emerging as mediator of a wide range of human diseases. Availability of IL-1 blockers that result in clinical benefits to patients with these diseases is creating a demand for biomarkers to diagnose as well as to predict and follow responses to therapy. Blood gene expression profiling can be used to identify such biomarkers. This review will summarize recent studies in the field and will discuss some of the challenges raised by the use of this technology in biomarker discovery

    

4. BEHRENS EM: Macrophage activation syndrome in rheumatic disease: What is the role of the antigen presenting cell?, Autoimmun.Rev., Vol. 7(4), 305-308., 2008
   Organism:Department of Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA, USAFAU - Behrens, Edward M
   Abstract:Macrophage Activation Syndrome (MAS), alternatively referred to as secondary hemophagocytic lymphohistiocytosis (HLH), is a complication of many rheumatic diseases, most commonly Systemic Juvenile Idiopathic Arthritis (SJIA). MAS consists of a fulminant picture of pan-cytopenia, hectic high fevers, hepatosplenomegaly, lymphadenopathy, rash, and central nervous systemic inflammation. It can arise from genetic defects in the cytotoxic effector response of CD8+ T-cells, resulting in an inability to terminate antigen presentation, which in turn leads to uncontrolled immune activation. However, in the case of most rheumatic diseases, no such defect in cytotoxic killing is present. Little is known about what the contributions from the antigen presenting cells are in the pathogenesis of MAS. In fact, macrophages may be playing a regulatory, anti-inflammatory in MAS. We review the proposed pathogenesis of MAS/HLH, what role macrophages may play in the disease, and the relationship of MAS to its most common associated rheumatic disease, SJIA

    

5. BOLT IB, CANNIZZARO E, SEGER Ret SAURENMANN RK: Risk Factors and Longterm Outcome of Juvenile Idiopathic Arthritis-Associated Uveitis in Switzerland, J.Rheumatol., Vol. ., 2008
   Organism:From Zurich University Children's Hospital, Zurich, Switzerland
   Abstract:OBJECTIVE: To determine rate, risk factors, and longterm outcome of uveitis in children with juvenile idiopathic arthritis (JIA) in Switzerland and compare the results with a study of a different center in Switzerland from 1992. METHODS: Retrospective analysis of the charts and ophthalmologists' reports of all patients with JIA in a tertiary care outpatient clinic between January 1, 1997, and December 31, 2005, for diagnosis, course, and outcome of uveitis. RESULTS: Uveitis occurred in 35/265 patients (13.2%) of our JIA cohort, which is similar to the 16% reported in the 1992 cohort. A positive test for antinuclear antibodies was the strongest risk factor. The JIA subgroup with the highest rate of uveitis was "other arthritis," followed by oligoarticular JIA. Extended and persistent course of oligoarticular JIA had a similar uveitis incidence, but all patients with extended-course disease developed uveitis before more than 4 joints were affected. After a mean followup of 5.62 years (range 0.5-15.17), 12/35 (34%) patients with uveitis had developed uveitis complications. Best corrected visual acuity was normal in 91% of patients. Only 5.6% of the affected eyes were legally blind as compared to 17.6% in the 1992 cohort. CONCLUSION: The rate of uveitis was 13.2% in our cohort of Swiss children and has not changed since 1992. Despite the high rate of uveitis complications, the longterm visual outcome was excellent

    

6. CHAN SM, HUDSON Met WEIS E: Anterior and intermediate uveitis cases referred to a tertiary centre in Alberta, Can.J.Ophthalmol., Vol. 42(6), 860-864., 2007
   Organism:Department of Ophthalmology, Royal Alexandra Hospital, Vancouver, BC stanleychan@ualbertacaFAU - Chan, Stanley M
   Abstract:BACKGROUND: We investigated the characteristics and causes of various uveitis subtypes in patients presenting to the Regional Eye Centre at the Royal Alexandra Hospital, University of Alberta, Edmonton, Alta., and estimated the incidence of anterior uveitis in northern Alberta. METHODS: A retrospective study was conducted of all patients presenting with uveitis to a single, full-time ophthalmologist at the Regional Eye Centre from September 2004 to June 2005. Uveitis was classified according to onset, severity, anatomical subtype, etiology, recurrence rate, and response to treatment. Statistical analysis was used to compare patients referred by ophthalmologists with those referred by non-ophthalmologists. RESULTS: Two hundred and nine eyes of 171 patients were included in the study. Ophthalmologist referrals consisted of 67.4% anterior, 14.0% intermediate, and 18.6% panuveitis, and non-ophthalmological referrals were 92.8% anterior, 5.4% intermediate, and 1.8% panuveitis. Referrals from ophthalmologists were significantly more likely to be chronic, recurrent, and (or) less responsive to treatment than referrals from other sources. INTERPRETATION: Referral bias strongly affects the proportions of uveitis subtypes seen. Human leukocyte antigen-B27-associated diseases (especially ankylosing spondylitis), sarcoidosis, and herpes infections should be considered among the most likely causes of uveitis to be diagnosed in this patient population

    

7. CHEN CC, ISOMOTO H, NARUMI Y, SATO K, OISHI Y, KOBAYASHI T, YANAGIHARA K, MIZUTA Y, KOHNO Set TSUKAMOTO K: Haplotypes of PADI4 susceptible to rheumatoid arthritis are also associated with ulcerative colitis in the Japanese population, Clin.Immunol., Vol. 126(2), 165-171., 2008
   Organism:Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, JapanFAU - Chen, Chun Chuan
   Abstract:Inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn's disease (CD), is a chronic inflammatory disorder characterized by intractable inflammation specific to the gastrointestinal tract. The precise etiology of IBD remains unknown. Recently, haplotypes of peptidylarginine deiminase type 4 (PADI4) have been identified as the rheumatoid arthritis (RA)-susceptible gene. PADI4 is located at 1p36, which is one of chromosomal loci susceptible for IBD. Then, we examined whether haplotypes and diplotypes of PADI4 are associated with IBD in the Japanese population. We studied haplotypes of PADI4 in 114 patients with UC, 83 patients with CD, and 200 gender-matched healthy controls by PCR-restriction fragment length polymorphism. Frequencies and distributions of haplotypes and diplotypes were compared statistically between patients and controls by logistic regression analysis. The frequency of haplotype 1 was significantly decreased in patients with UC, compared to that in controls (P=0.037; odds ratio (OR)=0.702). In contrast, the frequency of haplotype 2 in patients with UC was significantly higher than that in controls (P=0.003; OR=1.722). Moreover, of a total of 114 patients with UC, 15 (13.2%) had a diplotype homozygous for haplotype 2, the frequency being significantly higher than in controls (9/200, 4.5%; P=0.008, OR=3.215). Our results indicate that haplotype 1 of PADI4 is associated with non-susceptibility to UC, whereas haplotype 2 is susceptible to UC. Thus, it is likely that PADI4 is one of genetic determinants of UC in the Japanese population

    

8. GAJENDRAN VK, PETERSON B, SLATER RR, Jr.et SZABO RM: Long-term outcomes of dorsal intercarpal ligament capsulodesis for chronic scapholunate dissociation, J.Hand Surg.[Am.]., Vol. 32(9), 1323-1333., 2007
   Organism:Department of Orthopaedic Surgery, University of California, Davis, Sacramento, CA 95817, USAFAU - Gajendran, Varun K
   Abstract:PURPOSE: Chronic scapholunate dissociation is the most common cause of symptomatic wrist instability. In an attempt to restore normal carpal mechanics and prevent wrist arthrosis, we developed and tested biomechanically the dorsal intercarpal ligament capsulodesis (DILC). Previously, we reported good early clinical results for this procedure at an average follow-up period of 25 months. Here, we report on the functional and radiographic outcomes at a longer follow-up period of a minimum of 5 years. METHODS: Records of patients undergoing the DILC for chronic (greater than 6 weeks), flexible, static scapholunate dissociation were reviewed. Only patients with follow-up evaluation of greater than 60 months were included. Physical examination, radiographs, and validated outcome instruments were used to evaluate the patients. RESULTS: Twenty-one patients (22 wrists) met the inclusion criteria. Fifteen of 21 patients (16 wrists) were available for follow-up evaluation. Average follow-up period was 86 months. Physical examination revealed average wrist flexion and extension of 50 degrees and 55 degrees , respectively, radial and ulnar deviation of 17 degrees and 36 degrees , respectively, and grip strength of 43 kgf. Disabilities of the Arm, Shoulder and Hand, Short Form-12, and Mayo wrist scores averaged 19, 78, and 78, respectively. Radiographs revealed an average scapholunate angle and gap of 62 degrees and 3.5 mm, respectively. Eight of the 16 wrists in our study demonstrated arthritic changes on radiographs. CONCLUSIONS: The DILC does not consistently prevent radiographic deterioration and the development of arthrosis in the long-term; however, the level of functionality and patient satisfaction remained relatively high in 58% of our patients, suggesting a lack of correlation between the radiographic findings and development of arthrosis and the functional outcomes and patient satisfaction. We believe that the DILC is still a reasonable option for treating flexible static scapholunate dissociation in patients without radiographic signs of arthritis presenting with wrist pain despite conservative treatment. Prevention of radiographic deterioration and arthrosis remains an unsolved problem

    

9. GARTLEHNER G, HANSEN RA, JONAS BL, THIEDA Pet LOHR KN: Biologics for the treatment of juvenile idiopathic arthritis: a systematic review and critical analysis of the evidence, Clin.Rheumatol., Vol. 27(1), 67-76., 2008
   Organism:Ludwig Boltzmann Institute for Health Technology Assessments, Garnisongasse 7/20, 1090, Vienna, Austria gartlehner@schsrunceduFAU - Gartlehner, Gerald
   Abstract:Biologics are an important therapeutic option for treating patients with juvenile idiopathic arthritis (JIA). In adults, they are associated with rare but severe adverse events such as serious infections and malignancies. We reviewed systematically the evidence on the efficacy and safety of biologics for the treatment of JIA. We searched electronic databases up to August 2006. We limited evidence to prospective studies for efficacy but included retrospective observational evidence for safety. Outcomes of interest were clinical response, radiographic progression, quality of life, and adverse events. One randomized controlled trial (RCT) and 11 uncontrolled prospective studies provided data on efficacy; three additional studies assessed safety. The only RCT and six uncontrolled trials support the general efficacy of etanercept for the treatment of JIA. Internal and external validity of these studies are limited. The evidence on other biologic agents such as adalimumab, abatacept, anakinra, infliximab, rituximab, and tocilizumab is sparse or entirely missing. Because of the lack of sound long-term safety data, evidence is insufficient to draw firm conclusions about the balance of risks and benefits of any biologic agent for the treatment of JIA. Clinicians have to be aware of the lack of evidence supporting a long-term net benefit when considering biologics for patients with JIA

    

10. GERLONI V, PONTIKAKI I, GATTINARA Met FANTINI F: [Biological therapy with TNF-inhibitors in pediatric rheumatology. Review of the literature and personal experience], Reumatismo., Vol. 59(3), 244-261., 2007
    Organism:UOS di Reumatologia Infantile, Istituto Ortopedico Gaetano Pini, Milano, Italia gerloni@gpiniitFAU - Gerloni, V
    Abstract:The therapeutic approach to JIA is sometimes very troublesome and progression to erosive polyarthritis may occur in all JIA categories. Only Methotrexate has shown efficacy and safety in a large controlled trial. Nevertheless, in many cases, drug resistance or intolerance has led to try other therapeutic options, with still debatable results. Therefore, there has been space, in the last few years, for new therapies as the TNF-inhibitors. This therapeutic approach has shown a dramatic clinical benefit in active polyarticular refractory JIA: the rate and rapidity of response have exceeded those of all other studied DMARDs. Preliminary data show that they are efficacious also for other pediatric rheumatic disease (spondyloarthropathies, autoimmune uveitis, dermatomyositis, Kawasaki syndrome and some autoinflammatory diseases). TNF-inhibitors in JIA have demonstrated a favourable benefit-to-risk profile. However, as their use has increased worldwide, some unusual, usually not serious, adverse events have emerged. Severe infections, including TB, and deaths have been reported. Long-lasting active disease, systemic disease, concurrent and previous immunosuppressive therapies, all contribute to risk of infection and other serious AEs. Given the evidence that TNF has a primary role in the pathogenesis of JIA, particularly in joint destruction, neutralizing this cytokine early, within the window of opportunity, could halt or delay progression of joint damage and debilitating consequences of the disease. Thus, for JIA patients whose disease is not quickly controlled with MTX, TNF blockers may be considered as first-line treatment, although long-term safety data still need to be established

     

11. GOLMIA A, GRINBLAT B, FINGER E, KLIEMAN C, ASSIR Fet SCHEINBERG M: The development of erythema elevatum diutinum in a patient with juvenile idiopathic arthritis under treatment with abatacept, Clin.Rheumatol., Vol. 27(1), 105-106., 2008
    Organism:Hospital Israelita Albert Einstein and Research Institute, Sao Paulo, BrazilFAU - Golmia, Andrea
    Abstract:In this case report, we present the first report of erythema elevatum diutinum after treatment of juvenile idiopathic arthritis with abatacept. Although it could also be coincidental, in our case, the appearance of vasculitis was not blocked by the simultaneous administration of a stimulation inhibitor, and alerts to the fact that as effective as a abatacept may be to control of the inflammatory articular symptoms, this might not translate into control of the disease

     

12. HAZEN MM, WOODWARD AL, HOFMANN I, DEGAR BA, GROM A, FILIPOVICH AHet BINSTADT BA: Mutations of the hemophagocytic lymphohistiocytosis-associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis, Arthritis Rheum., Vol. 58(2), 567-570., 2008
    Organism:Children's Hospital Boston, Boston, MassachusettsFAU - Hazen, Melissa M
    Abstract:The clinical syndromes of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are both characterized by dysregulated inflammation with prolonged fever, hepatosplenomegaly, coagulopathy, hematologic cytopenias, and evidence of hemophagocytosis in the bone marrow or liver. While HLH is either inherited or acquired, children with severe rheumatic diseases, most notably systemic juvenile idiopathic arthritis, are at risk for MAS. The phenotypic similarity between HLH and MAS raises the possibility that they share common pathogenetic mechanisms. Familial forms of HLH have been attributed to mutations in the genes encoding perforin (PRF1) and Munc13-4 (UNC13D), among others, and are characterized by defective cytotoxic lymphocyte function. While some patients with systemic JIA have decreased levels of perforin protein expression and natural killer (NK) cell function, mutations of HLH-associated genes in patients with systemic JIA have not been reported. We report the case of an 8-year-old girl with systemic JIA without MAS who was found to have compound heterozygous mutations of UNC13D and reduced NK cell cytotoxic function. This case broadens the range of clinical phenotypes attributable to UNC13D mutations and offers new insights into the etiology and pathogenesis of systemic JIA

     

13. HE Y, LU A, ZHA Y, YAN X, SONG Y, ZENG S, LIU W, ZHU W, SU L, FENG X, QIAN Xet LU C: Correlations between symptoms as assessed in traditional chinese medicine (TCM) and ACR20 efficacy response: a comparison study in 396 patients with rheumatoid arthritis treated with TCM or Western medicine, J.Clin.Rheumatol., Vol. 13(6), 317-321., 2007
    Organism:Guangdong Traditional Chinese Medicine Hospital, Guangzhou, Guangdong Province, ChinaFAU - He, Yiting
    Abstract:OBJECTIVE: This research was designed to explore the role of joint and nonarticular clinical manifestations traditionally evaluated in Chinese herbal medicine in predicting efficacy of treatment for rheumatoid arthritis. METHODS: Three hundred ninety-six patients were randomly divided to receive Western medicine (WM) therapy, 197 cases; and traditional Chinese herbal medicine (TCM), 199 cases. A complete physical examination and 18 clinical manifestations typically assessed in TCM were recorded before the randomization. The WM therapy included diclofenac extended action tablets, methotrexate, and sulfasalazine. The TCM therapy included Glucosidorum Tripterygll Totorum tablets and Yishen Juanbi tablets. The American College of Rheumatology (ACR) response criteria were used for efficacy evaluation. All data were analyzed using the SPSS11.5 statistical package. RESULTS: ACR20 and 50 responses with WM treatment were higher at 24 weeks than in the TCM group. In the WM group, 89% achieved ACR20 whereas 65.8% on TCM reached this response In the WM group, efficacy was negatively related to subjective symptoms of dizziness, and positively related to joint tenderness and thirst as recorded at entry. In contrast, in the TCM group the efficacy was positively related to joint tenderness and joint pain, and negatively related to the joint stiffness and more nocturia. CONCLUSION: Symptoms including those not directly related to joints and those inquired about in TCM may have influence on the efficacy of therapy, and might merit further study to ascertain if they can be helpful to guide specific therapy

     

14. HILL AG, DARKO R, SEFFAH J, ADANU RM, ANARFI JKet DUDA RB: Health of urban Ghanaian women as identified by the Women's Health Study of Accra, Int.J.Gynaecol.Obstet., Vol. 99(2), 150-156., 2007
    Organism:Department of Population and International Health, Harvard School of Public Health, Boston, MA, USAFAU - Hill, A G
    Abstract:OBJECTIVE: The purpose of the Women's Health Study of Accra was to provide an assessment of the prevalence of communicable and non-communicable illnesses. METHOD: This was a prospective, community-based study that included an interview for medical illnesses, a comprehensive physical examination, and laboratory testing. A total of 1328 women were examined at Korle Bu Teaching Hospital, University of Ghana. RESULTS: Prevalent conditions included poor vision (66.8%), malaria (48.7%), pain (42.8%), poor dentition (41.6%), hypertension (40.2%), obesity (34.7%), arthritis (27.1%), chronic back pain (19.4%), abnormal rectal (16.0%) and pelvic examinations (12.7%), HIV in women age 24-29 (8.3%), and hypercholesterolemia (22.7%). Increasing age, lack of formal education, and low-income adversely affected health conditions. CONCLUSION: The high prevalence of preventable illnesses in this expanding urban population indicates that the health care services are obligated to develop and provide screening, preventive strategies and treatment for both general health and gynecologic health conditions

     

15. HOCHBERG MC, JOHNSTON SSet JOHN AK: The incidence and prevalence of extra-articular and systemic manifestations in a cohort of newly-diagnosed patients with rheumatoid arthritis between 1999 and 2006, Curr.Med.Res.Opin., Vol. 24(2), 469-480., 2008
    Organism:Division of Rheumatology and Clinical Immunology, University of Maryland School of Medicine, 10 South Pine Street, MSTF 8-34 Baltimore, MD 21201, USA mhochber@medicineumarylandeduFAU - Hochberg, Marc C
    Abstract:OBJECTIVE: To evaluate the incidence and prevalence of extra-articular (ExRA) and systemic (SysM) manifestations in a cohort of newly-diagnosed patients with rheumatoid arthritis (RA) in the United States. PATIENTS AND METHODS: Retrospective analysis using inpatient, outpatient, and pharmacy claims data contained in the Thomson Healthcare MarketScan research databases. Patients >or= 18 years of age with a diagnosis of RA (ICD-9-CM 714.0x) on three non-diagnostic claims on different days between January 1, 1999 and September 30, 2006, and at least 12 months of continuous enrollment prior to, and at least 2 years following diagnosis were included in the analysis. Thirty ExRA/SysM, classified into six groups (cardiovascular, blood, mucosa, pulmonary, other, and non-specific), were evaluated. Patients were followed until in-hospital death, disenrollment, or study end. RESULTS: A total of 16,752 patients were included (mean age 59.8 +/- 13.5 years; 72.0% female), and were followed up for a mean of 3.9 +/- 1.4 years. ExRA/SysM were experienced by 47.5% of patients, with cardiovascular (27.2%) the most common. The most frequent individual ExRA/SysM was 'other CVD' (17.2%). Female sex was associated with a reduced risk of cardiovascular ExRA/SysM (HR, 0.66; 95% CI, 0.61-0.72), and an increase in mucosa ExRA/SysM (HR, 2.55; 95% CI, 2.03-3.19). Prior treatment with methotrexate (MTX) was associated with significantly reduced risks of cardiovascular (HR 0.65; 95% CI, 0.59-0.72) and blood system (HR 0.71; 95% CI, 0.61-0.82) ExRA/SysM. Other significant associations were also evident: age, comorbidity as measured by CCI and CDS, and geographic region were associated with increased risks for some ExRA/SysM, while prior NSAID treatment and the presence of diabetes were associated with a lower risk for some ExRA/SysM. CONCLUSION: ExRA/SysM develop in approximately 47% of patients with RA within a few years of diagnosis. Prior treatment with some therapies used in RA management were associated with a reduced risk of developing some ExRA/SysM, while several demographic factors and the presence of comorbidities also affected the risk of developing ExRA/SysM. This analysis was restricted to patients with employer- or government-funded health insurance, while several potential predictors of ExRA/SysM could not be controlled for in the multivariate analysis, as they could not be measured using claims data. Hence, these results may not be generalizable to other groups of patients with RA

     

16. HUANG BE, AMOS CIet LIN DY: Detecting haplotype effects in genomewide association studies, Genet.Epidemiol., Vol. 31(8), 803-812., 2007
    Organism:Department of Biostatistics, University of North Carolina, North Carolina 27599-7420, USAFAU - Huang, B E
    Abstract:The analysis of genomewide association studies requires methods that are both computationally feasible and statistically powerful. Given the large-scale collection of single nucleotide polymorphisms (SNPs), it is desirable to explore the information contained in their interrelationships. In particular, utilizing haplotypes rather than individual SNPs and accounting for correlations of polymorphisms in adjustment for multiple testing can lead to increased power. We present a statistically powerful and numerically efficient method based on sliding windows of adjacent SNPs to detect haplotype-disease association in genomewide studies. This method consists of an efficient algorithm to calculate a proper likelihood-ratio statistic for any given window of SNPs, along with an accurate and efficient Monte Carlo procedure to adjust for multiple testing. Simulation studies using the HapMap data showed that the proposed method performs well in realistic situations. We applied the new method to a case-control study on rheumatoid arthritis and identified several loci worthy of further investigations

     

17. JAVIER RMet HACHULLA E: [Osteoarticular manifestations of Gaucher disease in adults: pathophysiology and treatment], Presse Med., Vol. 36(12 Pt 3), 1971-1984., 2007
    Organism:Service de Rhumatologie, Hopital de Hautepierre, Avenue Moliere, Strasbourg, France Rose-marieJavier@chru-strasbourgfrFAU - Javier, Rose-Marie
    Abstract:Gaucher disease frequently has severe osteoarticular manifestations that may be disabling. Ischemic phenomena cause the most serious complications and lead to irreversible lesions. Aseptic osteonecrosis of the hip is the most disabling complication; it causes intense early bone pain and often joint collapse and secondary osteoarthritis in young adults. Localized or systemic bone fragility explains osteopenia, osteoporosis, and fractures (vertebral collapse with irreversible kyphosis causing chronic morbidity). Although no double-blind randomized studies have assessed the bone effects of enzyme replacement therapy, it has been shown effective in reducing bone pain in about half of all treatment-naive patients within 1 to 2 years and in improving bone mineral density after 3 years. In open-label trials, substrate reduction therapy (miglustat) reduced both bone pain and bone marrow infiltration. Specific treatment for bone fragility, with bisphosphonates for example, should be considered after rigorous individualized evaluation and assessment of other risk factors

     

18. JOHNSON S, HULTON SA, BRUNDLER MA, MOSS C, HUISSOON Aet TAYLOR CM: End-stage renal failure in adolescence with Sjogren's syndrome autoantibodies SSA and SSB, Pediatr.Nephrol., Vol. 22(10), 1793-1797., 2007
    Organism:Department of Nephrology, Birmingham Children's Hospital, Birmingham, B4 6NH, UK sajohnson2@bhamacukFAU - Johnson, Sally
    Abstract:We describe two adolescents who presented with end-stage renal failure and clinical features suggestive of Sjogren's syndrome (SS). They both demonstrated severe, chronic, tubulointerstitial inflammation on renal biopsy, high-titre antinuclear antibodies, high immunoglobulin A and G concentrations, positive anti-SSA and anti-SSB antibodies, and negative anti-double-stranded DNA antibodies. One had subjective and objective evidence of the sicca complex (dry eyes and/or dry mouth) and fulfilled the commonly accepted SS consensus criteria. The other showed no evidence of the sicca complex but fulfilled modified criteria for juvenile SS. SS may be underrecognised as a cause of end-stage renal failure in childhood

     

19. KHANNA Ret SMITH MJ: Utilization and costs of medical services and prescription medications for rheumatoid arthritis among recipients covered by a state Medicaid program: a retrospective, cross-sectional, descriptive, database analysis, Clin.Ther., Vol. 29(11), 2456-2467., 2007
    Organism:Department of Pharmaceutical Systems and Policy, West Virginia University School of Pharmacy, Morgantown, WV 26506, USA rkhanna@hscwvueduFAU - Khanna, Rahul
    Abstract:BACKGROUND: Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by inflammation of synovial tissues that leads to joint swelling, stiffness and pain, and progressive joint destruction. There is currently limited information about demographic differences in the prevalence of RA and the utilization and costs of RA-related prescription medications and medical services among low-income populations. OBJECTIVES: This study assessed the prevalence of RA and the utilization and costs of RA-related medical services and prescription medications among recipients enrolled in a state Medicaid program. METHODS: A retrospective, cross-sectional, descriptive analysis of West Virginia (WV) Medicaid fee-for-service administrative claims data was conducted. Medical services claims for recipients aged between 15 and 64 years with a primary diagnosis code for RA during the calendar year 2003 were extracted. Unique recipient numbers obtained from these claims were used to extract the corresponding prescription claims. Prevalence and health care use rates were calculated by demographic categories. Costs were reported from the perspective of WV Medicaid. RESULTS: A total of 143,211 recipients aged between 15 and 64 years received WV Medicaid benefits. Among these, 1157 recipients (0.81%) had > or = 1 medical service claim (hospitalization, emergency department visit, or office visit) with a primary diagnosis of RA. The mean (SD) age of the sample was 47 (11.1) years. The highest rates of RA by age, sex, and race occurred among recipients aged 45 to 64 years (16.4:1000), females (10.1:1000), and whites (8.2:1000). Office visits accounted for the majority of medical services visits and costs. Among the sample, 67.8% had > or =1 prescription claim for a narcotic analgesic, 58.8% for an NSAID, 48.3% for an oral steroid, 40.1% for a disease-modifying antirheumatic drug, and 12.4% for a biologic agent. Medicaid paid a mean of $2379 per recipient for RA-related health care services. Prescription claims accounted for 74.6% of the total cost of RA care. Biologic agents accounted for the largest proportion (54.1% ) of prescription costs. CONCLUSIONS: The prevalence of RA and rates of health care services utilization for RA among recipients of WV Medicaid differed with regard to demographic characteristics. Utilization of RA-related prescription medication among recipients varied by pharmacotherapy class. This study presents baseline information that might be used as a model for future surveillance RA studies using payer administrative datasets

     

20. KOSINSKI M, JANAGAP CC, GAJRIA Ket SCHEIN J: Psychometric testing and validation of the Chronic Pain Sleep Inventory, Clin.Ther., Vol. 29 Suppl, 2562-2577., 2007
    Organism:Quality Metric, Inc, Lincoln, Rhode Island 02865, USA mkosinski@qualitymetriccomFAU - Kosinski, Mark
    Abstract:BACKGROUND: Patients with chronic pain often experience significant disruptions in sleep. A potential benefit of treatment aimed at pain relief is improved quality of sleep in patients with chronic pain. OBJECTIVE: The goal of this study was to evaluate the psychometric properties of the Chronic Pain Sleep Inventory (CPSI) and provide preliminary evidence of its construct validity in assessing sleep problems among patients with chronic pain. METHODS: Data came from four 12-week, multi-center, double-blind, randomized, placebo-controlled clinical trials of chronic low back pain and osteo-arthritis of the hip or knee. CPSI data were collected at baseline and 12 weeks. The 5 CPSI items measured trouble falling asleep (CPSI1), needing sleep medication (CPSI2), awakened by pain during the night (CPSI3) and in the morning (CPSI4), and overall quality of sleep (CPSI5) on a 100-mm visual analog scale. Exploratory and confirmatory factor analyses were conducted to evaluate the underlying dimensionality and structure of the CPSI scales. The known-groups method was used to assess validity by comparing CPSI item scores across groups of patients known to differ in the presence and severity of sleep problems as measured by the Physical Dependence Questionnaire. RESULTS: A total of 2674 patients were included in the study (mean age, 57.6 years; 61.0% female; 80.2% white). The majority of the patients were treated for chronic pain related to osteoarthritis of the hip or knee (n=2294). Findings revealed a single sleep problems index can be scored from 3 of the 5 CPSI items (CPSI1, CPSI3, and CPSI4). All 3 items attributed sleep problems to pain, with high factor loadings (>0.80) and a high internal consistency (>0.90). Moderate to high correlations (r >or= 0.50) between CPSI items demonstrated convergent validity, and weak correlations (r<0.50) with other health-related scales (the Western Ontario and McMaster Universities Osteo-arthritis Index and the 36-item Short-Form Health Survey) demonstrated discriminant validity. All CPSI items showed greater ability to discriminate and respond to changes in the presence and severity of sleep problems than the other health-related scales. CONCLUSIONS: CPSI items showed good construct validity, and the results support the scoring of a reliable single index from 3 of the 5 CPSI items that all attributed sleep problems to pain

     

21. LEEB BF, HAINDL PM, MAKTARI A, NOTHNAGL Tet RINTELEN B: Disease activity score-28 values differ considerably depending on patient's pain perception and sex, J.Rheumatol., Vol. 34(12), 2382-2387., 2007
    Organism:2nd Department of Medicine, Center for Rheumatology, State Hospital Korneuburg-Stockerau, Karl Landsteiner Institute for Clinical Rheumatology; A-2000 Stockerau, Landstrasse 18, Austria leebhumanis@kav-kostatFAU - Leeb, Burkhard F
    Abstract:OBJECTIVE: To determine if the Disease Activity Index including a 28-joint count (DAS28) is equally applicable for the total population with rheumatoid arthritis (RA). METHODS: Five hundred fifty-seven outpatients with RA [432 women, 125 men; median age 64 yrs (range 0-85), median disease duration 48 mo (range 2-548)] were enrolled consecutively into this cross-sectional study. DAS28, physician's global assessment of disease activity, patient's assessment of pain on visual analog scale, C-reactive protein (mg/dl), rheumatoid factor (RF), and disease duration were recorded. t-tests were applied for all comparisons of DAS28 values. Linear regression analysis was performed for each confounding factor. RESULTS: The mean DAS28 in female patients was 3.66 +/- 0.57 SEM, and in males 3.01 +/- 1.12 (p < 0.001). DAS values in patients with early RA (< 37 mo) were significantly higher than in patients with advanced RA (3.62 +/- 0.67 vs 3.37 +/- 0.81, respectively; p < 0.017). Regression analysis revealed a highly significant relationship between DAS28 score and patient's pain rating (r = 0.592, p < 0.0001). Pain exerted the greatest influence on the DAS28 (p < 0.0001), while of the other factors only age (p < 0.008 for females, p < 0.007 for males) was also significantly correlated with the DAS28 values. CONCLUSION: DAS28 values differ considerably depending primarily on the patient's pain perception and gender and to a lesser degree on patient's age, whereas results for disease duration and RF were inconclusive

     

22. MALEMBA JJet MBUYI-MUAMBA JM: Clinical and epidemiological features of rheumatic diseases in patients attending the university hospital in Kinshasa, Clin.Rheumatol., Vol. 27(1), 47-54., 2008
    Organism:Department of Internal Medicine, Kinshasa University Hospital, Post Box 123, Kinshasa XI, Democratic Republic of CongoFAU - Malemba, J J
    Abstract:The aim of the present retrospective and hospital-based study was to describe epidemiological and clinical features of rheumatic diseases in patients attending the University Hospital of Kinshasa (UHK). Rheumatic complaint was a reason for consultation in 12.1% of outpatients attending the Department of Internal Medicine of the UHK. Osteoarthritis was the most common rheumatic disease (59.2%), followed by soft tissue rheumatism (16.1%), gout (9.3%), and spondylarthropathies (7.5%). The cumulative frequency of autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, dermatomyositis, and mixed connective tissues disease) and the frequency of osteoporosis were 5.2 and 2.7%, respectively. Lumbar spine was the part of the skeleton mostly affected by osteoarthritis. Pathological fractures in osteoporosis, subcutaneous nodules, rheumatoid factor, and erosive bone lesions in rheumatoid arthritis were rarely found. Compared to the previous studies performed in the same hospital, our results disclose a threefold increase of rheumatic outpatients. The paucity of erosive arthritis and extra-articular manifestations suggest the less severity of rheumatoid arthritis in our patients. Likewise, the absence of femoral and wrist osteoporotic fractures and the scarcity of advanced vertebral crush fractures suggest the mildness of osteoporosis

     

23. MASIERO S, BONIOLO A, WASSERMANN L, MACHIEDO H, VOLANTE Det PUNZI L: Effects of an educational-behavioral joint protection program on people with moderate to severe rheumatoid arthritis: a randomized controlled trial, Clin.Rheumatol., Vol. 26(12), 2043-2050., 2007
    Organism:Department of Rehabilitation Medicine, School of Medicine, University of Padova, Padova, Italy stefmasiero@unipditFAU - Masiero, Stefano
    Abstract:The aim of this study was to asses the effects on pain, disability, and health status of an educational-behavioral joint protection program in a group of moderate-severe rheumatoid arthritis (RA) patients. Eighty-five subjects with RA in treatment with anti-tumor necrosis factor alpha (TNFalpha) drugs (infliximab) were enrolled into the study and randomized into either an experimental group (46, EG) or a control group (39, CG). We organized four EG meetings, which included information on pathophysiology and evolution of RA, joint protection during normal activities of daily living, suggestions on how to adapt the surrounding environment, and self-learning exercises to perform at home. Sociodemographic characteristics and degree of knowledge of the disease, measured by the Health Service Interview (HSI), were recorded at baseline. The outcome measures included the Visual Analogue Scale (VAS), the Arthritis Impact Measurement Scale 2 (AIMS2), and the Health Assessment Questionnaire (HAQ), which were administered at the beginning and end of the trial. Thirty-six patients from the EG (7 men and 29 women; mean age 54.2 years) and 34 from the CG (6 men and 28 women; mean age 52.2 years) completed the trial. No statistical differences in baseline evaluations were found between the two groups. According to the answers given on the HSI, the majority of our patients had poor knowledge of RA and its consequences. After a mean time of 8 months, the patients receiving educational training displayed a significant decrease, compared to the CG, in the VAS (p = 0.001), HAQ (p = 0.000), and physical (p =0.000), symptoms (p = 0.049), and social interaction (p = 0.045) scores on the AIMS2, but not in other items. Our study showed that 8 months after attending an educational-behavioral joint protection program, subjects with moderate-severe RA presented less pain and disability and thus an enhanced health status. This approach may efficiently complement drug therapy in these patients

     

24. MINDEN Ket NIEWERTH M: [Juvenile idiopathic arthritis - clinical subgroups and classification.], Z.Rheumatol., Vol. ., 2008
    Organism:Deutsches Rheumaforschungszentrum Berlin und Universitatskinderklinik, Charite Universitatsmedizin, Campus Virchow-Klinikum, Chariteplatz 1, 10117, Berlin, Deutschland, minden@drfzde
    Abstract:There have been major advances in the field of paediatric rheumatology over the last 15 years, which have included improvements in the classification of chronic arthritis, the most common chronic rheumatic disease in children and adolescents. A new classification was proposed by the International League of Associations for Rheumatology (ILAR) in 1995, which facilitated collaborative research and comparability of study results. According to the ILAR classification juvenile idiopathic arthritis (JIA) is a new term that encompasses the heterogeneous forms of arthritis of unknown cause, which begin before 16 years of age. The JIA classification identifies seven disease categories, which differ in their clinical presentation, outcome, and in some cases, genetic background, and which are characterized here on the basis of data from the national paediatric rheumatology database. Despite its usefulness for international research, the ILAR classification also has restrictions and further revision is therefore required. A special problem with a need for a solution is represented by the limited application in adult rheumatology

     

25. MOLLER B, KERSCHBAUMER G, KOMOR M, KERSCHBAUMER F, OTTMANN OG, HOELZER Det HOFMANN WK: Genomic imprinting of insulin-like growth factor 2 (IGF-2) in chronic synovitis, Growth Horm.IGF.Res., Vol. 17(6), 500-505., 2007
    Organism:Inselspital - University Hospital Bern, Klinik fur Rheumatologie, klinische Immunologie und Allergologie, CH-3010 Bern, Switzerland burkhardmoeller@inselchFAU - Moller, B
    Abstract:OBJECTIVE: To search for relaxation or loss of IGF-2 imprinting (LOI) in rheumatoid arthritis (RA) synovial tissues. DESIGN: The genotype of IGF-2 was determined in 25 freshly isolated synovial tissue samples with signs of active inflammation by polymerase chain reaction (PCR) and restriction fragment length polymorphism. Imprinting was determined in synovial tissue mononuclear cells (STMC) of five informative heterozygous patients by reverse transcriptase (RT)-PCR. Mitogen-stimulated peripheral blood mononuclear cells (PBMC) from six informative healthy donors were selected for control. RESULTS: In vitro proliferation of CD4+ and CD8+ PB T cells, and also of CD19+ PB B cells was detectable upon mitogen stimulation. Furthermore, MHC II molecule expression on synovial B and T cells indicated in vivo cell activation. Monoallelic IGF-2 expression was seen in PBMC cultures from two healthy donors under both, resting and stimulating conditions. In two other PBMC cultures, LOI occurred exclusively after 24 h of stimulation. PBMC from two other healthy donors showed LOI under both, resting and stimulating conditions. Mitogen induced and spontaneous LOI was reversible in each one PBMC culture after 72 h. In contrast, none of the informative STMC cultures showed LOI. CONCLUSIONS: LOI in lymphocytes may occur spontaneously or inducible. However, longstanding activation of lymphocytes in RA synovitis appears not to be related to this mechanism

     

26. MOMOHARA S, INOUE E, IKARI K, KAWAMURA K, TSUKAHARA S, MOCHIZUKI T, TOKI H, MIYAWAKI M, SAITO S, HARA M, KAMATANI N, YAMANAKA Het TOMATSU T: Risk factors for total knee arthroplasty in rheumatoid arthritis, Mod.Rheumatol., Vol. 17(6), 476-480., 2007
    Organism:Department of Orthopaedic Surgery, Institute of Rheumatology, Tokyo Women's Medical University, 10-22 Kawada, Shinjuku-ku, Tokyo, 162-0054, Japan smomohara@iortwmuacjpFAU - Momohara, Shigeki
    Abstract:We conducted a study to assess the predictive factors for total knee arthroplasty (TKA) in a cohort of rheumatoid arthritis (RA) patients recruited and followed prospectively for 5 years. A linked registry study using information from a large observational cohort of RA patients followed at the Institute of Rheumatology, Tokyo Women's Medical University (IORRA) was done. Baseline routine clinical and laboratory assessments were recorded. The data were analyzed using the multivariate piecewise-linear Cox (PL-Cox) regression model; the model initially included variables such as gender, age, duration of the disease, visual analog scale (VAS) generated by physicians (VAS-physician), patient-reported VAS for pain (VAS-pain), VAS for general health (VAS-GH), disability level using the Japanese version of the Health Assessment Questionnaire (J-HAQ), C-reactive protein, erythrocyte sedimentation rate, rheumatoid factor (RF), and hemoglobin. Of the 3945 patients registered at baseline, 955 (24.2%) had pain or tenderness in their knee joints, and 114 (11.9%) had TKA surgery in one or both knee joints. On PL-Cox regression, the variables with positive coefficients were J-HAQ, VAS-pain, VAS-physician, and RF positive; advanced age was associated with a reduced risk of TKA. The hazard ratios were: 0.920 for age >60 years; 2.64 for J-HAQ <1.5; 1.01 for J-HAQ >1.5; 1.47 for VAS-pain >6 (cm); 1.20 for VAS-physician >4 (cm); and 2.08 for RF positive. The consistently predictive factors for TKA in RA were age, J-HAQ, VAS-pain, VAS-physician, and RF positive. Age greater than 60 years was associated with a decreased risk of TKA, while J-HAQ from 0 to 1.5, VAS-pain >6 (cm), and VAS-physician >4 (cm) were associated with an increased risk for TKA surgery. These results suggest that, when treating RA patients, physicians should pay particular attention to pain complaints, the patient's daily activity level, and the RF factor status

     

27. MOORTHY LN, PETERSON MG, ONEL KBet LEHMAN TJ: Do children with lupus have fewer male siblings?, Lupus., Vol. 17(2), 128-131., 2008
    Organism:Department of Pediatrics, Division of Pediatric Rheumatology, Robert Wood Johnson Medical School-University of Medicine and Dentistry of New Jersey, New Brunswick, NJ, USA LNMoorthy@maccomFAU - Moorthy, L N
    Abstract:It is widely acknowledged that genetic factors play a significant role in the pathogenesis of systemic lupus erythematosus (SLE). However, the female preponderance remains unexplained. We hypothesized that the female preponderance in childhood SLE results from selection early in the course of conception against male fetuses bearing genetic material predisposing to SLE. If this hypothesis is accurate, there should be a decreased number of male children in families with a child with SLE. Alternatively, children with SLE would have fewer male siblings. Further, this hypothesis may apply to other diseases with a female predominance such as pauciarticular onset juvenile rheumatoid arthritis (PaJRA), and not apply to diseases without female preponderance such as systemic onset juvenile rheumatoid arthritis (SoJRA). Chart review of patients with childhood onset SLE and PaJRA revealed a greater number of female children in these families compared with families of patients with SoJRA. Large-scale epidemiologic studies with precise counting of miscarriages and abortions could help to confirm these findings. Detailed studies of genetic and maternal intrauterine factors are required to conclusively prove this hypothesis

     

28. NEROME Y, IMANAKA H, NONAKA Y, TSURU Y, MAENO N, TAKEZAKI T, MORI H, AKAIKE H, KUBOTA T, KAWANO Yet TAKEI S: A case of planned pregnancy with an interruption in infliximab administration in a 27-year-old female patient with rheumatoid-factor-positive polyarthritis juvenile idiopathic arthritis which improved after restarting infliximab and methotrexate, Mod.Rheumatol., Vol. ., 2008
    Organism:Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan, nerome@mkufmkagoshima-uacjp
    Abstract:We report a 27-year-old case of juvenile idiopathic arthritis (JIA) having been stopped infliximab during pregnancy. She was safely treated by infliximab therapy with premedications for preventing infusin reactions after her delivery, and then improved in the same manner as when she had been treated with infliximab therapy before pregnancy. As a result, it remains unclear whether or not we can use infliximab to control disease activities during pregnancy. In addition, it is also important to clarify whether or not premedications should be used when resuming infliximab treatment in such patients after pregnancy. These problems still remain controversial. More definitive data are needed in order to allow rheumatologists to better select the optimal TNF-alpha inhibitor therapy when treating pregnant JIA patients

     

29. NIEHUES T, FEYEN Oet TELIEPS T: [Concepts on pathogenesis of juvenile idiopathic arthritis.], Z.Rheumatol., Vol. ., 2008
    Organism:Zentrum fur Kinder- und Jugendmedizin, Helios Klinikum Krefeld, Akademisches Lehrkrankenhaus der Heinrich-Heine-Universitat Dusseldorf, Krefeld, Lutherplatz 40, 47805, Krefeld, Deutschland, timniehues@klinikum-krefeldde
    Abstract:There are various explanations for the development of juvenile idiopathic arthritis (JIA).There are indications that gene changes in the immune system can predispose to JIA and that regulation of the immune system is crucial in pathogenesis. The adaptive, acquired immune system probably plays a central role. Thus, in the case of JIA a conspicuous population of highly activated T-cells can be found in the synovia. B-cells are also involved, as indicated by the positive ANA titers in JIA patients. Regulatory T-cells (Tregs) attempt to prevent the expansion of autoreactive T-cells, as well as having a protective function.However, the natural or congenital immune system also plays a role. Thus a disorder of the inflammasome could underlie the cause of JIA with systemic onset. The interaction between congenital and adaptive immune systems shows that a distinct spatial and temporal separation between the two immune systems is becoming increasingly difficult. An infection- and virus-related immune reaction could also be the cause of JIA. Proinflammatory cytokines are of proven significance in pathogenesis in terms of how they are released under stress, for example.New genomic and proteomic techniques are able to produce individualized profiles for each patient and enable an increasingly fine separation between subtypes, thus improving therapeutic possibilities

     

30. OELZNER P, FRANKE S, LEHMANN G, EIDNER T, MULLER A, WOLF Get HEIN G: Soluble receptor activator of NFkappa B-ligand and osteoprotegerin in rheumatoid arthritis - relationship with bone mineral density, disease activity and bone turnover, Clin.Rheumatol., Vol. 26(12), 2127-2135., 2007
    Organism:Division of Rheumatology and Osteology, Department of Internal Medicine III, Friedrich Schiller University, Jena, Germany peteroelzner@meduni-jenadeFAU - Oelzner, P
    Abstract:The aim of our study was to investigate determinants of bone mineral density (BMD) measured by dual X-ray absorptiometry at the lumbar spine (BMD-LS) and at the femoral neck (BMD-FN) in patients with rheumatoid arthritis (RA) with special respect to bone resorbing proinflammatory cytokines and their physiological antagonists. In 142 RA patients the following parameters were measured in parallel with BMD: serum levels of soluble receptor activator of nuclear factor kappa-B-ligand (sRANKL), osteoprotegerin (OPG), interleukin (IL)-6, soluble glycoprotein 130 (sgp130), 25-hydroxyvitamin D3 (25OHD(3)), 1,25-dihydroxyvitamin D3 (1,25[OH](2)D(3)), intact parathyroid hormone, osteocalcin, ionized calcium, renal excretion of pyridinolin and deoxypyridinolin, C-reactive protein, and erythrocyte sedimentation rate (ESR). No significant differences of sRANKL, OPG, IL-6, and spg130 were found between patients with osteoporosis (47.9% of patients), osteopenia (36.6%), and normal BMD (15.5%). However, total sRANKL was significantly higher in postmenopausal women with osteoporosis at FN than in those without (p < 0.05) and showed a negative correlation with BMD-LS in patients older than 60 years (p = 0.01). BMD-LS and BMD-FN (p < 0.001) and total sRANKL (p < 0.01) were negatively related with the age of the patients. Only IL-6 (positive correlation, p < 0.001) and 1,25(OH)(2)D(3) (negative correlation, p < 0.001) but not sRANKL, OPG, and sgp130 were related to disease activity. Using multiple linear regression analysis, menopause was identified as the crucial negative determinant of BMD-LS (R (2) = 0.94, p = 0.001), whereas cumulative glucocorticoid dose (beta = -0.80, p = 0.001) and ESR (beta = -0.44, p = 0.016) were the negative determinants of BMD-FN (R (2) = 0.86, p = 0.001). The results indicate that influences of age and gender must be considered in investigations on the relationship between BMD and sRANKL in RA and that high serum levels of sRANKL seems to be associated with osteoporosis only in subgroups of RA patients

     

31. PETERSEN C, NORDMEYER S, MULLER-GODEFFROY E, FOELDVARI I, KUSTER RMet BULLINGER M: [Health-Related Quality of Life in Children and Adolescents with Juvenile Idiopathic Arthritis: Which Role do Age, Sex and Medical Parameters Play?], Klin.Padiatr., Vol. ., 2008
    Organism:1Institut und Poliklinik fur Medizinische Psychologie, Universitatsklinikum, Hamburg-Eppendorf, Hamburg
    Abstract:BACKGROUND: The assessment of health-related quality of life gains increasing importance in pediatric practice. In the field of rheumatic health conditions in young people only a few studies about this topic are available. Within a European study health-related quality of life of children and adolescents with idiopathic arthritis was assessed. The results of the German sample will be presented. Patients: A total of N=88 children and adolescents with juvenile idiopathic arthritis (57% female) between 8 und 16 years were included in the study. Method: Children and adolescents filled in a questionnaire. Health-related quality of life was assessed with the DISABKIDS instrument. Results: No age or gender effects on health-related quality of life were detected. The variable "limitation of mobility" as well as the item "pain" showed the strongest relationship with the health-related quality of life dimensions. Conclusions: Health-related quality of life is decisively influenced by the consequences of the health condition. For clinical practice the study showed that patients with mobility limitations and/or pain need heightened attention which should especially concentrate on the social and mental dimensions of health-related quality of life

     

32. PRELOG M, SCHWARZENBRUNNER N, SAILER-HOECK M, KERN H, KOPPELSTAETTER C, WURZNER R, ZIMMERHACKL LBet BRUNNER J: Indications for a Disturbed Peripheral T-Cell Homeostasis in Juvenile Idiopathic Arthritis (JIA): Absent Expansion of CD28- T-Cells and No Decrease of Naive T-Cells in Cytomegalovirus-positive Patients with JIA, J.Rheumatol., Vol. ., 2008
    Organism:From the Departments of Pediatrics, Internal Medicine, and Hygiene, Microbiology and Social Medicine, Medical University Innsbruck, Innsbruck, Austria
    Abstract:OBJECTIVE: To investigate the influence of latent cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infections on CD28-expressing T-cell subpopulations and replicative senescence of naive T-cells as a marker for aging of the immune system in children with juvenile ideopathic arthritis (JIA). METHODS: T-cell subpopulations were analyzed from 24 patients with JIA and 61 healthy age-matched controls by fluorescence activated cell sorting. Relative telomere length (RTL) in CD4+CD28+CD45RA+ (naive) T-cells was measured by quantitative polymerase chain reaction. RESULTS: Although confirming known data of expansions of CD28- T-cells and tendency of decreasing naive T-cells in CMV-seropositive healthy individuals, our findings did not show a marked influence of latent EBV or CMV infection on CD28-expressing T-cells in patients with JIA. In contrast, CMV was an independent factor for loss of CD28, regardless of age, in healthy controls. Irrespective of serology results for CMV or EBV, patients with JIA showed signficantly decreased RTL compared to age-matched controls. Regression lines for RTL and age revealed decreased RTL with advancing age in CMV-positive and EBV-positive subjects. The evidence that findings for CMV-positive JIA patients did not resemble the findings of healthy CMV-positive controls, namely expansion of CD28- T-cells and decrease of naive T-cells, may support the theory of a disturbed peripheral T-cell homeostasis in JIA. CONCLUSION: Diminished mechanisms of T-cell homeostasis and premature aging of the immune system may play a role in the pathogenesis of JIA

     

33. ROSTOM S, AMINE B, BENSABBAH R, ABOUQAL Ret HAJJAJ-HASSOUNI N: Hip involvement in juvenile idiopathic arthritis, Clin.Rheumatol., Vol. ., 2008
    Organism:Department of Rheumatology (Pr N Hajjaj-Hassouni), El Ayachi Hospital, University Hospital of Rabat-Sale, Sale, 11000, Morocco, rostomsamira2003@yahoofr
    Abstract:We analyzed the clinical, biological, and radiological aspects of hip involvement in juvenile idiopathic arthritis (JIA) in a developing country.The recruited patients fulfilled the International League Against Rheumatism criteria for the diagnosis of the JIA. Clinical, biological, and radiological parameters relating to the JIA were collected. Hip involvement was assessed according to clinical and radiological data related to hip disease. One hundred twenty-one patients were included (68 girls and 53 boys). The mean age of the disease onset was 9 +/- 4.2 years (1-16 years).The mean age of the patients at the time of the study was 15 +/- 10 years (2-46 years). The duration of the disease was 5 +/- 8.5 years (0.5-39 years). Forty cases (33%) of the hip involvement were noted. The mean age was 24 +/- 10.03 years (3-46 years); the sex ratio was 1:3. The mean duration of the hip disease was 0.6 +/- 3.6 years (3-14 years). Hip arthritis seemed to be more frequent in polyarticular and enthesitis-related arthritis. The severity of the hip involvement was significantly correlated with early disease onset, disease duration, subtypes, and high disability (for all these data p < 0.05). This study suggested that in JIA hip involvement was more frequent in enthesitis-related arthritis and polyarticular subtypes. It was correlated with the severity and the early disease onset of the JIA, which was similar to reported data

     

34. SACKS JJ, HELMICK CG, LUO YH, ILOWITE NTet BOWYER S: Prevalence of and annual ambulatory health care visits for pediatric arthritis and other rheumatologic conditions in the United States in 2001-2004, Arthritis Rheum., Vol. 57(8), 1439-1445., 2007
    Organism:Centers for Disease Control and Prevention, Atlanta, Georgia 30341-3724, USA jjs3@cdcgovFAU - Sacks, Jeffrey J
    Abstract:OBJECTIVE: To estimate the prevalence of and the annual number of ambulatory health care visits for pediatric arthritis and other rheumatologic conditions. METHODS: We used physician office visit, outpatient department visit, and emergency department visit data from the 2001-2004 National Ambulatory Medical Care Survey and 2001-2004 National Hospital Ambulatory Medical Care Survey to estimate annual visits for the International Classification of Diseases, Ninth Revision, Clinical Modification codes thought to represent significant pediatric arthritis and other rheumatologic conditions (SPARC). We converted visit estimates into prevalence estimates using data on the number of prior annual visits per patient. Synthetic estimates for states were produced using national rates. RESULTS: The average annualized estimate of the number of children with SPARC was 294,000 (95% confidence interval [95% CI] 188,000-400,000). The annualized number of ambulatory health care visits for SPARC was 827,000 (95% CI 609,000-1,044,000). CONCLUSION: Pediatric arthritis estimates have varied widely because it is an umbrella term for which there are many definitions and because it is a relatively uncommon condition from a population surveillance perspective. Our estimates suggest that arthritis-related health care visits impose a substantial burden on the pediatric health care system. One advantage of this surveillance paradigm is that it has established a starting point for tracking the national prevalence of arthritis and rheumatologic conditions in children on an ongoing basis using existing infrastructure rather than expensive new surveys. This surveillance system will help us monitor and predict the health care needs of patients with these conditions

     

35. SAITO Y, TORIUMI S, OTAKE Ret SUZUKI M: [Adult-onset Still's disease following a severe sore throat and fever. Case report], Nippon Jibiinkoka Gakkai Kaiho., Vol. 111(1), 21-24., 2008
    Organism:Department of Otolaryngology, Takeda General Hospital, FukushimaFAU - Saito, Yuki
    Abstract:Adult-onset Still's disease (AOSD) is characterized by fever, rash, and joint pain and may lead to chronic arthritis. The cause of AOSD is unknown, and it is rare. In children, Still's Disease is called systemic juvenile rheumatoid arthritis. We encountered a patient with adult-onset Still's disease following a severe sore throat and fever. The patient was a 17-year-old woman who consulted our hospital because of a sore throat and fever. She was admitted and treated with antibiotics, but the fever persisted. Laboratory parameters of inflammatory activity increased at an accelerated rate, and after ruling out sepsis, EBV-associated disease, and malignant lymphoma, a diagnosis of AOSD was made. Steroid therapy was very effective. When acute pharyngitis is observed in association with significant changes in laboratory parameters despite mild local symptoms, or when pharyngitis is observed in association with joint pain, continuous fever, and a rash, it is important to consider AOSI)

     

36. SANDHU RS, TREHARNE GJ, JUSTICE EA, JORDAN AC, SARAVANA S, OBRENOVIC K, ERB N, KITAS GDet ROWE IF: Accessibility and quality of secondary care rheumatology services for people with inflammatory arthritis: a regional survey, Clin.Med., Vol. 7(6), 579-584., 2007
    Organism:Primary Care Musculoskeletal Research Centre, Keele UniversityFAU - Sandhu, R S
    Abstract:Secondary care rheumatology services for patients with inflammatory arthritis (IA) in the West Midlands were audited using Arthritis and Musculoskeletal Alliance (ARMA) standards of care. Questionnaires were analysed from 1,715 patients in 11 rheumatology departments. ARMA standards recommend full multidisciplinary team assessment; referral rates to nurse specialists (52.3%), physiotherapists (48.7%) and occupational therapists (36.5%) were, however, lower than expected. Attendance at existing hospital-led education groups was rare (8.9%), awareness of existing helplines was moderate (59.2%) but the proportion of patients reporting satisfaction with advice about their disease was high (80.5%). Significant variations were found between departments. For patients with IA < 2 years (n = 236), 84.5% were seen by a rheumatologist within the ARMA standard of 12 weeks of referral; diagnosis of a type of IA was made at the first rheumatology appointment in 66.4%; 82.8% of rheumatoid arthritis patients had commenced disease-modifying drugs, although time to commencement varied across departments. This study raises issues regarding provision of rheumatology services, prioritisation of patient referral and patient education

     

37. SHIMADA M, ONIZUKA M, MACHIDA S, SUZUKI R, KOJIMA M, MIYAMURA K, KODERA Y, INOKO Het ANDO K: Association of autoimmune disease-related gene polymorphisms with chronic graft-versus-host disease, Br.J.Haematol., Vol. 139(3), 458-463., 2007
    Organism:Department of Haematology and Oncology, Tokai University School of Medicine, Isehara, Kanagawa, JapanFAU - Shimada, Masako
    Abstract:Chronic graft-versus-host disease (GVHD) is the most common cause of poor outcomes after haematopoietic stem cell transplantation (HSCT), while the pathophysiology of chronic GVHD remains poorly understood. As both chronic GVHD and autoimmune disease share clinical features, we speculated that autoimmune disease-related genes might be candidate chronic GVHD-related genes. Recent large-scale cohort studies showed that Fc receptor-like 3 gene (FCRL3) single nucleotide polymorphism (SNP) and peptidylarginine deiminases citrullinating enzymes 4 gene (PADI4) haplotype were associated with autoimmune disease. The present study investigated the association between polymorphisms of these two genes and the incidence of chronic GVHD. We analysed 123 cases of Japanese human leucocyte antigen-matched sibling recipients and their donors who underwent HSCT. Although PADI4, which is the rheumatoid arthritis-specific related gene, was not associated with the occurrence of chronic GVHD, the recipient FCRL3-169C/C genotype was significantly less frequent in chronic GVHD patients than in those without chronic GVHD (P = 0.0086). There was no relationship between FCRL3 polymorphism and acute GVHD. As FCRL3 is expressed by B cells and might have an important role in immunoregulation, this significant protective genetic effect raises the question of whether FCRL3 might also be involved in the pathogenesis of chronic GVHD

     

38. SIMON D: Management of growth retardation in juvenile idiopathic arthritis, Horm.Res., Vol. 68 Suppl 5, 122-125., 2007
    Organism:Service d'Endocrinologie Pediatrique, Hopital Robert Debre, Paris, France Dominiquesimon@rdbaphpfrFAU - Simon, Dominique
    Abstract:BACKGROUND: Inflammation and glucocorticoid therapy are major factors in the growth retardation seen in children with severe forms of juvenile idiopathic arthritis (JIA). It has been recently shown that tumor necrosis factor (TNF)-alpha antagonist therapy can improve growth velocity in JIA patients; however, the recombinant human soluble TNF-alpha receptor fusion protein etanercept has had limited efficacy in systemic forms of JIA. For several years, growth hormone (GH) has been used to treat growth retardation in patients with JIA receiving glucocorticoids. GH treatment can normalize growth velocity and prevent the severe loss of height; however, catch-up growth markedly varies with the severity of the inflammatory state and the steroid doses used during GH treatment. Recently, early institution of GH treatment has been shown to maintain normal growth in children with JIA. CONCLUSIONS: These promising results show the need for careful monitoring of growth in children with JIA, the utility of GH therapy before the onset of severe growth delay and the potential for preservation of long-term growth during disease progression

     

39. SINGH R, AGGARWAL Aet MISRA R: Th1/Th17 cytokine profiles in patients with reactive arthritis/undifferentiated spondyloarthropathy, J.Rheumatol., Vol. 34(11), 2285-2290., 2007
    Organism:Department of Immunology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, IndiaFAU - Singh, Rajeev
    Abstract:OBJECTIVE: Data on synovial fluid (SF) cytokine concentrations in patients with reactive arthritis (ReA) or undifferentiated spondyloarthropathy (uSpA) are limited and contradictory. We measured levels of several proinflammatory and immunoregulatory cytokines in SF and sera from patients with ReA/uSpA. METHODS: Interleukin 17 (IL-17), IL-6, interferon-g (IFN-g), and IL-12p40, and immunoregulatory cytokines IL-10 and transforming growth factor-beta (TGF-beta) were assayed using ELISA in SF specimens from 51 patients with ReA/uSpA (ReA 21, uSpA 30), 40 patients with rheumatoid arthritis (RA), and 11 patients with osteoarthritis (OA). IL-17, IL-6, IFN-g, and IL-10 levels were also measured in paired sera samples from patients with ReA/uSpA. RESULTS: SF concentrations of IL-17, IL-6, TGF-beta, and IFN-g were significantly higher in patients with ReA/uSpA as compared to RA patients (for IL-17 median 46 pg/ml, range < 7.8-220 vs median < 7.8 pg/ml, range < 7.8-136, p < 0.05; for TGF-beta median 4.2 ng/ml, range 1.32-12 vs median 3.01 ng/ml, range 0.6-9.6, p < 0.01; for IL-6 median 58 ng/ml, range 2-540 vs median 34.5 ng/ml, range < 0.009-220, p < 0.05; for IFN-g median 290 pg/ml, range < 9.4-1600 vs median 100 pg/ml, range < 9.4-490, p < 0.05). SF levels of IL-10 were comparable but the ratio of IFN-g/IL-10 was significantly higher in ReA/uSpA patients than RA patients (median 3.18, range 0.06-200 for ReA/uSpA vs median 1.0, range 0.03-26.9 for RA; p < 0.05). IL-17, IL-6, IL-10, and IFN-g SF levels were significantly higher than paired serum levels in ReA/uSpA patients (p < 0.01 for IL-17, p < 0.0001 for IL-6, p < 0.0001 for IL-10, and p < 0.001 for IFN-g). CONCLUSION: Increased IL-17, IL-6, TGF-beta, and IFN-g concentrations in ReA/uSpA than in RA suggest that Th1 and Th17 cells could be the major agents in inflammation in ReA/uSpA

     

40. SINGH S, SAMANT Ret JOSHI VR: Adult onset Still's disease: a study of 14 cases, Clin.Rheumatol., Vol. 27(1), 35-39., 2008
    Organism:Department of Rheumatology, PD Hinduja National Hospital & Medical Research Center, VS Marg, Mahim (w), Mumbai-16, IndiaFAU - Singh, S
    Abstract:We studied the clinical profile, laboratory parameters, disease course, and outcomes of patients with adult onset Still's disease (AOSD). A retrospective analysis of adult patients with Still's disease diagnosed from 2000 to 2004 was carried out. Their clinical features and laboratory findings at presentation, disease course, and outcomes were analyzed. Data of 14 patients with Still's disease were analyzed. The age at disease onset ranged from 16 to 59 years with a mean of 29.85, the male to female ratio being 9:5. The mean duration of illness from onset of symptoms to presentation was 14.5 months (range). The most common clinical manifestations were fever (n = 14), articular symptoms (n = 14), rash (n = 8), weight loss (n = 12), and sore throat (n = 5). Elevated ESR was present in all patients with a mean of 98.3 mm at 1 h. Hepatic enzymes were elevated in seven patients at disease onset. The mean duration of follow up was 19.14 months (range). Three patients progressed to chronic arthropathy. Cyclosporine led to dramatic recovery in five patients. Macrophage activation syndrome (MAS) was present in two patients, one after sulfasalazine therapy. One patient with MAS died. Still's disease, although uncommon, has characteristic constellation of clinical and laboratory features and should be considered in the differential diagnosis of fever of unknown origin. Nonsteroidal anti-inflammatory drugs, steroids, and methotrexate may not be always effective, and cyclosporine is an effective drug in resistant cases. Sulfasalazine should be avoided in cases of AOSD

     

41. THORNTON J, ASHCROFT D, O'NEILL T, ELLIOTT R, ADAMS J, ROBERTS C, ROONEY Met SYMMONS D: A systematic review of the effectiveness of strategies for reducing fracture risk in children with juvenile idiopathic arthritis with additional data on long-term risk of fracture and cost of disease management, Health Technol.Assess., Vol. 12(3), 1-208., 2008
    Organism:Arthritis Research Campaign Epidemiology Unit, School of Translational Medicine, University of Manchester, UKFAU - Thornton, J
    Abstract:OBJECTIVES: To review outcome measures and treatment costs in children with juvenile idiopathic arthritis (JIA) and low bone mineral density (BMD) and/or fragility fractures. To review evidence for effectiveness and safety of bisphosphonates and calcium and/or vitamin D in these children.To assess long-term bone health in adults with JIA. DATA SOURCES: Major databases were searched up to July 2005 for effectiveness studies and up to January 2005 for costs. REVIEW METHODS: A structured search strategy was conducted. For the evaluation of long-term bone health, outcome data were derived from two cohorts of adult patients with JIA. As there were few published cost data, an ongoing UK longitudinal study (CAPS) provided background data on the cost of managing JIA. RESULTS: Sixteen studies (78 children with JIA) were included. At baseline, the children had BMD below the expected values for age- and sex-matched children; treatment with bisphosphonates increased BMD with mean percentage increases in spine BMD varying from 4.5 to 19.1%. None of the studies with control groups compared results between the intervention and control groups, they only compared each group with its own baseline. Overall, studies were heterogeneous in design, of variable quality and with no consistency in methods of assessing and reporting outcomes. Hence, data could not be combined or an effect size calculated. A further 43 papers were included in the safety review; side-effects were generally transient. Two studies assessed treatment with calcium and/or vitamin D; BMD was increased from 0.75 to 0.830 g/cm2 after 6 months and BMD Z-score from -2.8 to -2.3 after 6 months and -2.4 after 1 year. There are relatively few long-term studies on the occurrence of low BMD and fragility fractures in children with JIA, with most studies only following children for 1 or 2 years. However, the long- and short-term data indicate that children with JIA have a lower BMD and more fractures than children without JIA. There are very few data on long-term bone health from adults who have JIA, but studies indicate that low BMD persists into adulthood, although adults in remission from JIA may attain the same BMD as healthy adults. From the available data, any predictors of low BMD and fractures in children and adults with JIA remain uncertain. No studies were found that discussed the costs of treating children with JIA and low BMD and/or fragility fractures. In CAPS, 297 of 457 children with JIA attended a 12-month follow-up visit. The mean annual total cost per child in the first year after diagnosis was 1649 pounds (standard deviation 1093 pounds, range 401-6967 pounds). The highest cost component was appointments with paediatric rheumatologists. The study is continuing to accrue and follow up patients and further analyses will be undertaken as the study progresses. CONCLUSIONS: BMD, adjusted for size, should be assessed as the primary outcome in studies of bone health in children with JIA. Quantitative computed tomography could be used where equipment is available as it offers the advantage of measuring volumetric density. Bisphosphonates are a promising treatment for osteoporosis in children with JIA, but the quality of the current evidence is poor. The accurate assessment of outcome is crucial. There are still uncertainties about the use of bisphosphonates in children, including whether the positive effects of treatment continue over time, the length of treatment and the maximal bone mass gain that can be achieved. Adults with JIA may have persistent low BMD compared with an otherwise healthy population together with an increased risk of fracture. There are no studies evaluating the costs of treating children with JIA and low BMD and/or fragility fractures. There are few data evaluating the costs of treating JIA in general. In the first 12 months after diagnosis, children with all JIA disease subtypes consume large, but highly variable, quantities of health service resources, the largest component being the consultant rheumatology appointments. Data from a larger cohort, over a longer period, are required to substantiate these results further. Further research is needed to assess more clearly the role and permit licensing of bisphosphonates for treatment of children, and in particular, longer-term studies

     

42. TRISTANO AG: Macrophage activation syndrome: A frequent but under-diagnosed complication associated with rheumatic diseases, Med.Sci.Monit., Vol. 14(3), RA27-RA36, 2008
    Organism:Department of Internal Medicine, Dr Domingo Luciani Hospital, Caracas, Venezuela and Department pf Pharmaceutical and Administrative Sciences, College of Pharmacy, Nova Southeastern University, Fort Lauderdale, FL, USAFAU - Tristano, Antonio G
    Abstract:Macrophage activation syndrome (MAS) or hemophagocytic syndrome is a severe complication of chronic rheumatic diseases especially in systemic-onset juvenile rheumatoid arthritis (JRA). Although the cause of MAS is unknown, dysregulation of macrophage-lymphocyte interactions with subsequent increases in the levels of both T cell-derived and macrophage-derived cytokines could be involved in this syndrome, leading to an intense systemic inflammatory reaction, which accounts for the main clinical picture. Patients usually present with an acute febrile illness, hepatosplenomegaly, lymphadenopathy, cutaneous and mucosal bleeding, pancytopenia, and central nervous system, cardiac, and renal involvement. Treatment of MAS in patients with rheumatic diseases has not been standardized yet, but it commonly includes a variety of agents such as high-dose corticosteroids, cyclosporine, cyclophosphamide, etoposide, and intravenous immunoglobulin (IVIG). This article reviews the current literature about the pathogenesis, clinical manifestation, diagnosis, and treatment of this severe complication associated with rheumatic diseases.<br />

     

43. TYNJALA P, KOTANIEMI K, LINDAHL P, LATVA K, AALTO K, HONKANEN Vet LAHDENNE P: Adalimumab in juvenile idiopathic arthritis-associated chronic anterior uveitis, Rheumatology (Oxford)., Vol. 47(3), 339-344., 2008
    Organism:Department of Pediatric Rheumatology, Hospital for Children and Adolescents, Helsinki, Finland pirjotynjala@husfiFAU - Tynjala, P
    Abstract:OBJECTIVE: To evaluate the efficacy of adalimumab in juvenile idiopathic arthritis (JIA)-associated uveitis. METHODS: Retrospective observational study of 20 patients with JIA and chronic uveitis on adalimumab treatment. The ocular inflammation and improvement was assessed according to the Standardization of Uveitis Nomenclature criteria. RESULTS: At the initiation of adalimumab, the mean age of patients was 13.4 yrs and the mean duration of uveitis 8.7 yrs. Seventeen (85%) patients had polyarticular JIA and 19 (95%) had previously been on anti-TNF treatment. The mean duration of adalimumab therapy was 18.7 months. Of the 20 patients, 7 (35%) showed improved activity, 1 (5%) worsening activity and in 12 (60%) no change was observed in the activity of uveitis. Those with improved activity were younger and had shorter disease duration. The mean number of flares/yr decreased from 1.9 to 1.4 during adalimumab treatment. Serious adverse events or side-effects were not observed. Seven patients discontinued adalimumab during the follow-up: six because of inefficacy and one because of inactive uveitis. CONCLUSION: Adalimumab is a potential treatment option in JIA-associated uveitis, even in patients non-responsive to previous other anti-TNF therapy

     

44. WERNER CA, KALISKI KS, SALAZAR QK, BUSTOS ML, ROJAS RM, BAUMERT LCet LEAL LH: [Knowledge about their disease and treatment among patients with rheumatoid arthritis], Rev.Med.Chil., Vol. 134(12), 1500-1506., 2006
    Organism:Servicio de Medicina Interna y Unidad de Reumatologia, Hospital Dr Hernan Henriquez Aravena, Temuco amwerner@surnetclFAU - Werner C, Ana Maria
    Abstract:BACKGROUND:The transfer of information in the physician-patient relationship is important, especially in chronic diseases such as Rheumatoid Arthritis (RA), since it influences the perception and behavior that the patient has about his or her illness. AIM: To evaluate the level of knowledge and concern of their illness and treatment and their relationship with functional disability, perception of the pain and global assessment of disease activity, in patients with RA. PATIENTS AND METHODS: Cross sectional study of 104 patients (mean age 56 years, 100 women) with RA. Demographic and clinical variables were registered. The knowledge about their illness and requirement of further information and concern about aspects of the illness and treatment, were assessed. Physical functioning was measured using the Health Assessment Questionnaire (HAQ). A visual analogue (VAS) and Likert scales were used for a global assessment of disease activity. RESULTS: Sixty percent of patients had only primary school studies. The median evolution of the illness was 14 years. Ninety eight percent knew their diagnosis and 91% required further information. There was a high degree of concern about the disease and treatment. The average HAQ score was 0.9. There was a statistically significant relationship between HAQ score and pain VAS (r =0.41, p <0.01). There was a moderate agreement between the global assessment of disease activity made by patients and physicians (Kappa =0.499; p =0.000). CONCLUSIONS: Even though patients with RA are informed about their disease, they require further information. Their highest concern is about the functional consequences of RA and they perceive a higher activity of the disease than their treating physicians

     

45. WOO P: Anakinra treatment for systemic juvenile idiopathic arthritis and adult onset Still disease, Ann.Rheum.Dis., Vol. 67(3), 281-282., 2008
    Organism:

46. WORCH J, RITTER Jet FRUHWALD MC: Presentation of acute promyelocytic leukemia as granulocytic sarcoma, Pediatr.Blood Cancer., Vol. 50(3), 657-660., 2008
    Organism:University Children's Hospital Muenster, Department of Pediatric Hematology and Oncology, Muenster, GermanyFAU - Worch, Jennifer
    Abstract:Granulocytic sarcoma (GS) is a localized tumor composed of immature myeloid cells. This extramedullary tumor can present before, concurrent with or after the diagnosis of acute myeloid leukemia. GS is extremely uncommon in acute promyelocytic leukemia (APL). As a proportion of patients never develop systemic disease, correct and timely diagnosis may be rather difficult, but is a prerequisite for optimal outcome. GS should be considered in the differential diagnosis of children with unusual bone lesions. We describe a patient with GS who presented with symptoms mimicking osteomyelytis or rheumatoid disease

     

47. WU FQ, LUAN Z, LAI JM, TANG XF, LU J, LIU ZWet WANG TY: [Treatment of refractory rheumatism among preschool children with autologous peripheral blood hematopoietic stem cell transplantation], Zhonghua Er.Ke.Za Zhi., Vol. 45(11), 809-813., 2007
    Organism:Department of Pediatric Rheumatology, Capital Institute of Pediatrics, Beijing 100020, ChinaFAU - Wu, Feng-qi
    Abstract:OBJECTIVE: To investigate the feasibility and safety of autologous peripheral blood hematopoietic stem cell transplantation (auto-PBHSCT) and its therapeutic effect on refractory rheumatism among preschool children. METHODS: Three boys with juvenile rheumatoid arthritis (JRA), juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM) respectively, 3 to 6 years old with the mean age of 5 years with 3.5 to 22 months course of disease with 14 months on average, received auto-PBHSCT. Their conditions were so severe that conventional therapy failed to control the diseases. The changes of both clinical manifestations and immunologic indexes were observed before and after transplantation with long term following up at specialty clinic of rheumatism. RESULT: The time when neutrophil count >or= 0.5 x 10(9)/L in the 3 children was days +9, +13 and +11 respectively, that of platelet count >or= 20 x 10(9)/L was days +14, +18 and +13 respectively. The cellular immune function remained abnormal with CD4 cells at a low level and CD4/CD8 being inverted. As to the JDM child, the skin rash had disappeared and his muscle tone was improved to grade 5 within one month after the transplantation. The EMG and serum creatase level returned to normal and muscle MRI findings were improved greatly within 2 months after the transplantation. As to the JSLE child, skin rash and proteinuria had disappeared, MRI of brain showed that the pathological changes had been absorbed and EEG returned to normal 3 months after the transplantation, all the autoantibodies turned to negative within 8 months after transplantation. As to the JRA child, the arthritis had been improved remarkably within 3 weeks after auto-PBHSCT. There was no swelling of joints nor movement limitation 3 months post transplantation. The steroids and immunosuppressive drugs were discontinued post transplantation. Cushing syndrome disappeared. Their body heights increased by 10 to 15 cm in the past 18 months, and they all returned to school. There was no relapse during follow-up periods of 25 - 27 months. CONCLUSION: The therapy with auto-PBHSCT for refractory rheumatism among preschool children was remarkably effective in a short-term, yet the safety and long-term effect still need to be further studied

     

48. YOKOTA S: [Recent topics on rheumatism and collagen diseases in childhood], Nippon Naika Gakkai Zasshi., Vol. 96(10), 2226-2234., 2007
    Organism:

49. ZHA QL, HE YTet YU JP: [Correlations between diagnostic information and therapeutic efficacy in rheumatoid arthritis analyzed with decision tree model], Zhongguo Zhong.Xi.Yi.Jie.He.Za Zhi., Vol. 26(10), 871-876., 2006
    Organism:Jiangxi University of Traditional Chinese Medicine, NanchangFAU - Zha, Qing-lin
    Abstract:OBJECTIVE: To explore the correlations between diagnostic information and therapeutic efficacy in rheumatoid arthritis (RA) with decision tree model analysis. METHODS: Three hundred and ninety seven patients came from 9 clinical centers were randomly divided into the Western medicine (WM) group (n=194) treated with non-steroidal anti-inflammatory drugs and slow-acting antirheumatic drug and the Chinese medicine (CM) group (n=203) with basic therapy and syndrome-differentiation dependant TCM treatment. TCM and WM diagnostic information were collected. The ACR 20 was used for efficacy evaluation and the information of patients before treatment was analyzed by SAS 8.2 statistical package. Through single-factor exploratory analysis, odds ratio of efficacy and variable was calculated taken P < 0.2 as the including criteria for data mining analysis with decision tree model. All data were classified into the training set (75%) and verifying set (25%) with efficacy as the variable for layering to make further verification of the data-mining analysis. RESULTS: Twenty variables were included in the CM group and 26 in the WM group in the data-mining model. In the former, 9 variables were positively correlated to the efficacy, including degree of arthralgia, tenderness and morning stiffness, number of swollen joint, and joint with tenderness, levels of IgM, rheumatoid factor (RF), C-reactive protein (CRP), and total assessment from doctor; and disease duration and degree of nocturnal polyuria were negatively correlated to that. While in the latter, 8 were positively correlated to the efficacy, including erythrocyte sedimentation rate (ESR), sour and weak waist and knees, white fur in tongue, joint ache and stiffness, swollen joint, and total assessment from doctor and patient, and red tongue with yellow fur and leucocyte count negatively correlated to it. Data mining with decision tree analysis revealed that different combinations of morning stiffness, slight red tongue, joint tenderness and nocturnal polyuria in the CM group, and those of white fur in tongue, CRP level, leucocyte count and morning stiffness in the WM group showed different efficacy, which were also verified in the randomly chosen verifying set. CONCLUSION: To analyze the correlations between diagnostic information and therapeutic efficacy with decision tree analysis is conformed to the theory of TCM in applying treatment according to syndrome differentiation individually, thus it would contribute to elevate the accuracy of therapy

     

50. ZHENG WJ, TANG FL, ZHAO Y, CHEN Het DONG Y: [Prevalence of antiepithelial cell antibody in systemic vasculitis and identification of the target antigen thereof], Zhonghua Yi.Xue.Za Zhi., Vol. 85(46), 3272-3276., 2005
    Organism:Department of Rheumatology, Peking Union Medical College Hospital, Beijing 100730, ChinaFAU - Zheng, Wen-jie
    Abstract:OBJECTIVE: To investigate the prevalence of antiepithelial cell antibody (AECA) in systemic vasculitis (SV) and the target antigen thereof. METHOD: Sera of 113 patients with SV of different kinds, 46 patients with Behcet's disease, 23 patients with Takayasu arteritis, 19 patients with Wegener's granulomatosis, 8 patients with polyarteritis nodosa, 7 patients with microscopic polyangiitis, and 10 patients with Churg-Strauss syndrome were collected to detect the protein expression of AECA by Western blotting, with the protein of the endothelial cells of the line EA. hy926 line as substrate. Two-dimensional electrophoresis combined with immunoblotting, liquid chromatography-electrospray ionization mass spectrography was used to detect the target antigens related to vasculitis. Sera of 57 patients with systemic lupus erythematosus (SLE), 25 patients with rheumatoid arthritis (RA), and 20 healthy persons were collected as controls. RESULTS: The AECA-positive rate of the SV patients was 69.0%, not significantly different from that of the SLE patients (66.7%), but significantly higher than those of the RA patients (6.7%, P < 0.01) and healthy persons (0, P < 0.01). The AECA from the SV patients reacted with the endothelial cell (EC) antigens with the molecular size of 26 to 125 kDa, and the AECA from the SLE patients reacted with the EC antigens with the molecular size of 15 to 97 kDa. The EC antigens with the molecular size of 47 kDa was commonly found in the sera of the AECA-positive SV patients and SLE patients, however, was not found in the RA, and polymyositis-dermatomyositis patients. The EC protein reacted by 47 kDa protein was identified by proteomic techniques as alpha-enolase. CONCLUSION: A group of heterogeneous antibodies, AECA can be found frequently in patients with SV and SLA. AECA reacts against a common EC antigen, alpha-enolase

     

51. ZIRKZEE EJ, SNEEP AC, DE BUCK PD, ALLAART CF, PEETERS AJ, RONDAY HK, WESTEDT ML, LE CESSIE Set VLIET VLIELAND TP: Sick leave and work disability in patients with early arthritis, Clin.Rheumatol., Vol. 27(1), 11-19., 2008
    Organism:Department of Rheumatology C1-R, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The NetherlandsFAU - Zirkzee, Elisabeth J M
    Abstract:We studied the occurrence of sick leave and work disability, the presence of workplace adaptations and the usage of professional guidance related to working problems in patients with early arthritis. Inclusion criteria were arthritis symptoms of less than 2 years duration and a paid job at the time of diagnosis. Assessments were done in connection with an early arthritis clinic (EAC) at entry into the cohort and 12 months thereafter by means of a questionnaire comprising questions on sick leave (absenteeism from work reported to the employer), work disability (receiving a full or partial work disability pension), unemployment, work adaptations and professional guidance related to working problems. Fifty-seven of the 69 participants (83%) had an arthritis symptom duration of <6 months. The number of patients with sick leave due to arthritis in the past 12 months decreased from 28 (41%) at study entry to 18 (26%) after 12 months of follow-up. The number of patients receiving a work disability pension increased from 5 (7%) at study entry to 13 (19%) after 12 months of follow-up (10 partial and 3 full). Sick leave in the 12 months before study entry appeared to be the most important predictor of the institution or increase in a work disability pension (odds ratio, 16.1; 95%CI, 1.8-142.8). Between study entry and follow-up, the number of patients with workplace adaptations increased from 20 (29%) to 28 (42%), whereas the number of patients receiving vocational guidance decreased from 48 (70%) to 36 (52%). In patients with early arthritis and a paid job, arthritis-related sick leave was common and occurred in part before patients entered the EAC and a diagnosis was made. About 20% of the patients became permanently work disabled, with partial work disability being more common than full work disability. Considerable proportions of patients received workplace adaptations and professional guidance with working problems