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Objective: To update the recommendations for the treatment of rheumatoid arthritis (RA) with biological therapies, endorsed by the Portuguese Society of Rheu -matology (SPR). Methods: These treatment recommendations were formulated by Portuguese rheumatologists based on litera -ture evidence and consensus opinion. At a national meeting the 11 recommendations were discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the recommendations. Results: These recommendations cover general aspects as shared decision, prospective registry in Reuma.pt, asses sment of activity and RA impact and treatment objec tive. Consensus was also achieved regarding specific aspects as initiation of biologic therapy, assessment of res pon se, switching and definition of persistent remission. Conclusion: These recommendations may be used for guidance of treatment with biological therapies in patients with RA. As more evidence becomes available and more therapies are licensed, these recommendations will be updated. publishersversion published

Background: Methotrexate (MTX) is the first-line drug in the treatment of rheumatoid arthritis (RA) and the most commonly prescribed disease modifying antirheumatic drug. Moreover, it is also used as an adjuvant drug in patients under biologic therapies, enhancing the efficacy of biologic agents. Objectives: To review the literature and update the Portuguese recommendations for the use of MTX in rheumatic diseases first published in 2009. Methods: The first Portuguese guidelines for the use of MTX in rheumatic diseases were published in 2009 and were integrated in the multinational 3E Initiative (Evi - dence Expertise Exchange) project. The Portuguese rheumatologists based on literature evidence and consensus opinion formulated 13 recommendations. At a national meeting, the recommendations included in this document were further discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the updated recommendations. Results: Results presented in this article are mainly in accordance with previous guidelines, with some new information regarding hepatitis B infection during MTX treatment, pulmonary toxicity monitoring, hepatotoxi - city management, association with hematologic neoplasms, combination therapy and tuberculosis scree - ning during treatment. Conclusion: The present recommendations combine scientific evidence with expert opinion and attained desirable agreement among Portuguese rheumatologists. The regular update of these recommendations is essen - tial in order to keep them a valid and useful tool in daily practice. publishersversion published

Patient registries are key instruments aimed at a better understanding of the natural history of diseases, at assessing the effectiveness of therapeutic interventions, as well as identifying rare events or outcomes that are not captured in clinical trials. However, the potential of registries goes far beyond these aspects. For example, registries promote the standardization of clinical practice, can also provide information on domains that are not routinely collected in clinical practice and can support decision-making. Being aware of the importance of registries, the Portuguese Society of Rheumatology developed the Rheumatic Diseases Portuguese Register- Reuma.pt - which proved to be an innovative ins - trument essential to a better understanding of systemic immune-mediated rheumatic diseases. Objective: To describe the contribution of Reuma.pt to the knowledge of systemic immune-mediated rheumatic diseases. Results: Reuma.pt is widely implemented, with 77 centres actively contributing to the recruitment and follow- -up of patients. Reuma.pt follows in a standardized way patients with the following systemic inflam matory rheumatic diseases: rheumatoid arthritis (n=6.218), pso - riatic arthritis (n=1.498), spondyloarthritis (n=2.529), juvenile idiopathic arthritis (n =1.561), auto-inflammatory syndromes (n=122), systemic lupus erythematosus (n =1.718), systemic sclerosis (n=180) and vasculitis (n=221). This platform is intended for use as an electronic medical record, provides standardized assessment of patients and support to the clinical decision, thereby contributing to a better quality of care of rheumatic patients. The research based on Reuma.pt identified genetic determinants of susceptibility and response to therapy, characterized in detail systemic rheumatic diseases and their long-term impact, critically appraised the performance of instruments for monitoring the disease acti - vity, established the effectiveness and safety of biolo gic therapies and identified predictors of response, and proactively engaged patients in the management of their disease. Conclusion: Reuma.pt is an innovative tool, widely established in the country that contributes to a clinical practice of excellence and simultaneously to increase the knowledge of systemic immune-mediated rheuma - tic diseases. Additionally, Reuma.pt fosters patients' participation in the management of the disease. publishersversion published

Our aims were to evaluate the correlation between Juvenile Arthritis Disease Activity Score 27-joint reduced count (JADAS27) with erythrocyte sedimentation rate (ESR) and JADAS27 with C-reactive protein (CRP) scores and to test the agreement of both scores on classifying each disease activity state. We also aimed at verifying the correlation of the 2 scores across juvenile idiopathic arthritis (JIA) categories and to check the correlation between JADAS27-ESR and clinical JADAS27 (JADAS27 without ESR).

Objective: To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. Methods: The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed. Results: A cohort of 56 patients with a mean age at di- sease onset of 12.6±4.04 years (mean±1SD) (range, 1.0- -17.0 years) and a mean period of follow-up of 5.5±5.4 years. Forty six (82.1%) patients were female. The most common disease manifestations were musculoskeletal (87.5%), mucocutaneous (80.3%) and haematological abnormalities (75%). Lupus nephritis was diagnosed in 46.4% of patients and consisted of glomerular ne - phritis in all cases. Neuropsychiatric manifestations occurred in 21.4% but severe central nervous system complications were uncommon, as brain infarcts and organic brain syndrome in 4 (7.1%) patients. Antinuclear antibodies and anti-double stranded DNA were positive in most patients in (98.2% and 71.4% respec- tively), as well as low C3 and/or C4 were observed frequently (85.7%). Generally, most patients had a good response to therapy as demonstrated by a significant decreasing of SLEDAI score from disease presentation to the last evaluation. The SLEDAI at diagnosis, the maximum SLEDAI and the incidence of complications were significantly higher in patients with neurolupus and/or lupus nephritis. Therapy included oral steroids (87.5%), hydroxychloroquine (85.7%), azathioprine (55.4%), IV cyclophosphamide (28.6%) along with other drugs. Six (10.7%) patients were treated with rituximab. Long-term remission was achieved in 32%, disease was active in 68%, adverse reactions to therapy occurred in 53.6% and complications/severe manifestations in 23.2%. Two patients died, being active disease and severe infection the causes of death. Conclusions: This study suggests that in our patients the clinical and laboratory features observed were similar to juvenile systemic lupus erythematosus patients from other series. Clinical outcome was favourable in the present study. Complications from therapy were frequent. Objective: To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. Methods: The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed. Results: A cohort of 56 patients with a mean age at di- sease onset of 12.6±4.04 years (mean±1SD) (range, 1.0-17.0 years) and a mean period of follow-up of 5.5±5.4 years. Forty six (82.1%) patients were female. The most common disease manifestations were musculoskeletal (87.5%), mucocutaneous (80.3%) and haematological abnormalities (75%). Lupus nephritis was diagnosed in 46.4% of patients and consisted of glomerular nephritis in all cases. Neuropsychiatric manifestations occur red in 21.4% but severe central nervous system complications were uncommon, as brain infarcts and organic brain syndrome in 4 (7.1%) patients. Antinuclear antibodies and anti-double stranded DNA were positive in most patients in (98.2% and 71.4% respectively), as well as low C3 and/or C4 were observed frequently (85.7%). Generally, most patients had a good response to therapy as demonstrated by a significant decreasing of SLEDAI score from disease presentation to the last evaluation. The SLEDAI at diagnosis, the maximum SLEDAI and the incidence of complications were significantly higher in patients with neurolupus and/or lupus nephritis. Therapy included oral steroids (87.5%), hydroxychloroquine (85.7%), azathioprine (55.4%), IV cyclophosphamide (28.6%) along with other drugs. Six (10.7%) patients were trea- ted with rituximab. Long-term remission was achieved in 32%, disease was active in 68%, adverse reactions to therapy occurred in 53.6% and complications/severe manifestations in 23.2%. Two patients died, being active disease and severe infection the causes of death. Conclusions: This study suggests that in our patients the clinical and laboratory features observed were similar to juvenile systemic lupus erythematosus patients from other series. Clinical outcome was favourable in the present study. Complications from therapy were frequent.

Made available in DSpace on 2022-04-28T19:55:21Z (GMT). No. of bitstreams: 0 Previous issue date: 2001-01-01 We report the cross-cultural adaptation and validation into Brazilian-Portuguese of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children regardless the underlying disease. The Brazilian CHAQ was revalidated, while the CHQ has been derived from the Portuguese version. A total of 471 subjects were enrolled: 157 patients with JIA (27% systemic onset, 38% polyarticular onset, 9% extended oligoarticular subtype, and 26% persistent oligoarticular subtype) and 314 healthy children. The CHAQ discriminated clinically healthy subjects from JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and lower overall well-being scores when compared to their healthy peers. Also the CHQ discriminated clinically healthy subjects from JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being score when compared to their healthy peers. In conclusion the Brazilian versions of the CHAQ-CHQ are reliable and valid tools for the combined physical and psychosocial assessment of children with JIA. © Copyright Clinical and Experimental Rheumatology 2001. Universidade Estadual Paulista, Botucatu Laboratorio di Informatica Medica IRCCS S. Matteo, Pavia Universidade Federal de Uberlândia, Minas Gerais Universidade de São Paulo, Ribeirão Preto Hospital das Clinicas Faculdade de Medicina Universidade de São Paulo Universidade Federal do Rio de Janeiro Universidade Federal de São Paulo Instituto Português de Reumatologia, Lisbon Faculdade de Medicina de Botucatu UNESP Departamento de Pediatria, 18618-970 Botucatu, Sâo Paulo Universidade Estadual Paulista, Botucatu Faculdade de Medicina de Botucatu UNESP Departamento de Pediatria, 18618-970 Botucatu, Sâo Paulo

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Patient registries are key instruments aimed at a better understanding of the natural history of diseases, at assessing the effectiveness of therapeutic interventions, as well as identifying rare events or outcomes that are not captured in clinical trials. However, the potential of registries goes far beyond these aspects. For example, registries promote the standardization of clinical practice, can also provide information on domains that are not routinely collected in clinical practice and can support decision-making. Being aware of the importance of registries, the Portuguese Society of Rheumatology developed the Rheumatic Diseases Portuguese Register- Reuma.pt - which proved to be an innovative ins - trument essential to a better understanding of systemic immune-mediated rheumatic diseases. Objective: To describe the contribution of Reuma.pt to the knowledge of systemic immune-mediated rheumatic diseases. Results: Reuma.pt is widely implemented, with 77 centres actively contributing to the recruitment and follow- -up of patients. Reuma.pt follows in a standardized way patients with the following systemic inflam matory rheumatic diseases: rheumatoid arthritis (n=6.218), pso - riatic arthritis (n=1.498), spondyloarthritis (n=2.529), juvenile idiopathic arthritis (n =1.561), auto-inflammatory syndromes (n=122), systemic lupus erythematosus (n =1.718), systemic sclerosis (n=180) and vasculitis (n=221). This platform is intended for use as an electronic medical record, provides standardized assessment of patients and support to the clinical decision, thereby contributing to a better quality of care of rheumatic patients. The research based on Reuma.pt identified genetic determinants of susceptibility and response to therapy, characterized in detail systemic rheumatic diseases and their long-term impact, critically appraised the performance of instruments for monitoring the disease acti - vity, established the effectiveness and safety of biolo gic therapies and identified predictors of response, and proactively engaged patients in the management of their disease. Conclusion: Reuma.pt is an innovative tool, widely established in the country that contributes to a clinical practice of excellence and simultaneously to increase the knowledge of systemic immune-mediated rheuma - tic diseases. Additionally, Reuma.pt fosters patients' participation in the management of the disease. publishersversion published