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Objectives To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis. Methods Patients newly treated for 6 months with MTX enrolled in the Paediatric Rheumatology International Trials Organization (PRINTO) MTX trial. Bivariate and logistic regression analyses were used to identify baseline predictors of poor response according to the American College of Rheumatology pediatric (ACR-ped) 30 and 70 criteria. Results In all, 405/563 (71.9%) of patients were women; median age at onset and disease duration were 4.3 and 1.4 years, respectively, with anti-nuclear antibody (ANA) detected in 259/537 (48.2%) patients. With multivariate logistic regression analysis, the most important determinants of ACR-ped 70 non-responders were: disease duration >1.3 years (OR 1.93), ANA negativity (OR 1.77), Childhood Health Assessment Questionnaire (CHAQ) disability index> 1.125 (OR 1.65) and the presence of right and left wrist activity (OR 1.55). Predictors of ACR-ped 30 non-responders were: ANA negativity (OR 1.92), CHAQ disability index> 1.14 (OR 2.18) and a parent's evaluation of child's overall wellbeing

Made available in DSpace on 2022-04-29T08:44:24Z (GMT). No. of bitstreams: 0 Previous issue date: 2011-01-01 Objective: To compare the demographic features, presenting manifestations, diagnostic investigations, disease course, and drug therapies of children with juvenile dermatomyositis (JDM) followed in Europe and Latin America. Methods: Patients were inception cohorts seen between 1980 and 2004 in 27 paediatric rheumatology centres. The following information was collected through the review of patient charts: sex; age at disease onset; date of disease onset and diagnosis; onset type; presenting clinical features; diagnostic investigations; course type; and medications received during disease course. Results: Four hundred and ninety patients (65.5% females, mean onset age 7.0 years, mean disease duration 7.7 years) were included. Disease presentation was acute or insidious in 57.1% and 42.9% of the patients, respectively. The course type was monophasic in 41.3% of patients and chronic polycyclic or continuous in 58.6% of patients. The more common presenting manifestations were muscle weakness (84.9%), Gottron's papules (72.9%), heliotrope rash (62%), and malar rash (56.7%). Overall, the demographic and clinical features of the 2 continental cohorts were comparable. European patients received more frequently high-dose intravenous methylprednisolone, cyclosporine, cyclophosphamide, and azathioprine, while methotrexate and antimalarials medications were used more commonly by Latin American physicians. Conclusion: The demographic and clinical characteristics of JDM are similar in European and Latin American patients. We found, however, several differences in the use of medications between European and Latin American paediatric rheumatologists. © Clinical and Experimental Rheumatology 2011. Istituto di Ricovero e Cura a Carattere Scientifico G Gaslini, Genova Great Ormond Street Hospital for Children, London UCL Institute of Child Health, London Universidade Federal do Rio de Janeiro, Rio de Janeiro Universidade do Estado do Rio de Janeiro, Rio de Janeiro Hospital General de Ninos Pedro de Elizalde Buenos Aires Hospital de Pediatria Juan P. Garrahan, Buenos Aires Hospital das Clinicas Faculdade de Medicina de Ribeirao Preto Universidade de Sao Paulo, Ribeirao Preto Hospital General de Mexico, Mexico City Centro Medical National La Raza, Mexico City Hospital Pequeno Principe, Curitiba Parana Centro Medico Nacional Siglo XXI, Mexico City Faculdade de Medicina de Botucatu Universitade Estadual Paulista, Botucatu Instituto da Criança and Division of Rheumatology Faculdade de Medicina Universidade de São Paulo, São Paulo Presidio Ospedaliera Ospedali Civili Riuniti, Sciacca Ospedale Policlinico Università degli Studi di Chieti, Chieti Ospedale A. Meyer, Firenze Azienda Ospedaliera Universitaria, Messina Università di Torino, Torino Spedali Civili, Brescia Università Cattolica Sacro Cuore, Rome Università degli Studi di Genova, Genova Faculdade de Medicina de Botucatu Universitade Estadual Paulista, Botucatu

Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo.A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used.Vaccination card was seen by 100% of the professionals at the first visit and by 36% annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44%, 64%, and 48%, respectively. The professionals were divided into two groups: Group A (≤ 15 years of practice, n = 12) and B (≥ 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P0.05).The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice.

Background Systemic lupus erythematosus (SLE) is a complex multi-system disease, characterized by both autoimmune and autoinflammatory clinical and laboratory features. The role of type I interferon (IFN) in SLE has been demonstrated from the 2000s, by gene expression analyses showing significant over-expression of genes related to type I IFN signalling pathway (IFN signature). However, several studies questioned the role of measuring the intensity of IFN signature (IFN score) to chase SLE activity. We would assess if the IFN signature can help the clinical and therapeutic stratification of patients with pediatric SLE. Methods We measured the IFN score in peripheral whole blood from a series of subjects with childhood-onset SLE and correlated the results with clinical and laboratory parameters. Results Thirty-one subjects were included in the study, among which the 87% displayed a positive IFN score. The only significant relation was found for high IFN score in subjects with normocomplementemia. No correlation was observed between IFN score and SLEDAI-2K, BILAG-2004 and SLICC. Patients with high IFN score and normal complement levels also presented lower anti-dsDNA antibodies. Conclusions The integration between IFN signature analysis and complement levels may easily distinguish two groups of subjects, in which the autoimmune or autoinflammatory component of the disease seems to be prevalent.

Made available in DSpace on 2013-08-12T19:10:16Z (GMT). No. of bitstreams: 0 Previous issue date: 2010-01-15 Made available in DSpace on 2013-09-30T19:20:41Z (GMT). No. of bitstreams: 0 Previous issue date: 2010-01-15 Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-20T15:33:16Z No. of bitstreams: 0 Made available in DSpace on 2014-05-20T15:33:16Z (GMT). No. of bitstreams: 0 Previous issue date: 2010-01-15 Myositis Association European Union Objective. To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.Methods. Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL).Results. A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%.Conclusion. This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage. Ist Ricovero & Cura Carattere Sci G Gaslini, Genoa, Italy Univ Genoa, Genoa, Italy Great Ormond St Hosp Sick Children, London WC1N 3JH, England UCL, Inst Child Hlth, London, England Univ Fed Rio de Janeiro, Rio de Janeiro, Brazil Universidade do Estado do Rio de Janeiro (UERJ), BR-20550011 Rio de Janeiro, Brazil Hosp Gen Ninos Pedro de Elizalde, Buenos Aires, DF, Argentina Fdn IRCCS Policlin, Milan, Italy Hosp Pediat Juan P Garrahan, Buenos Aires, DF, Argentina Univ São Paulo, Fac Med Ribeirao Preto, Hosp Clin, Ribeirao Preto, Brazil Hosp Gen Mexico City, Mexico City, DF, Mexico Fdn Ist Ricovero & Cura Carattere Sci Policlin S, Pavia, Italy Ctr Med Natl La Raza, Mexico City, DF, Mexico Hosp Pequeno Principe, Curitiba, Parana, Brazil Clin Pediat 1, Padua, Italy Ctr Med Nacl Siglo XXI, Mexico City, DF, Mexico Osped Pediat Bambino Gesu, Rome, Italy Osped Villa Monna Tessa, Florence, Italy Univ Naples Federico 2, Naples, Italy Ist Ortoped Gaetano Pini, Milan, Italy Univ estadual Paulista, Botucatu, SP, Brazil II Clin Pediat, Cagliari, Italy Univ São Paulo, São Paulo, Brazil Ist Ricovero & Cura Carattere Sci Burlo Garofalo, Trieste, Italy Univ estadual Paulista, Botucatu, SP, Brazil EU: AML/B7-311/970666/II-0246-FI