FM-CUR-Artigos em Revistas Nacionais
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- Ten years of a systemic sclerosis clinic in a tertiary referral centre - insights and future directionsPublication . Martins, Patrícia; Dourado, Eduardo; Fonseca, João Eurico; Cordeiro, Inês; Romão, Vasco C.; Resende, CatarinaSystemic sclerosis (SSc) is an uncommon condition, with a wide range of manifestations, characterized by specific antibody production, vasculopathy and fibrosis of the skin and other internal organs. It is a complex disease, which is estimated to be rare in Portugal, although specific incidence data are missing. The aetiology of SSc remains unknown, but is likely to be multifactorial, involving genetic and environmental aspects. Its management is challenging and often requires a multidisciplinary approach. In 2011, we established a dedicated outpatient clinic for patients with SSc. Clinical data of every patient with a confirmed diagnosis of SSc is prospectively registered in Reuma.pt/SSc. In this manuscript, we aim to describe the general functioning of our SSc outpatient clinic, and to characterise the population of patients with SSc who are followed herein.
- The safety and persistence of intravenous iloprost in systemic sclerosisPublication . Martins, Patrícia; Dourado, Eduardo; Fonseca, João Eurico; Romão, Vasco C.; Resende, CatarinaIntroduction: Vasculopathy is a crucial feature of systemic sclerosis (SSc). It occurs in almost every patient with SSc, with Raynaud's phenomenon (RP) and digital ulcers (DU) having a great impact on the quality of patients' lives. Intravenous (IV) iloprost, a synthetic analogue of prostacyclin, is broadly used to treat RP and DU secondary to SSc. Currently, there is no standard protocol defined for the iloprost treatment of SSc-associated RP and DU, and, consequently, the management of this treatment is largely based on each centre's experience. Objective: The objective of this study is to evaluate the safety profile of a particular scheme of IV iloprost used in our centre as the standard treatment of SSc-related vascular complications. Methods: We retrospectively evaluated the clinical records of SSc patients, classified according to the 2013 European Alliance of Associations for Rheumatology (EULAR) criteria (31) with SSc-related DU and/or severe RP not responsive to CCB, receiving or who have received IV iloprost infusions from January 1st 2011 to March 31st 2021 Results: Within this time frame, 60 patients (n=44 for DU; n=16 for severe RP) were treated with a monthly 10-hour IV iloprost perfusion with a dosing regimen adapted to individual tolerance. Forty-nine of these 60 patients (81.7%) were on iloprost for more than one year. Within 12 months of therapy, 40 patients have healed the DUs (90.9%), with only 4 patients maintaining active DUs. A significant clinical improvement in RP at 12 months was observed in 87.5% (n=14/16) of SSc patients with severe RP. Eleven AE implying treatment dose/frequency adjustments or suspension were recorded (18.3% of patients): severe headache (n=5), hypotension (n=3), tachycardia (n=1), flushing (n=1) and generalised erythroderma (n=1). In all patients, the perfusion rate was reduced in the following treatment sessions with good tolerance, with the exception of the patient with the generalised erythroderma reaction, who suspended the perfusion and was later switched to bosentan. After a mean follow-up time of 6.9 (+/-) 4.0 years of treatment (range 0.06-22), 24 patients (40%) stopped the therapy, 14 (58.3%) of whom due to clinical improvement. The overall 5-, and 10-year survival rates of IV iloprost were 68.2% and 55.6%, respectively. Conclusion: SSc patients who received this flexible IV iloprost regimen achieved clinical improvement, reflected in the high persistence rate of the drug, with a good tolerability profile. In addition, most side effects were mild and easily managed.
- Investigação clínica da iniciativa do investigador em Portugal: identificação de problemas e propostas para melhoriaPublication . Ferreira, João Pedro; Leite-Moreira, Adelino; Da Costa-Pereira, Altamiro; Soares, António José; Robalo-Cordeiro, Carlos; Jerónimo, Cármen; Gavina, Cristina; Pinto, Fausto J.; Schmitt, Fernando; Saraiva, Francisca; Vasques-Nóvoa, Francisco; Canhao, Helena; Cyrne-Carvalho, Henrique; Palmeirim, Isabel; Pimenta, Joana; Fonseca, João Eurico; Firmino-Machado, João; Correia Pinto, Jorge; Gonçalves, Lino; Castelo Branco, Miguel; Sousa, Nuno; Fontes de Carvalho, Ricardo; Machado Luciano, Teresa; Gil Oliveira, Tiago; Resende Oliveira, CatarinaPortugal tem um problema crónico de subfinanciamento da investigação científica. Apesar do investimento nacional ter vindo a aumentar nos últimos anos, este ainda é manifestamente insuficiente. A Fundação para a Ciência e Tecnologia (FCT) financia anualmente projetos de investigação em todas as áreas científicas, limitando o valor máximo a €250 000 por projeto. Todavia, a área científica na qual a investigação clínica está incluída – “Clinical Medicine, Immunology and Infection” – financia maioritariamente projetos de matriz translacional e não tem por norma apoiar ensaios clínicos (EC) ou estudos multicêntricos epidemiológicos e de coorte da iniciativa do investigador, área onde o financiamento é hoje em dia praticamente inexistente, mesmo para estudos clínicos exploratórios e proof-of-concept. É importante salientar que o potencial de retorno do investimento em ensaios clínicos é enorme. Em Portugal, por cada €1 investido em ensaios clínicos obtém-se em média um retorno de €1,99, isto é, um retorno de praticamente 200%.
- Management of infections in rheumatic patients receiving biological therapies. the Portuguese Society of Rheumatology recommendationsPublication . Teixeira, Lídia; Fonseca, Ana Rita; Eugénio, Gisela; Rodrigues, Marília; Khmelinskii, Nikita; Fernandes, Sílvia; Serra, Sofia; Costa, Tiago; Santos, MariaIntroduction: Infections are a major cause of morbidity and mortality in systemic inflammatory rheumatic diseases and the management of infectious complications in patients under biological therapies deserves particular attention. Objective: Develop evidence-based recommendations for the management of infections in rheumatic patients receiving biological therapies. Methods: A search in PubMed (until 10 November 2014) and EMBASE (until 20 December 2014) databases was performed. Patients with systemic inflammatory rheumatic diseases treated with approved biologics in whom infections occurred were included. Search results were submitted to title and abstract selection, followed by detailed review of suitable studies. Information regarding presentation of the infectious complication, its diagnosis, treatment, and outcome, as well as maintenance or discontinuation of the biological agent was extracted and subsequently pooled according to the type of infection considered. Results of literature review were presented and critically reviewed in a dedicated meeting by a multidisciplinary panel. Recommendations were then formulated using the Delphi method. Finally, the level of agreement among rheumatologists was voted using an online survey. Results: Fifteen recommendations were issued. Nine general recommendations concerned the assessment of infectious risk before and while on biologics, the procedures in case of suspected infection and the management of biologics during infectious complications. Six specific recommendations were developed for respiratory, urinary, gastrointestinal, skin, osteoarticular and disseminated infections. Conclusion: These fifteen recommendations are intended to help rheumatologists in the management of infections in patients on biological therapy. They integrate an extensive literature review, expert opinion and inputs from Portuguese rheumatologists.
- Portuguese recommendations for the use of ultrasound in rheumatologyPublication . Polido Pereira, Joaquim; Serra, Sara; Teixeira, Filipa; Ponte, Cristina; Cerqueira, Marcos; Cruz, Margarida; Araújo, Filipe; Barros, Rita; Costa, Tiago; Santos-Faria, Daniela; Lopes, Carina; Madruga-Dias, João; Oliveira, Margarida; Teixeira, Rui Lourenço; Vilar, António; Falcão, Sandra; Saraiva, Fernando; Figueiredo, GuilhermeIntroduction: Ultrasound (US) is a relatively cheap, easily available and reliable method to improve the care of rheumatic patients. However, its use in rheumatology practice is very heterogeneous and needs to be standardized. Objectives: To develop recommendations for the use of US in rheumatic diseases endorsed by the Portuguese Society of Rheumatology. Methods: A systematic literature review of the available recommendations on the use of ultrasound in rheumatic diseases was performed and presented in a Portuguese Society of Rheumatology meeting to a subgroup of rheumatologists and rheumatology trainees with special interest in the subject. The most important topics to be addressed were selected and assigned to subgroups for literature review and draft recommendations. Following an iterative process of consensus, the final recommendations were developed, and their level of agreement voted anonymously online. A recommendation was approved when the average level of agreement was ≥ 7.5 in a 10-point Likert scale. Results: Fourteen recommendations were produced regarding nine rheumatology topics: rheumatoid arthritis, spondyloarthritis, connective tissue diseases, polymyalgia rheumatica, vasculitis, crystal-deposition diseases, soft tissue rheumatism, osteoarthritis and ultrasound-guided procedures. Conclusion: We developed an up-to-date guidance in the form of recommendations for the use of US in nine different areas of rheumatology. As ultrasound is an important imaging modality with increasing use in the rheumatology setting, and there are frequent technological advances in the ultrasound machines and probes, in parallel with continuous associated research, these recommendations should be regularly updated.
- Osteoblasts and bone formationPublication . Caetano-Lopes, Joana; Canhao, Helena; Fonseca, João EuricoBone is constantly being remodelled in a dynamic process where osteoblasts are responsible for bone formation and osteoclasts for its resorption. Osteoblasts are specialized mesenchymal cells that undergo a process of maturation where genes like core-binding factor α1 (Cbfa1) and osterix (Osx) play a very important role. Moreover, it was found recently that the Wnt/β-catenin pathway plays a part on osteoblast differentiation and proliferation. In fact, mutations on some of the proteins involved in this pathway, like the low-density lipoprotein receptor related protein 5/6 (LRP5/6), lead to bone diseases. Osteoblasts have also a role in the regulation of bone resorption through receptor activator of nuclear factor-κB (RANK) ligand (RANKL), that links to its receptor, RANK, on the surface of pre-osteoclast cells, inducing their differentiation and fusion. On the other hand, osteoblasts secrete a soluble decoy receptor (osteoprotegerin, OPG) that blocks RANK/RANKL interaction by binding to RANKL and, thus, prevents osteoclast differentiation and activation. Therefore, the balance between RANKL and OPG determines the formation and activity of osteoclasts. Another factor that influences bone mass is leptin, a hormone produced by adipocytes that have a dual effect. It can act through the central nervous system and diminish osteoblasts activity, or can have an osteogenic effect by binding directly to its receptors on the surface of osteoblast cells.
- Posterior reversible encephalopathy syndrome and digital gangrene in a patient with granulomatosis with polyangiitis : a rare case reportPublication . Martins, Patrícia; Valido, Ana; Melo, Ana Teresa; Santareno, Sofia; Sousa, Rita; Inácio, João R.; Oliveira-Ramos, Filipa; Ponte, Cristina; Romeu, JoséGranulomatosis with Polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis(AAV), classically associated with the presence of proteinase 3 (PR3)-ANCA. GPA has a wide range of clinical manifestations, with multiorgan involvement, particularly upper and lower respiratory tract, and kidneys. One of itsrarest manifestationsis digital ischemia or gangrene that accounts for <1% of cases. Posterior reversible posterior leukoencephalopathy syndrome (PRES) is a syndrome characterized by headache, visual changes, confusion, and seizures, accompanied by distinctive neuroimaging features, which are usually reversible. Rare cases of its association with vasculitis, including GPA, have been described. Others conditions such as end-stage renal disease, immunosuppressive drug use and eclampsia are additional risk factors for the development of PRES.
- Loss to follow-up in registries of rheumatic patients treated with biologics : a potential information bias in assessing pharmacovigilance and efficacy outcomesPublication . Valido, Ana; Silva-Dinis, Joana; Machado, Ana Rita; Gonçalves, Maria João; Romão, Vasco C.; Saavedra, Maria João; Fonseca, João EuricoBackground: The information associated with loss to follow-up (LFU) patients may affect real-world data evaluation of the use of biologics that is not being adequately captured in registries. Methods: We identified all patients(Pts) treated with biologics in our center who had no visits registered for more than 6 months, in the Rheumatic Diseases Portuguese Register, Reuma.pt. We retrieved baseline information from Reuma.pt and from the hospital electronic clinical record. We then performed a telephonic interview to characterize the reasons for LFU at our day care unit. For Pts unable to be contacted by telephone a letter of invitation to an appointment at the hospital was sent. Results: From a total of 794 Ptsregistered in Reuma.pt at our center with active biologic therapy 227 did not have any information registered in the last 6 months. Of this, 36 Pts were on biologic therapy prescribed by other departments and maintained follow-up in these departments. 102 Pts had suspended biologic administration by medical indication and this information was registered in the hospital electronic clinical records but not updated in Reuma.pt. For 89 Pts no information could be retrieved from either the hospital electronic clinical record or Reuma.pt and we classified these Pts as true LFU. 26 of these LFU Pts were being followed up in another Rheumatology center. 26 of the LFU Pts died. 11 Pts had an adverse effect. 4 Pts of the LFU were considering to be in remission. We were not able to contact 15 of the LFU pts. Conclusion: Identifying LFU Pts and clarifying the reason for the loss of data in a register contributes to a better knowledge on strategies to discontinue biologics in stable pts, to a better pharmacovigilance of adverse effects and to more efficiency in data capture by registries. Due to data protection reasons it was impossible to have access to the Pts's death certificates.
- How did COVID-19 pandemic changed the Portuguese rheumatology?Publication . Vieira De Sousa, Elsa Cristina; Fonseca, João Eurico; Santos, MariaWe have been facing a tremendous challenge motivated by the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic. With a few days of warning Rheumatologists had to rethink their established conventional management of patients based on presential appointments, to ensure ongoing adequate care to rheumatic patients. The first operating principles were to maintain rheumatic diseasesstability, treatment access and protection from the potentialrisk of infection by SARS-CoV-2.
- The GO-DACT protocol : a multicentre, randomized, double-blind, parallel-group study to compare the efficacy of golimumab in combination with methotrexate (MTX) versus MTX monotherapyPublication . Vieira de Sousa, Elsa Cristina; Canhao, Helena; Alves, Pedro; Rodrigues, Ana Maria; Teixeira, Filipa; Tavares-Costa, José; Bernardo, Alexandra; Pimenta, Sofia; Pimentel-Santos, Fernando; Gomes, João L; Aguiar, Renata; Videira, Taciana; Pinto, Patrícia; Catita, Cristina; Santos, Helena; Borges, Joana; Sequeira, Graça; Ribeiro, Célia; Teixeira, Lídia; Ávila-Ribeiro, Pedro; Martins, Fernando M; Ribeiro, Ruy M.; Fonseca, João EuricoThe GO-DACT is an investigator-initiated, national, multicentric randomized placebo-controlled double-blinded trial, that assesses dactylitis as primary endpoint. Psoriatic arthritis patients naïve to methotrexate and biologic disease modifying anti-rheumatic drugs, with at least one active dactylitis, were assigned to golimumab in combination with methotrexate or placebo in combination with methotrexate, for 24 weeks. Both clinical (dactylitis severity score and the Leeds dactylitis index) and imaging (high resolution magnetic resonance imaging), among others, were assessed as outcomes. The main objective of GO-DACT is to provide evidence to improve the treatment algorithm and care of psoriatic arthritis patients with active dactylitis. In this manuscript we describe the GO-DACT protocol and general concepts of the methodology of this trial.