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The effects of physical exercise on axial spondyloarthritis - a systematic review. 体育锻炼对轴性脊椎关节炎的影响——一项系统综述。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-08-27
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。
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引用次数: 0
Butterfly Vertebra: the mimicker of vertebral fragility fractures. 蝴蝶脊椎:脊椎脆性骨折的模仿者。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-08-05
Ana Bento da Silva, Maria Helena Lourenço, Maria João Gonçalves, Jaime C Branco, Manuela Costa
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引用次数: 0
Immune-mediated skin lesions related to biological disease-modifying antirheumatic drugs: a 22-year experience of a tertiary center. 免疫介导的皮肤损伤与生物疾病修饰抗风湿病药物相关:一项在三级中心22年的经验。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-19
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.

引言:免疫介导的皮肤损伤(IMSL)可能会导致治疗中断。尽管这些病变以前很少被描述,但真正的发病率尚不清楚。目的:在一个大型慢性炎性风湿性疾病患者队列中,探讨与bDMARD相关的IMSL的累积发病率、管理和结果。探讨IMSL发展的可能关联和风险因素。方法:对接受至少一种bDMARD治疗至少6个月的类风湿性关节炎(RA)、脊柱炎(SpA)和银屑病关节炎(PsA)患者进行回顾性单中心研究。收集与bDMARD特征和结果相关的IMSL。结果:共纳入989例RA、SpA和PsA患者。27名患者(2.7%)表现出可能与bMARD相关的IMSL,其中银屑病是最常见的IMSL(n=12,44.4%),其次是药物诱导的红斑狼疮(n=6)、斑秃(n=3)和白细胞碎屑血管炎(n=2)。IMSL导致27名患者中有18名(66.7%)退出bDMARD。患有IMSL的患者在诊断时年龄较小(p=0.038),病程较长(p=0.018),bDMARD治疗持续时间较长(p=0.008),既往bDMARD数量较高(p<0.001)。在IMSL患者组中,接受阿达木单抗治疗的患者比例显著较高(p<0.001)。在多变量回归模型中,既往bDMARD(OR 2.13,95%CI 1.47-3.10,p<0.001,)和阿达木单抗(OR 4.60,95%CI 1.96-10.80,p<001)的数量是IMSL发展的统计学显著预测因素。结论:在我们的研究中,与bDMARD相关的IMSL的累计发病率估计为2.7%。诊断年龄越小、疾病持续时间越长、bDMARD治疗持续时间越久、既往bDMARD次数越多以及阿达木单抗治疗与IMSL发展风险增加独立相关。
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引用次数: 0
Content validity of a patient-reported experience measure (CQRA-PREM) for patients with rheumatoid arthritis in Portugal. 葡萄牙类风湿性关节炎患者报告经验测量(CQRA-PREM)的内容有效性。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-15
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.

背景:评估类风湿性关节炎(RA)患者的认知对他们的健康结果和整体护理体验有积极影响。制定了《类风湿性关节炎患者报告经验测量质量调试》(CQRA-PREM),旨在向卫生专业团队提供RA患者的感知和反馈。该PREM已在英国和荷兰得到验证和使用。在葡萄牙,没有经过验证的PREM来评估RA患者的经验。目的:为葡萄牙人翻译、文化改编和验证CQRA-PEM的内容。方法:采用焦点小组进行定性研究,评价CQRA-PREM内容的有效性。两名研究人员首先将CQRA-PREM翻译成葡萄牙语,并对其进行文化改编,经过后译,一个专家小组就CQRA-Prum的葡萄牙语初步版本达成一致。RA患者是从三级大学医院中心的风湿病中心招募来参加焦点小组会议的。在焦点小组之前,他们填写了CQRA-PEM的初步版本,包括7个领域和24个项目(Likert量表1-5)。结果:12名参与者(中位54岁(45-58岁);92%的女性)被纳入两个焦点组。问卷的所有领域均具有3或以上的中位数。出现了七大主题和六个子主题。与会者认为调查表非常清楚和简单,并提出了充分的问题。患者指出,获得尊严和尊重的治疗极其重要,并认为多学科团队的意识以及支持计划和组织的介绍是需要改进的领域。结论:葡萄牙版的CQRA-PEM是可接受的,其内容从RA患者的角度来看是有效的,可以评估医疗服务提供的护理质量。
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引用次数: 0
Juvenile presentation of a pigmented villonodular synovitis of the wrist. 手腕色素沉着绒毛结节性滑膜炎的幼年表现。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-11
Margarida Correia, Carla Campinho Ferreira, Emanuel Costa, Roberto Silva, Ana Roxo Ribeiro, Marcos Cerqueira
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引用次数: 0
Acta Reumatológica Portuguesa / ARP Rheumatology: 50 years at a glance - five decades by five Rheumatologists. 葡萄牙风湿病杂志:50年一览——五位风湿病学家的50年。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-01
Luís Cunha Miranda, Carlos Vaz, Elsa Vieira Sousa, Maria João Salvador, Maria José Santos
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引用次数: 0
Giant cell arteritis after radiation therapy: cause or coincidence? 放射治疗后的巨细胞动脉炎:病因还是巧合?
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-01
Tomás Fontes, Pedro Avila-Ribeiro, Joana Martins-Martinho, Tiago Tavares-Abreu, Ana Amado, Cristina Ponte
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引用次数: 0
Cytomegalovirus and rheumatic diseases: cases-based review. 巨细胞病毒与风湿性疾病:基于病例的综述。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-01
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.

巨细胞病毒(CMV)感染是一种常见且典型的良性疾病,发生在具有免疫功能的个体中。然而,免疫功能受损的患者再次激活的风险更大,导致更严重的后果。风湿性疾病患者机会性感染的风险特别高,这是由于疾病本身所传达的固有免疫抑制状态以及使用强效免疫抑制药物,如糖皮质激素、环磷酰胺和利妥昔单抗。关于风湿性疾病患者CMV感染的预防性或先发制人治疗的数据有限。本文报告两例风湿性疾病并发巨细胞病毒感染的病例。第一个病例描述了一名患有嗜酸性肉芽肿伴多血管炎的患者,之前曾接受糖皮质激素和环磷酰胺治疗,后来发展为CMV结肠炎伴肠穿孔。第二个病例涉及一名患有系统性红斑狼疮的女性,她被诊断为CMV脑膜炎。这两个病例都强调了建立监测和预防这些患者CMV感染指南的重要性。
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引用次数: 0
The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register. 葡萄牙风湿病注册中心的特发性炎症性肌病模块。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-01 Epub Date: 2023-05-30
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.

目的:描述葡萄牙风湿病登记册(Reuma.pt/肌炎)的特发性炎症性肌病(IIM)模块及其队列中的患者。方法:Reuma.pt是一个基于网络的系统,在注册表中收集标准化的患者文件。这是一项多中心开放队列研究,包括截至2022年1月在Reuma.pt/肌炎中注册的患者。结果:Reuma.pt-肌炎旨在记录临床实践中的所有相关数据,包括疾病特异性诊断和分类标准、临床表现、免疫数据和疾病活动评分。280名患者包括在内,71.4%为女性,89.4%为白人,诊断时的中位年龄和疾病持续时间分别为48.9岁(33.6-59.3)和5.3岁(3.0-9.8)。根据2017年EULAR/ACR标准,患者被分为明确(N=57/118,48.3%)、可能(N=23/118,19.5%)或可能(N=2/118,1.7%)IIM。最常见的疾病亚型是皮肌炎(DM,N=122/280,43.6%)、多发性肌炎(N=59/280,21.1%)和重叠综合征肌炎(N=41/280,14.6%)。最常见的症状是近端肌无力(N=180/215,83.7%)和关节痛(N=127/249,52.9%),最常见的临床症状为Gottron征(N=75/184,40.8%)和日光疹(N=101/252,40.1%)。器官受累包括肺部(N=78/230,33.9%)和心脏(N=11/229,4.8%)。大多数患者表达肌炎特异性抗体(MSA,N=158/242,65.3%)或肌炎相关抗体(MAA,112/242,46.3%)。最常见的是抗SSA/SSB(N=70/231,30.3%)、抗Jo1(N=56/236,23.7%)和抗Mi2(N=31/212,14.6%)。大多数患者的肌电图模式为肌肉病变(N=101/138,73.2%),磁共振肌肉水肿(N=33/62,53.2%),高CK(N=154/200,55.0%)和醛缩酶水平(N=74/135,54.8%),最常见的是癌症(N=3/11,27.3%)。大多数癌症相关肌炎患者患有DM(N=8/11,72.7%),并表达MSA(N=6/11)和/或MAA(N=3/11)。最常用的药物是糖皮质激素(N=201/280,71.8%)、甲氨蝶呤(N=117/280,41.8%)、羟氯喹(N=87/280,31.1%)、硫唑嘌呤(N=85/280,30.4%)和霉酚酸酯(N=56/280,20.0%)。在最后一次随访中,MMT8中位数为150(142-150),改良DAS皮肤为0(0-1),整体VAS为10(0-50)mm,HAQ为0.125(0.000-1.125)。结论:Reuma.pt/肌炎充分反映了炎症性肌病患者的主要特征,在本首次报告中,描述了一个频繁涉及肌肉、关节、皮肤和肺部的异质性人群。
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引用次数: 0
Portuguese referrals to pediatric rheumatology - multicentric study. 葡萄牙儿童风湿病转诊-多中心研究。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-07-01
Hugo Parente, Francisca Guimarães, Maria Helena Lourenço, Margarida Santos Faria, Rita Pinheiro Torres, Sónia Carvalho, Vanessa Fraga, Daniela Peixoto
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引用次数: 0
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ARP Rheumatology
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