Browsing by Author "Fonseca, JE"
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- 2016 Update of the Portuguese Recommendations for the Use of Biological Therapies in Children and Adolescents with Juvenile Idiopathic ArthritisPublication . Santos, MJ; Conde, M; Mourão, AF; Ramos, FO; Cabral, M; Brito, I; Ramos, MP; Marques, RC; Gomes, SM; Guedes, M; Gonçalves, MJ; Estanqueiro, P; Zilhão, C; Rodrigues, M; Henriques, C; Salgado, M; Canhão, H; Fonseca, JE; Gomes, JMTo provide evidence-based guidance for the rational and safe prescription of biological therapies in children and adolescents with juvenile idiopathic arthritis (JIAs) considering the latest available evidence and the new licensed biologics. Rheumatologists and Pediatricians with expertise in Pediatric Rheumatology updated the recommendations endorsed by the Portuguese Society of Rheumatology and the Portuguese Society of Pediatrics based on published evidence and expert opinion. The level of agreement with final propositions was voted using an online survey. RESULTS: In total, 20 recommendations to guide the use of biological therapy in children and adolescents with JIAs are issued, comprising 4 general principles and 16 specific recommendations. A consensus was achieved regarding the eligibility and response criteria, maintenance of biological therapy, and procedures in case of non-response, for each JIA category. Specific recommendations concerning safety procedures were also updated. These recommendations take into account the specificities of each JIA category and are intended to continuously improve the management of JIA patients.
- Association of Body Mass Index with Juvenile Idiopathic Arthritis Disease Activity: a Portuguese and Brazilian Collaborative AnalysisPublication . Neto, A; Mourão, AF; Oliveira-Ramos, F; Campanilho-Marques, R; Estanqueiro, P; Salgado, M; Guedes, M; Piotto, D; Emi Aikawa, N; Melo Gomes, J; Cabral, M; Conde, M; Figueira, R; Santos, MJ; Fonseca, JE; Terreri, MT; Canhão, HObjective: To investigate the relationship between body mass index (BMI) and disease activity in patients with Juvenile Idiopathic Arthritis (JIA). Methods: Patients with JIA, aged ≤18 years, registered at the Rheumatic Diseases Portuguese Register (Reuma.pt) in Portugal and Brazil were included. Ageand sex-specific BMI percentiles were calculated based on WHO growth standard charts and categorized into underweight (P<3), normal weight (3≤P≤85), overweight (8597). Disease activity was assessed by Juvenile Arthritis Disease Activity Score (JADAS-27). Uni- and multivariable analyses were performed. Results: A total of 275 patients were included. The prevalence of underweight, normal weight, overweight and obesity was 6.9%, 67.3%, 15.3% and 10.5%, respectively. Underweight patients had significantly higher number of active joints (p<0.001), patient’s/parent’s global assessment of disease activity (PGA) (p=0.020), physician’s global assessment of disease activity (PhGA) (p<0.001), erythrocyte sedimentation rate (ESR) (p=0.032) and overall higher JADAS-27 (p<0.001), compared to patients with normal weight, overweight and obesity. In the multivariable regression, normal weight (B=-9.43, p<0.01), overweight (B=-9.30, p=0.01) and obesity (B=-9.12, p=0.01) were significantly associated with lower disease activity compared to underweight, when adjusted for age, gender, country, ethnicity, JIA category and therapies used. The diagnosis of RF- (B=3.65, p=0.006) or RF+ polyarticular JIA (B=5.29, p=0.024), the absence of DMARD therapy (B=5.54, p<0.001) and the use of oral GC (B=4.98, p=0.002) were also associated with higher JADAS-27. Conclusion: We found an independent association between underweight and higher disease activity in patients with JIA. Further studies are needed to understand the underlying mechanisms of this association.
- Bone Histomorphometry RevisitedPublication . Vidal, B; Pinto, A; Galvão, MJ; Santos, AR; Rodrigues, A; Cascão, R; Abdulghani, S; Caetano-Lopes, J; Ferreira, A; Fonseca, JE; Canhão, HBone histomorphometry is defined as a quantitative evaluation of bone micro architecture, remodelling and metabolism. Bone metabolic assessment is based on a dynamic process, which provides data on bone matrix formation rate by incorporating a tetracycline compound. In the static evaluation, samples are stained and a semi-automatic technique is applied in order to obtain bone microarchitectural parameters such as trabecular area, perimeter and width. These parameters are in 2D, but they can be extrapolated into 3D, applying a stereological formula. Histomorphometry can be applied to different areas; however, in recent decades it has been a relevant tool in monitoring the effect of drug administration in bone. The main challenge for the future will be the development of noninvasive methods that can give similar information. In the herein review paper we will discuss the general principles and main applications of bone histomorphometry.
- Idiopathic Inflammatory Myopathies: State of the Art on Clinical Practice Guidelines [corrected]Publication . Meyer, A; Scirè, CA; Talarico, R; Alexander, T; Amoura, Z; Avcin, T; Barsotti, S; Beretta, L; Blagojevic, J; Burmester, G; Cavazzana, I; Cherrin, P; Damian, L; Doria, A; Fonseca, JE; Furini, F; Galetti, I; Houssiau, F; Krieg, T; Maddalena, L; Launay, D; Campanilho-Marques, R; Martin, T; Matucci-Cerinic, M; Moinzadeh, P; Montecucco, C; Moraes-Fontes, MF; Mouthon, L; Neri, R; Paolino, S; Piette, Y; Rednic, S; Tamirou, F; Tincani, A; Toplak, N; Bombardieri, S; Hachulla, E; Mueller-Ladner, U; Schneider, M; Smith, V; Vieira, A; Cutolo, M; Mosca, M; Cavagna, LIdiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
- Mixed connective tissue disease: state of the art on clinical practice guidelinesPublication . Chaigne, B; Scirè, CA; Talarico, R; Alexander, T; Amoura, Z; Avcin, T; Beretta, L; Doria, A; Guffroy, A; Guimarães, V; Hachulla, É; Krieg, T; Launay, D; Lepri, G; Moinzadeh, P; Müller-Ladner, U; Rednic, S; Rodrigues, A; Tas, SW; van Vollenhoven, RF; Vieira, A; Bombardieri, S; Fonseca, JE; Galetti, I; Schneider, M; Smith, V; Cutolo, M; Mosca, M; Fischer-Betz, RMixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients.
- Portuguese Recommendations for the Use of Biological and Targeted Synthetic Disease-Modifying Antirheumatic Drugs in Patients with Rheumatoid Arthritis - 2020 UpdatePublication . Fernandes, BM; Guimarães, F; Almeida, DE; Neto, A; Tavares-Costa, J; Roxo Ribeiro, A; Quintal, A; Pereira, JP; Silva, L; Nóvoa, TS; Faustino, A; Vaz, C; Khmelinskii, N; Samões, B; Dourado, E; Silva, JL; Barcelos, A; Mariz, E; Guerra, M; Santos, MJ; Silvério-António, M; Teixeira, RL; Romão, VC; Santos, H; Santos-Faria, D; Azevedo, S; Rodrigues, A; Dias, JM; Lopes, C; Pinto, P; Couto, M; Miranda, LC; Bernardo, A; Cruz, M; Teixeira, F; Mourão, AF; Neto, A; Teixeira, V; Cordeiro, A; Barreira, S; Inês, LS; Capela, S; Sepriano, A; Canhão, H; Fonseca, JE; Duarte, C; Bernardes, MObjective: To update the recommendations for the treatment of rheumatoid arthritis (RA) with biological and targeted synthetic disease-modifying antirheumatic drugs (bDMARDs and tsDMARDs), endorsed by the Portuguese Society of Rheumatology (SPR). Methods: These treatment recommendations were formulated by Portuguese rheumatologists taking into account previous recommendations, new literature evidence and consensus opinion. At a national meeting, in a virtual format, three of the ten previous recommendations were re-addressed and discussed after a more focused literature review. A first draft of the updated recommendations was elaborated by a team of SPR rheumatologists from the SPR rheumatoid arthritis study group, GEAR. The resulting document circulated among all SPR rheumatologists for discussion and input. The level of agreement with each of all the recommendations was anonymously voted online by all SPR rheumatologists. Results: These recommendations cover general aspects such as shared decision, treatment objectives, systematic assessment of disease activity and burden and its registry in Reuma.pt. Consensus was also achieved regarding specific aspects such as initiation of bDMARDs and tsDMARDs, assessment of treatment response, switching and definition of persistent remission. Conclusion: These recommendations may be used for guidance of treatment with bDMARDs and tsDMARDs in patients with RA. As more evidence becomes available and more therapies are licensed, these recommendations will be updated.
- The 2021 Portuguese Society of Ophthalmology Joint Guidelines with Paediatric Rheumatology on the Screening, Monitoring and Medical Treatment of Juvenile Idiopathic Arthritis-Associated UveitisPublication . Leal, I; Miranda, V; Fonseca, C; Barbosa-Breda, J; Cordeiro Sousa, D; Mesquita-Marques, P; Araújo, J; Silva, MI; Pedrosa, AC; Palmares, J; Furtado, MJ; Macedo, M; Lages, V; Fonseca, S; Gonçalves, R; Ruão, M; Gomes Rodrigues, F; Ribeiro, M; Proença, R; Almeida, M; Liverani, M; Morais Pina, S; Bernardo, M; Nogueira, V; Guerra Pinto, R; Pinto Ferreira, F; Pinto Proença, R; Domingues, I; Guedes, M; Cordeiro, M; Fragata, F; Berens, O; Gregório, T; Brito, I; Oliveira-Ramos, F; Fonseca, JE; Figueira, LAim: To develop the first Ophthalmology joint guidelines with Paediatric Rheumatology with recommendations on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis (JIA-U), endorsed by the Portuguese Society of Ophthalmology (SPO). Methods: A systematic literature review was conducted to include publications up to July 14th 2020, with no language restrictions, in order to include all the international position papers/guidelines concerning the medical management of JIA-U and randomised clinical trials assessing the efficacy and safety of medical treatment in this field. We searched through MEDLINE (PubMed), Scopus, Web of Science and Cochrane Library. The Delphi modified technique to generate consensus was used. Preliminary evidence statements were subject to an anonymous agreement assessment and discussion process using an online survey, followed by further discussion and update at a national meeting. A draft of the manuscript with all recommendations was then circulated among all participants and suggestions were incorporated. The final version was again circulated before publication. Results: Twenty-six recommendations were developed focusing on the following topics: general management (3), screening and follow-up of uveitis (4), treatment (17) and health education in JIA-U among patients and families (2). Conclusion: These guidelines were designed to support the shared medical management of patients with JIA-U and emphasize the need for a multidisciplinary approach between Ophthalmology and Paediatric Rheumatology regarding the comprehensive care of JIA-U. We acknowledge that updating these recommendations will be warranted in the future, as more evidence becomes available.
- 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-Sousa, E; Canhão, H; Alves, P; Rodrigues, AM; Teixeira, F; Tavares-Costa, J; Bernardo, A; Pimenta, S; Pimentel-Santos, F; Gomes, JL; Aguiar, R; Videira, T; Pinto, P; Catita, C; Santos, H; Borges, J; Sequeira, G; Ribeiro, C; Teixeira, L; Ávila-Ribeiro, P; Martins, F; Ribeiro, R; Fonseca, JEThe 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.
- Undifferentiated connective tissue disease: state of the art on clinical practice guidelinesPublication . Antunes, M; Scirè, CA; Talarico, R; Alexander, T; Avcin, T; Belocchi, C; Doria, A; Franceschini, F; Galetti, I; Govoni, M; Hachulla, E; Launay, D; Lepri, G; Macieira, C; Matucci-Cerinic, M; Montecucco, CM; Moraes-Fontes, MF; Mouthon, L; Paolino, S; Ramoni, V; Tani, C; Tas, SW; Tincani, A; Van Vollenhoven, R; Zen, M; Fonseca, J; Bombardieri, S; Fonseca, JE; Schneider, M; Smith, V; Cutolo, M; Mosca, M; Beretta, LThe term 'undifferentiated connective tissue disease' (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.