Browsing by Author "Vieira, A"
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- Antiphospholipid Syndrome: State of the Art on Clinical Practice GuidelinesPublication . Limper, M; Scirè, CA; Talarico, R; Amoura, Z; Avcin, T; Basile, M; Burmester, G; Carli, L; Cervera, R; Costedoat-Chalumeau, N; Doria, A; Dörner, T; Eurico Fonseca, J; Galetti, I; Hachulla, E; Launay, D; Lourenco, F; Macieira, C; Meroni, P; Montecucco, CM; Moraes-Fontes, MF; Mouthon, L; Nalli, C; Ramoni, V; Tektonidou, M; van Laar, JM; Bombardieri, S; Schneider, M; Smith, V; Vieira, A; Cutolo, M; Mosca, M; Tincani, AAntiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies. This review aims to identify existing clinical practice guidelines (CPG) as part of the ERN ReCONNET project, aimed at evaluating existing CPGs or recommendations in rare and complex diseases. Seventeen papers providing important data were identified; however, the literature search highlighted the scarceness of reliable clinical data to develop CPGs. With no formal clinical guidelines in place, diagnosis and treatment of APS is largely based on consensus and expert opinion. Patients' unmet need refers to the understanding of the disease and its clinical picture and implications, the need of education for patients, family members and healthcare providers, as well as to the development of monitoring pathways involving multiple healthcare providers.
- Continuous Infusion of Piperacillin/Tazobactam in Septic Critically Ill Patients - a Multicenter Propensity Matched AnalysisPublication . Gonçalves-Pereira, J; Serra Oliveira, B; Janeiro, S; Estilita, J; Monteiro, C; Salgueiro, A; Vieira, A; Gouveia, J; Paulino, C; Bento, L; Póvoa, PThe clinical efficacy of continuous infusion of piperacillin/tazobactam in critically ill patients with microbiologically documented infections is currently unknown. We conducted a retrospective multicenter cohort study in 7 Portuguese intensive care units (ICU). We included 569 critically ill adult patients with a documented infection and treated with piperacillin/tazobactam admitted to one of the participating ICU between 2006 and 2010. We successfully matched 173 pairs of patients according to whether they received continuous or conventional intermittent dosing of piperacillin/tazobactam, using a propensity score to adjust for confounding variables. The majority of patients received 16g/day of piperacillin plus 2g/day of tazobactam. The 28-day mortality rate was 28.3% in both groups (p = 1.0). The ICU and in-hospital mortality were also similar either in those receiving continuous infusion or intermittent dosing (23.7% vs. 20.2%, p = 0.512 and 41.6% vs. 40.5%, p = 0.913, respectively). In the subgroup of patients with a Simplified Acute Physiology Score (SAPS) II>42, the 28-day mortality rate was lower in the continuous infusion group (31.4% vs. 35.2%) although not reaching significance (p = 0.66). We concluded that the clinical efficacy of piperacillin/tazobactam in this heterogeneous group of critically ill patients infected with susceptible bacteria was independent of its mode of administration, either continuous infusion or intermittent dosing.
- Doença de Crohn - Ulcerações Genitais MúltiplasPublication . Cunha, D; Rodrigues, A; Afonso, A; Vieira, A; Freitas, J; Cardoso, JAs ulcerações genitais são frequentemente motivo de envio à consulta de Venereologia. A Doença Inflamatória Intestinal, mesmo na ausência de sintomatologia digestiva, deverá ser sempre uma hipótese de diagnóstico diferencial. A Doença de Crohn é uma doença granulomatosa inflamatória do intestino, crónica e idiopática. A sintomatologia é maioritariamente digestiva, no entanto esta patologia pode ser acompanhada ou precedida de manifestações extraintestinais. Identificam-se lesões mucocutâneas em cerca de 10% dos doentes quando do diagnóstico da doença intestinal, as quais podem constituir patologias específicas ou reactivas. Observou-se uma doente de 28 anos de idade, natural de Cabo Verde, por múltiplas ulcerações, na maioria grosseiramente lineares, localizadas ao períneo, região perianal e sagrada. As lesões eram dolorosas, francamente exsudativas e acompanhavam-se de queixas de astenia, anorexia e perda ponderal. Foi efectuada biópsia de uma úlcera e o exame histopatológico da mucosa intestinal revelou um processo inflamatório com múltiplas células gigantes multinucleadas embora sem verdadeiros granulomas. A colonoscopia e o exame histopatológico da biópsia de mucosa intestinal confirmaram o diagnóstico de Doença de Crohn. O estudo laboratorial efectuado permitiu excluir patologias infeciosas como as Doenças de Transmissão Sexual e a Tuberculose. Durante o internamento a doente teve ainda uma dermatose clinicamente sugestiva de Síndroma de Sweet (que se confirmou no exame histopatológico da biópsia de uma das lesões) e uma perturbação psiquiátrica reactiva grave, com ideação suicida.
- Hemorragias do Sistema Nervoso Central em HemofiliaPublication . Leão, C; Dominguez, C; Vieira, A; Diniz, MJ; Carvalho, F
- 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.
- Sjögren's Syndrome: State of the Art on Clinical Practice GuidelinePublication . Romão, V; Talarico, R; Scirè, CA; Vieira, A; Alexander, T; Baldini, C; Gottenberg, JE; Gruner, H; Hachulla, E; Mouthon, L; Orlandi, M; Pamfil, C; Pineton de Chambrun, M; Taglietti, M; Toplak, N; van Daele, P; van Laar, JM; Bombardieri, S; Schneider, M; Smith, V; Cutolo, M; Mosca, M; Mariette, XSjögren's syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. Up to 30% to 50 % of patients with SS may present systemic disease; moreover, there is an increased risk for the development of non-Hodgkin's lymphoma that occurs in a minority of patients. The present work was developed in the framework of the European Reference Network (ERN) dedicated to Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET). In line with its goals of aiming to improve early diagnosis, treatment and care of rare connective and musculoskeletal diseases, ERN-ReCONNET set to review the current state of clinical practice guidelines (CPGs) in the rare and complex connective tissue diseases of interest of the network. Therefore, the present work was aimed at providing a state of the art of CPGs for SS.
- The Impact of COVID-19 on Rare and Complex Connective Tissue Diseases: the Experience of ERN ReCONNETPublication . Talarico, R; Aguilera, S; Alexander, T; Amoura, Z; Antunes, A; Arnaud, L; Avcin, T; Beretta, L; Bombardieri, S; Burmester, G; Cannizzo, S; Cavagna, L; Chaigne, B; Cornet, A; Costedoat-Chalumeau, N; Doria, A; Ferraris, A; Fischer-Betz, R; Fonseca, J; Frank, C; Gaglioti, A; Galetti, I; Grunert, J; Guimarães, V; Hachulla, E; Houssiau, F; Iaccarino, L; Krieg, T; Limper, M; Malfait, F; Mariette, X; Marinello, D; Martin, T; Matthews, L; Matucci-Cerinic, M; Meyer, A; Montecucco, C; Mouthon, L; Müller-Ladner, U; Rednic, S; Romão, V; Schneider, M; Smith, V; Sulli, A; Tamirou, F; Taruscio, D; Taulaigo, A; Terol, E; Tincani, A; Ticciati, S; Turchetti, G; van Hagen, P; van Laar, J; Vieira, A; de Vries-Bouwstra, J; Cutolo, M; Mosca, MDuring the COVID-19 pandemic, the need to provide high-level care for a large number of patients with COVID-19 has affected resourcing for, and limited the routine care of, all other conditions. The impact of this health emergency is particularly relevant in the rare connective tissue diseases (rCTDs) communities, as discussed in this Perspective article by the multi-stakeholder European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET). The clinical, organizational and health economic challenges faced by health-care providers, institutions, patients and their families during the SARS-CoV-2 outbreak have demonstrated the importance of ensuring continuity of care in the management of rCTDs, including adequate diagnostics and monitoring protocols, and highlighted the need for a structured emergency strategy. The vulnerability of patients with rCTDs needs to be taken into account when planning future health policies, in preparation for not only the post-COVID era, but also any possible new health emergencies.
- The Use of Recombinant Activated FVII (rFVIIa, Novoseven®) in the Treatment of Patients with Haemophilia and InhibitorsPublication . Diniz, MJ; Galvão, M; Tavares, A; Vieira, A; Falcão, MT; Catarino, C; Cruz, C