Nuno Pina Gonçalves, Mariana Emília Santos, Manuel Silvério-António, Helena Donato, Fernando M Pimentel-Santos, Eduardo Cruz
Aim: To collect and summarize the available scientific evidence that evaluates the effects of physical exercise interventions on axial spondyloarthritis (axSpA).
Methods: A systematic review was conducted in accordance to the guidance of Preferred Reporting Items for Systematic review and Meta-Analysis (PRISMA) to collect randomized controlled trials on the PubMed, Embase and Web of Science Core Collection databases. The search strategy included terms regarding physical exercise interventions targeted to axSpA participants and all of its variants in multiple combinations adapted to each one of the databases regarding its own special requirements. Several outcomes were defined: Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath ankylosing spondylitis functional index (BASFI), Bath Ankylosing Spondylitis Metrology Index (BASMI), ASDAS (Ankylosing Spondylitis Disease Activity Score), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), the 36-item short form health survey (SF-36) and the Ankylosing Spondylitis Quality of Life questionnaire (ASQoL). Two independent researchers screened the titles and abstracts followed by full-text analysis when suitable, using EndnoteTM online. Selected articles, according to exclusion/inclusion criteria defined, were submitted to data extraction and bias assessment was performed for each study's outcomes using the Cochrane risk-of-bias tool for randomized trials.
Results: A total of 2063 articles were identified through the electronic databases search. After removal of duplicates, 1435 were eligible for screening, of which 45 articles went through full text evaluation. Only 24 articles met the inclusion/exclusion criteria. Physical exercise contributes for a statistically significant improvement of BASDAI in 13 studies, BASFI in 10, BASMI in 6, ASDAS in 3, CRP in 2, ESR in 1, SF-36 in 2 and ASQoL in 3.No major adverse effects were reported and an overall benefit was noted with the implementation of physical exercise as a treatment modality for axSpA.
Conclusion: Physical exercise seems to be an effective non-pharmacological therapy for axSpA, with positive effects in disease activity, physical function, and quality of life.
目的:收集和总结评价体育锻炼干预对轴性脊椎关节炎(axSpA)疗效的现有科学证据,Embase和Web of Science核心收藏数据库。搜索策略包括关于针对axSpA参与者的体育锻炼干预措施的术语,以及其所有变体的多个组合,这些组合适用于关于其自身特殊要求的每个数据库。定义了几个结果:巴斯强直性脊柱炎疾病活动指数(BASDAI)、巴斯强直性脊椎炎功能指数(BASFI)、巴斯脊柱炎计量指数(BASMI)、ASDAS(强直性脊柱炎活动评分)、C反应蛋白(CRP)、红细胞沉降率(ESR),36项简式健康调查(SF-36)和强直性脊柱炎生活质量问卷(ASQoL)。两名独立研究人员对标题和摘要进行了筛选,然后在适当的时候使用EndnoteTM在线进行全文分析。根据定义的排除/纳入标准,将选定的文章进行数据提取,并使用随机试验的Cochrane偏倚风险工具对每项研究的结果进行偏倚评估。结果:通过电子数据库检索,共检索到2063篇文章。去除重复后,1435篇文章符合筛选条件,其中45篇文章通过了全文评估。只有24篇文章符合纳入/排除标准。体育锻炼有助于13项研究中BASDAI的统计学显著改善,10项研究中有BASFI,6项研究中的BASMI,3项研究中ASDAS,2项研究中CRP,1项研究中ESR,SF-36 2例,ASQoL 3例。没有重大不良反应的报告,并注意到体育锻炼作为axSpA的治疗方式的总体益处。结论:体育锻炼似乎是axSpA有效的非药物治疗,对疾病活动、身体功能和生活质量有积极影响。
{"title":"The effects of physical exercise on axial spondyloarthritis - a systematic review.","authors":"Nuno Pina Gonçalves, Mariana Emília Santos, Manuel Silvério-António, Helena Donato, Fernando M Pimentel-Santos, Eduardo Cruz","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Aim: </strong>To collect and summarize the available scientific evidence that evaluates the effects of physical exercise interventions on axial spondyloarthritis (axSpA).</p><p><strong>Methods: </strong>A systematic review was conducted in accordance to the guidance of Preferred Reporting Items for Systematic review and Meta-Analysis (PRISMA) to collect randomized controlled trials on the PubMed, Embase and Web of Science Core Collection databases. The search strategy included terms regarding physical exercise interventions targeted to axSpA participants and all of its variants in multiple combinations adapted to each one of the databases regarding its own special requirements. Several outcomes were defined: Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath ankylosing spondylitis functional index (BASFI), Bath Ankylosing Spondylitis Metrology Index (BASMI), ASDAS (Ankylosing Spondylitis Disease Activity Score), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), the 36-item short form health survey (SF-36) and the Ankylosing Spondylitis Quality of Life questionnaire (ASQoL). Two independent researchers screened the titles and abstracts followed by full-text analysis when suitable, using EndnoteTM online. Selected articles, according to exclusion/inclusion criteria defined, were submitted to data extraction and bias assessment was performed for each study's outcomes using the Cochrane risk-of-bias tool for randomized trials.</p><p><strong>Results: </strong>A total of 2063 articles were identified through the electronic databases search. After removal of duplicates, 1435 were eligible for screening, of which 45 articles went through full text evaluation. Only 24 articles met the inclusion/exclusion criteria. Physical exercise contributes for a statistically significant improvement of BASDAI in 13 studies, BASFI in 10, BASMI in 6, ASDAS in 3, CRP in 2, ESR in 1, SF-36 in 2 and ASQoL in 3.No major adverse effects were reported and an overall benefit was noted with the implementation of physical exercise as a treatment modality for axSpA.</p><p><strong>Conclusion: </strong>Physical exercise seems to be an effective non-pharmacological therapy for axSpA, with positive effects in disease activity, physical function, and quality of life.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"None"},"PeriodicalIF":0.0,"publicationDate":"2023-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41177180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ana Bento da Silva, Maria Helena Lourenço, Maria João Gonçalves, Jaime C Branco, Manuela Costa
{"title":"Butterfly Vertebra: the mimicker of vertebral fragility fractures.","authors":"Ana Bento da Silva, Maria Helena Lourenço, Maria João Gonçalves, Jaime C Branco, Manuela Costa","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"None"},"PeriodicalIF":0.0,"publicationDate":"2023-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41141113","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ana Martins, Daniela Oliveira, Frederico Rajão Martins, Rafaela Nicolau, Filipe Oliveira Pinheiro, Maria Seabra Rato, Sofia Pimenta, Lúcia Costa, Miguel Bernardes
Introduction: Immune-mediated skin lesions (IMSL) can be very disabling leading to treatment discontinuation. Although these lesions have rarely been previously described, the true incidence is unknown.
Objective: To explore the cumulative incidence, management and outcomes of IMSL related to bDMARD in a large cohort of patients with chronic inflammatory rheumatic diseases. To explore possible associations and risk factors for IMSL development.
Methods: A retrospective single-center study of patients with rheumatoid arthritis (RA), spondylarthritis (SpA) and psoriatic arthritis (PsA) that had been treated with at least one bDMARD for at least 6 months was conducted. IMSL related to bDMARD characteristics and outcomes were collected.
Results: A total of 989 patients with RA, SpA and PsA were included. Twenty-seven patients (2.7%) presented IMSL potentially related to bDMARD, being psoriasis the most common IMSL (n=12, 44.4%), followed by drug-induced lupus erythematosus (n=6), alopecia areata (n=3) and leukocytoclastic vasculitis (n=2). IMSL led to withdrawal of bDMARD in 18 of the 27 patients (66.7%). Patients with IMSL had younger age at diagnosis (p=0.038), longer disease duration (p=0.018), longer duration of bDMARD treatment (p=0.008), and higher number of previous bDMARDs (p < 0.001) than patients without IMSL. In the group of patients with IMSL there was a significantly higher percentage of patients treated with adalimumab (p < 0.001). In multivariate regression model, the number of previous bDMARDs (OR 2.13, 95%CI 1.47-3.10, p < 0.001) and treatment with adalimumab (OR 4.60, 95%CI 1.96-10.80 , p < 0.001) were statistically significant predictive factors for IMSL development.
Conclusion: In our study, IMSL related to bDMARDs had an estimated cumulative incidence of 2.7%. Younger age at diagnosis, longer disease duration, longer duration of bDMARD treatment, higher number of previous bDMARDs and treatment with adalimumab were independently associated with an increased risk of IMSL development.
{"title":"Immune-mediated skin lesions related to biological disease-modifying antirheumatic drugs: a 22-year experience of a tertiary center.","authors":"Ana Martins, Daniela Oliveira, Frederico Rajão Martins, Rafaela Nicolau, Filipe Oliveira Pinheiro, Maria Seabra Rato, Sofia Pimenta, Lúcia Costa, Miguel Bernardes","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Introduction: </strong>Immune-mediated skin lesions (IMSL) can be very disabling leading to treatment discontinuation. Although these lesions have rarely been previously described, the true incidence is unknown.</p><p><strong>Objective: </strong>To explore the cumulative incidence, management and outcomes of IMSL related to bDMARD in a large cohort of patients with chronic inflammatory rheumatic diseases. To explore possible associations and risk factors for IMSL development.</p><p><strong>Methods: </strong>A retrospective single-center study of patients with rheumatoid arthritis (RA), spondylarthritis (SpA) and psoriatic arthritis (PsA) that had been treated with at least one bDMARD for at least 6 months was conducted. IMSL related to bDMARD characteristics and outcomes were collected.</p><p><strong>Results: </strong>A total of 989 patients with RA, SpA and PsA were included. Twenty-seven patients (2.7%) presented IMSL potentially related to bDMARD, being psoriasis the most common IMSL (n=12, 44.4%), followed by drug-induced lupus erythematosus (n=6), alopecia areata (n=3) and leukocytoclastic vasculitis (n=2). IMSL led to withdrawal of bDMARD in 18 of the 27 patients (66.7%). Patients with IMSL had younger age at diagnosis (p=0.038), longer disease duration (p=0.018), longer duration of bDMARD treatment (p=0.008), and higher number of previous bDMARDs (p < 0.001) than patients without IMSL. In the group of patients with IMSL there was a significantly higher percentage of patients treated with adalimumab (p < 0.001). In multivariate regression model, the number of previous bDMARDs (OR 2.13, 95%CI 1.47-3.10, p < 0.001) and treatment with adalimumab (OR 4.60, 95%CI 1.96-10.80 , p < 0.001) were statistically significant predictive factors for IMSL development.</p><p><strong>Conclusion: </strong>In our study, IMSL related to bDMARDs had an estimated cumulative incidence of 2.7%. Younger age at diagnosis, longer disease duration, longer duration of bDMARD treatment, higher number of previous bDMARDs and treatment with adalimumab were independently associated with an increased risk of IMSL development.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"None"},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41150415","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Raquel Rainho, Daniela Oliveira, Miguel Bernardes, Lúcia Costa, Carlos Vaz, João Almeida Fonseca, Cristina Jácome
TBackground: The evaluation of perceptions of patients with rheumatoid arthritis (RA) has a positive influence in their health outcomes and overall experience of care. The Commissioning for Quality in Rheumatoid Arthritis Patient-Reported Experience Measure (CQRA-PREM) was developed to provide the perceptions and the feedback of the patients with RA to the health professionals team. This PREM is already validated and in use in the United Kingdom (UK) and Netherlands. In Portugal, there is no validated PREM to evaluate the experience of patients with RA.
Objective: To translate, cultural adapt and validate the content of the CQRA-PREM for the Portuguese population.
Methods: A qualitative study using focus groups was conducted to evaluate CQRA-PREM content validity. The CQRA-PREM was first translated and cultural adapted to Portuguese by two researchers, and after back translation, a panel of experts agreed on the preliminary Portuguese version of CQRA-PREM. Patients with RA were recruited from a rheumatology center at a tertiary university hospital center to participate in focus group meetings. Before the focus group they filled in the preliminary version of CQRA-PREM, with its 7 domains and 24 items (Likert scale 1-5).
Results: Twelve participants (median 54 (45-58) years old; 92% female) were included in two focus groups. All domains of the questionnaire had medians of 3 or above. Seven major themes and six subthemes emerged. Participants considered the questionnaire as very clear and simple and with adequate questions. Patients pointed as extremely important being treated with dignity and respect and considered the awareness of the multidisciplinary team and the presentation of support programs and organizations as areas for improvement.
Conclusion: The Portuguese version of the CQRA-PREM is acceptable and its content is valid in the perspective of patients with RA to assess the quality of care provided by the healthcare services.
{"title":"Content validity of a patient-reported experience measure (CQRA-PREM) for patients with rheumatoid arthritis in Portugal.","authors":"Raquel Rainho, Daniela Oliveira, Miguel Bernardes, Lúcia Costa, Carlos Vaz, João Almeida Fonseca, Cristina Jácome","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>TBackground: The evaluation of perceptions of patients with rheumatoid arthritis (RA) has a positive influence in their health outcomes and overall experience of care. The Commissioning for Quality in Rheumatoid Arthritis Patient-Reported Experience Measure (CQRA-PREM) was developed to provide the perceptions and the feedback of the patients with RA to the health professionals team. This PREM is already validated and in use in the United Kingdom (UK) and Netherlands. In Portugal, there is no validated PREM to evaluate the experience of patients with RA.</p><p><strong>Objective: </strong>To translate, cultural adapt and validate the content of the CQRA-PREM for the Portuguese population.</p><p><strong>Methods: </strong>A qualitative study using focus groups was conducted to evaluate CQRA-PREM content validity. The CQRA-PREM was first translated and cultural adapted to Portuguese by two researchers, and after back translation, a panel of experts agreed on the preliminary Portuguese version of CQRA-PREM. Patients with RA were recruited from a rheumatology center at a tertiary university hospital center to participate in focus group meetings. Before the focus group they filled in the preliminary version of CQRA-PREM, with its 7 domains and 24 items (Likert scale 1-5).</p><p><strong>Results: </strong>Twelve participants (median 54 (45-58) years old; 92% female) were included in two focus groups. All domains of the questionnaire had medians of 3 or above. Seven major themes and six subthemes emerged. Participants considered the questionnaire as very clear and simple and with adequate questions. Patients pointed as extremely important being treated with dignity and respect and considered the awareness of the multidisciplinary team and the presentation of support programs and organizations as areas for improvement.</p><p><strong>Conclusion: </strong>The Portuguese version of the CQRA-PREM is acceptable and its content is valid in the perspective of patients with RA to assess the quality of care provided by the healthcare services.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"None"},"PeriodicalIF":0.0,"publicationDate":"2023-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41153019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Luís Cunha Miranda, Carlos Vaz, Elsa Vieira Sousa, Maria João Salvador, Maria José Santos
{"title":"Acta Reumatológica Portuguesa / ARP Rheumatology: 50 years at a glance - five decades by five Rheumatologists.","authors":"Luís Cunha Miranda, Carlos Vaz, Elsa Vieira Sousa, Maria João Salvador, Maria José Santos","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"2 3","pages":"182-187"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41239295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Tomás Fontes, Pedro Avila-Ribeiro, Joana Martins-Martinho, Tiago Tavares-Abreu, Ana Amado, Cristina Ponte
{"title":"Giant cell arteritis after radiation therapy: cause or coincidence?","authors":"Tomás Fontes, Pedro Avila-Ribeiro, Joana Martins-Martinho, Tiago Tavares-Abreu, Ana Amado, Cristina Ponte","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"2 3","pages":"279-281"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41239297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Maria Helena Lourenço, João Borralho, Inês Silva, João Alves, Rita Sampaio, Kamal Mansinho, Jaime Cunha Branco
Cytomegalovirus (CMV) infection is a common and typically benign disease in immunocompetent individuals. However, immunocompromised patients are at a greater risk of reactivation, leading to more severe outcomes. Patients with rheumatic diseases have a particularly high risk of opportunistic infections due to both the inherent immunosuppressive state conveyed by the disease itself and the use of potent immunosuppressant drugs, such as glucocorticoids, cyclophosphamide, and rituximab. Limited data are available regarding prophylactic or preemptive treatment of CMV infection in patients with rheumatic diseases. In this article the authors present two cases of rheumatic conditions complicated by CMV infection. The first case describes a patient with eosinophilic granulomatosis with polyangiitis, previously treated with glucocorticoids and cyclophosphamide, who developed CMV colitis with bowel perforation. The second case involves a woman with systemic lupus erythematosus who was diagnosed with CMV meningitis. Both cases reinforce the importance of establishing guidelines for surveillance and prophylaxis of CMV infection in these patients.
{"title":"Cytomegalovirus and rheumatic diseases: cases-based review.","authors":"Maria Helena Lourenço, João Borralho, Inês Silva, João Alves, Rita Sampaio, Kamal Mansinho, Jaime Cunha Branco","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Cytomegalovirus (CMV) infection is a common and typically benign disease in immunocompetent individuals. However, immunocompromised patients are at a greater risk of reactivation, leading to more severe outcomes. Patients with rheumatic diseases have a particularly high risk of opportunistic infections due to both the inherent immunosuppressive state conveyed by the disease itself and the use of potent immunosuppressant drugs, such as glucocorticoids, cyclophosphamide, and rituximab. Limited data are available regarding prophylactic or preemptive treatment of CMV infection in patients with rheumatic diseases. In this article the authors present two cases of rheumatic conditions complicated by CMV infection. The first case describes a patient with eosinophilic granulomatosis with polyangiitis, previously treated with glucocorticoids and cyclophosphamide, who developed CMV colitis with bowel perforation. The second case involves a woman with systemic lupus erythematosus who was diagnosed with CMV meningitis. Both cases reinforce the importance of establishing guidelines for surveillance and prophylaxis of CMV infection in these patients.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"2 3","pages":"269-274"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41239296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eduardo Dourado, Ana Teresa Melo, Raquel Campanilho-Marques, Matilde Bandeira, Patrícia Martins, Vanessa Fraga, José Luís Ferraro, André Saraiva, Marlene Sousa, Hugo Parente, Catarina Soares, Ana Margarida Correia, Diogo Esperança Almeida, Sara Paiva Dinis, Ana Sofia Pinto, Filipe Oliveira Pinheiro, Maria Seabra Rato, Tiago Beirão, Beatriz Samões, Bernardo Santos, Carolina Mazeda, Ana Teodósio Chícharo, Margarida Faria, Agna Neto, Maria Helena Lourenço, Luísa Brites, Marília Rodrigues, Joana Silva-Dinis, João Madruga Dias, Filipe C Araújo, Nádia Martins, Maura Couto, Ana Valido, Maria José Santos, Sofia Barreira, João Eurico Fonseca
Aims: To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort.
Methods: Reuma.pt is a web-based system with standardised patient files gathered in a registry. This was a multicentre open cohort study, including patients registered in Reuma.pt/myositis up to January 2022.
Results: Reuma.pt/myositis was designed to record all relevant data in clinical practice and includes disease-specific diagnosis and classification criteria, clinical manifestations, immunological data, and disease activity scores. Two hundred eighty patients were included, 71.4% female, 89.4% Caucasian, with a median age at diagnosis and disease duration of 48.9 (33.6-59.3) and 5.3 (3.0-9.8) years. Patients were classified as having definite (N=57/118, 48.3%), likely (N=23/118, 19.5%), or possible (N=2/118, 1.7%) IIM by 2017 EULAR/ACR criteria. The most common disease subtypes were dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), and myositis in overlap syndromes (N=41/280, 14.6%). The most common symptoms were proximal muscle weakness (N=180/215, 83.7%) and arthralgia (N=127/249, 52.9%), and the most common clinical signs were Gottron's sign (N=75/184, 40.8%) and heliotrope rash (N=101/252, 40.1%). Organ involvement included lung (N=78/230, 33.9%) and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent were anti-SSA/SSB (N=70/231, 30.3%), anti-Jo1 (N=56/236, 23.7%), and anti-Mi2 (N=31/212, 14.6%). Most patients had a myopathic pattern on electromyogram (N=101/138, 73.2%), muscle oedema in magnetic resonance (N=33/62, 53.2%), and high CK (N=154/200, 55.0%) and aldolase levels (N=74/135, 54.8%). Cancer was found in 11/127 patients (8.7%), most commonly breast cancer (N=3/11, 27.3%). Most patients with cancer-associated myositis had DM (N=8/11, 72.7%) and expressed MSA (N=6/11) and/or MAA (N=3/11). The most used drugs were glucocorticoids (N=201/280, 71.8%), methotrexate (N=117/280, 41.8%), hydroxychloroquine (N=87/280, 31.1%), azathioprine (N=85/280, 30.4%), and mycophenolate mofetil (N=56/280, 20.0%). At the last follow-up, there was a median MMT8 of 150 (142-150), modified DAS skin of 0 (0-1), global VAS of 10 (0-50) mm, and HAQ of 0.125 (0.000-1.125).
Conclusions: Reuma.pt/myositis adequately captures the main features of inflammatory myopathies' patients, depicting, in this first report, a heterogeneous population with frequent muscle, joint, skin, and lung involvements.
{"title":"The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register.","authors":"Eduardo Dourado, Ana Teresa Melo, Raquel Campanilho-Marques, Matilde Bandeira, Patrícia Martins, Vanessa Fraga, José Luís Ferraro, André Saraiva, Marlene Sousa, Hugo Parente, Catarina Soares, Ana Margarida Correia, Diogo Esperança Almeida, Sara Paiva Dinis, Ana Sofia Pinto, Filipe Oliveira Pinheiro, Maria Seabra Rato, Tiago Beirão, Beatriz Samões, Bernardo Santos, Carolina Mazeda, Ana Teodósio Chícharo, Margarida Faria, Agna Neto, Maria Helena Lourenço, Luísa Brites, Marília Rodrigues, Joana Silva-Dinis, João Madruga Dias, Filipe C Araújo, Nádia Martins, Maura Couto, Ana Valido, Maria José Santos, Sofia Barreira, João Eurico Fonseca","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Aims: </strong>To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort.</p><p><strong>Methods: </strong>Reuma.pt is a web-based system with standardised patient files gathered in a registry. This was a multicentre open cohort study, including patients registered in Reuma.pt/myositis up to January 2022.</p><p><strong>Results: </strong>Reuma.pt/myositis was designed to record all relevant data in clinical practice and includes disease-specific diagnosis and classification criteria, clinical manifestations, immunological data, and disease activity scores. Two hundred eighty patients were included, 71.4% female, 89.4% Caucasian, with a median age at diagnosis and disease duration of 48.9 (33.6-59.3) and 5.3 (3.0-9.8) years. Patients were classified as having definite (N=57/118, 48.3%), likely (N=23/118, 19.5%), or possible (N=2/118, 1.7%) IIM by 2017 EULAR/ACR criteria. The most common disease subtypes were dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), and myositis in overlap syndromes (N=41/280, 14.6%). The most common symptoms were proximal muscle weakness (N=180/215, 83.7%) and arthralgia (N=127/249, 52.9%), and the most common clinical signs were Gottron's sign (N=75/184, 40.8%) and heliotrope rash (N=101/252, 40.1%). Organ involvement included lung (N=78/230, 33.9%) and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent were anti-SSA/SSB (N=70/231, 30.3%), anti-Jo1 (N=56/236, 23.7%), and anti-Mi2 (N=31/212, 14.6%). Most patients had a myopathic pattern on electromyogram (N=101/138, 73.2%), muscle oedema in magnetic resonance (N=33/62, 53.2%), and high CK (N=154/200, 55.0%) and aldolase levels (N=74/135, 54.8%). Cancer was found in 11/127 patients (8.7%), most commonly breast cancer (N=3/11, 27.3%). Most patients with cancer-associated myositis had DM (N=8/11, 72.7%) and expressed MSA (N=6/11) and/or MAA (N=3/11). The most used drugs were glucocorticoids (N=201/280, 71.8%), methotrexate (N=117/280, 41.8%), hydroxychloroquine (N=87/280, 31.1%), azathioprine (N=85/280, 30.4%), and mycophenolate mofetil (N=56/280, 20.0%). At the last follow-up, there was a median MMT8 of 150 (142-150), modified DAS skin of 0 (0-1), global VAS of 10 (0-50) mm, and HAQ of 0.125 (0.000-1.125).</p><p><strong>Conclusions: </strong>Reuma.pt/myositis adequately captures the main features of inflammatory myopathies' patients, depicting, in this first report, a heterogeneous population with frequent muscle, joint, skin, and lung involvements.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"188-199"},"PeriodicalIF":0.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41157379","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}