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- Síndromes de fadiga pós-infeção na era da COVID longa: o caso da encefalomielite miálgica/síndrome de fadiga crónicaPublication . Sepulveda, Nuno; Malato, João; Winck, João Carlos; Carneiro, António Vaz; Hoffman, Joan Serra; Branco, JaimeFaz mais de quatro anos que a Organização Mundial da Saúde (OMS) declarou o início da pandemia de COVID-19. Agora, a doença já não é considerada uma prioridade de saúde pública em Portugal. Contudo, este período de maior acalmia tem revelado um outro problema já reconhecido pelo Serviço Nacional de Saúde (SNS) e pela comunidade médica portuguesa: alguns indivíduos continuam a manifestar vários sintomas após a aparente resolução da infeção pelo novo coronavírus. Alguns desses indivíduos parecem entrar numa fase crónica dos seus sintomas. Quando a duração dos sintomas atinge a barreira dos três meses, esses indivíduos recebem um diagnóstico de ‘COVID longa’ ou de ‘condição pós-COVID-19’. O quadro clínico desses pacientes é bastante variável, podendo prevalecer um cansaço persistente e profundo sem razão aparente e um mal-estar após atividades físicas, mentais e emocionais. Em particular, esse mal-estar pós-esforço (post-exertional malaise ou PEM) só desaparece ao fim de mais de 24 horas, o que sugere um processo lento de recuperação por parte do organismo. Os doentes de COVID longa podem também relatar a presença de cefaleias, problemas de concentração, perdas de memória, dispneia, entre outros sintomas.
- In-hospital mortality of high-risk pulmonary embolism: a nationwide population-based cohort study in Portugal from 2010 to 2018Publication . Calé, R.; Ascenção, Raquel; Bulhosa, C.; Pereira, Helder; Borges, Margarida; Costa, J.; Caldeira, DanielBackground: The mortality associated with high-risk pulmonary embolism (PE) is remarkably high, and reperfusion to unload right ventricle should be a priority. However, several registries report reperfusion underuse. In Portugal, epidemiological data about the incidence, rate of reperfusion and mortality of high-risk PE are not known. Methods: Nationwide population-based temporal trend study in the incidence and outcome of high-risk PE, who were admitted to hospitals of the National Health Service in Portugal between 2010 and 2018. High-risk PE was defined as patients with PE who developed cardiogenic shock or cardiac arrest. International Classification of Diseases (ICD), 9th and 10th revision, Clinical Modification codes, were used for data from the period between 2010 and 2016 (ICD-9-CM) and 2017-2018 (ICD-10-CM), respectively. The assessment focused on trends in the use of reperfusion treatment, which was defined by application of thrombolysis or pulmonary embolectomy. A comparison was made between the use or non-use of reperfusion therapy in order to examine trends in in-hospital mortality among high-risk PE cases. Results: From 2010 and 2018, there were 40.311 hospitalization episodes for PE in adult patients at hospitals of the National Health Service in mainland Portugal. There was a significant increase in the annual incidence of PE (41/100.000 inhabitants in 2010 to 46/100.000 in 2018; R2=0.582, p = 0.010). The average annual incidence was 45/100.000 inhabitants/year, with 2,7% of the PE episodes (1104) categorized as high-risk. The mortality rate associated with high-risk PE was high, although it has decreased over the years (74.2% in 2010 to 63.6% in 2018; R2=0.484; p = 0.022). Thrombolytic therapy was underused in high-risk PE, and its usage has not increased in recent years (17.3% in 2010 to 21.1% in 2018, R2=-0.127; p = 0.763). Surgical pulmonary embolectomy was used in 0.27% of cases, and there was no registry of catheter-directed thrombolysis. Patients with high-risk PE undergoing reperfusion therapy had lower in-hospital mortality compared to non-reperfused patients (OR=0.52; IC95% 0.38-0.70). Conclusion: In Portugal, between 2010 and 2018, very few patients with PE developed high-risk forms of the disease, but the mortality rate among those patients was high. The low reperfusion rate could be associated with high in-hospital mortality and highlights the need to implement advanced therapies, as an alternative to systemic thrombolysis.
- Epidemiology of psoriasis in Portugal: a population-based studyPublication . Torres, Tiago; Filipe, Paulo; Menezes Brandão, Francisco; Figueiredo, Américo; Pinto Soares, António; Sousa Basto, Artur; Rebelo, Clarisse; Correia, Osvaldo; Ferreira, Paulo; Brasileiro, Ana; Mendes-Bastos, Pedro; Paiva-Lopes, Maria João; Marques Pinto, Gabriela; Severo, Milton; Mendonça, Denisa; Oliveira, Pedro; Selores, Manuela; Massa, António; Pereira, Marta; Tavares Bello, RuiIntroduction: Psoriasis is a common, chronic, and inflammatory skin disorder with a high personal, social and economic burden and important implications for healthcare systems. The aim of this study was to provide an epidemiological characterization of individuals with psoriasis in Portugal. Material and methods: A large observational, cross-sectional, nationwide, population-based survey study developed by the Portuguese Psoriasis Group of the Portuguese Society of Dermatology and Venereology (GPP-SPDV). A structured questionnaire was designed and applied by experienced interviewers to a random, representative sample of Portuguese individuals with psoriasis and/or psoriatic arthritis. Patients were considered to have psoriasis if they replied positively to one of the following questions: "Does any physician have ever diagnosed you with psoriasis?" or "Do you have a skin disorder characterized by scaling, reddish skin lesions located in the elbows/knees/scalp?". Results: A total of 6381 individuals were interviewed, of which 283 met the criteria for psoriasis, corresponding to a prevalence rate of 4.4% (95% CI 3.95 - 4.98). Out of the participants that met psoriasis criteria, 24% had suggestive signs/symptoms but did not have a clinical diagnosis established and were not being monitored by a physician. Although more than 70% of participants had active disease (scaling, erythema, or pruritus) and one third had joint symptoms, only 12% were on systemic treatment. Fifty percent of participants with psoriasis (n = 139) had relevant comorbidities (most frequently depression/anxiety and cardiometabolic diseases). Sixteen percent of participants with psoriasis (n = 46) reported that psoriasis interfered with their daily activities (median impact of 5 in a 0 - 10 scale) and 12% mentioned the disease had an impact in their sexual life (median impact of 5 in a 0 - 10 scale). Conclusion: The results of this study suggest that the prevalence rate of psoriasis is likely to be high in Portugal, and several gaps exist at different levels of healthcare delivery to these patients, from diagnosis to treatment. This study provides important data for the future planning of interventions targeting the improvement of psoriasis care in Portugal.
- 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.
- O treino de ortostatismo (tilt training) aumenta a reserva vasoconstritora em doentes com síncope reflexa neurocardiogénicaPublication . Laranjo, Sergio; Oliveira, Mario; Tavares, Cristiano; Geraldes, Vera; Santos, Sofia; Oliveira, Eunice; Ferreira, Rui; Rocha, IsabelNeurocardiogenic syncope (NCS) is a common clinical entity resulting from an excessive reflex autonomic response, particularly during orthostatism. Treatment options are controversial and of limited effectiveness. Tilt training (TT) is a promising option to treat these patients. However, its mechanism of action and clinical impact remain unclear. Objective: To characterize hemodynamic and autonomic responses during a TT program in patients with NCS refractory to conventional measures. Methods: We studied 28 patients (50% male, mean age 41±14 years) without structural heart disease, with NCS documented by tilt testing. The TT program included 9 tilt sessions (3 times a week, 30 min) (60° - 6 sessions, 70° - 3 sessions), under ECG and blood pressure monitoring combined with home orthostatic self-training and 10° head-up during sleep. Systolic volume, cardiac output, total peripheral resistance, baroreflex sensitivity and heart-rate variability were computed. Patients were reassessed at 1 month and every 6 months for a maximum of 36 months (24±12 months). Results: Over the course of the TT program there was a significant increase in total peripheral resistance (1485±225 vs. 1591±187 dyn·s·cm(-5), p<0.05), with a decrease in standard deviation (206±60 vs. 150±42, p<0.05). During follow-up, syncope recurred in five patients (19%), with a significant reduction in the number of episodes (4.0±3.2/patient in the 12 months before TT vs. 1.4±0.8/patient post-TT, p<0.05). Conclusion: In refractory NCS, TT may be an effective therapeutic option, with long-term benefits. These results appear to be due to an increase in vasoconstrictor reserve combined with a reduction in its variance.
- Update on atopic dermatitisPublication . Torres, Tiago; Ferreira, Eduarda Osório; Gonçalo, Margarida; Mendes-Bastos, Pedro; Selores, Manuela; Filipe, PauloWith an increasing prevalence during the past decades, atopic dermatitis has become a global health issue. A literature search following a targeted approach was undertaken to perform this non-systematic review, which intends to provide an overview of the epidemiology, pathophysiology, clinical features, comorbidities, and current therapies for the treatment of atopic dermatitis. In sum, this is a heterogeneous skin disorder associated with variable morphology, distribution, and disease course. Although not completely understood, its pathogenesis is complex and seems to result from a combination of genetic and environmental factors that induce skin barrier dysfunction, cutaneous and systemic immune dysregulation, skin microbiota dysbiosis, and a strong genetic influence. Diagnosis is based on specific criteria that consider patient and family history and clinical manifestations. Overall disease severity must be determined by evaluating both objective signs and subjective symptoms. Therapeutic goals require a multistep approach, focusing on reducing pruritus and establishing disease control. Patients should be advised on basic skin care and avoidance of triggers. Topical anti-inflammatory agents should be considered in disease flares or chronic/recurrent lesions. In case of inadequate response, phototherapy, systemic immunosuppressants and, more recently, dupilumab, should be added. Nevertheless, the treatment of moderate-to-severe atopic dermatitis remains challenging and novel, efficacious, safe and targeted treatments are urgently needed. In conclusion, although the last few years have seen important improvement in the understanding of the disease, future research in atopic dermatitis will continue exploring gene-environment interactions and how it affects pathophysiology, disease severity, and treatment outcomes.
- Atherosclerosis in the primary health care setting: a real-word data studyPublication . Ascenção, Raquel; Alarcão, Joana; Araujo, Francisco; Costa, João; Fiorentino, Francesca; Gil, Victor; Gouveia, Miguel; Lourenço, Francisco; Mello e Silva, Alberto; Carneiro, António Vaz; Borges, MargaridaIntroduction and objectives: To characterize patients with atherosclerosis, a disease with a high socioeconomic impact, in the Lisbon and Tagus Valley Health Region. Methods: A cross-sectional observational study was carried out through the Lisbon and Tagus Valley Regional Health Administration primary health care database, extracting data on the clinical and demographic characteristics and resource use of adult primary health care users with atherosclerosis during 2016. Different criteria were used to define atherosclerosis (presence of clinical manifestations, atherothrombotic risk factors and/or consumption of drugs related to atherosclerosis). Comparisons between different subpopulations were performed using parametric tests. Results: A total of 318 692 users were identified, most of whom (n=224 845 users; 71%) had no recorded clinical manifestations. The subpopulation with clinical manifestations were older (72.0±11.5 vs. 71.3±11.0 years), with a higher proportion of men (58.0% vs. 45.9%), recorded hypertension (78.3% vs. 73.5%) and dyslipidemia (55.8% vs. 53.5%), and a lower proportion of recorded obesity (18.2% vs. 20.8%), compared to those without clinical manifestations (p<0.001). Mean blood pressure, LDL-C and glycated hemoglobin values were lower in the subpopulation with manifestations (142/74 vs. 146/76 mmHg, 101 vs. 108 mg/dl, and 6.80 vs. 6.84%, respectively; p<0.001). Each user with atherosclerosis attended 4.1±2.9 face-to-face medical consultations and underwent 8.6±10.0 laboratory test panels, with differences in subpopulations with and without clinical manifestations (4.4±3.2 vs. 4.0±2.8 and 8.3±10.3 vs. 8.7±9.8, respectively; p<0.001). Conclusions: About one in three adult primary health care users with atherosclerosis have clinical manifestations. The results suggest that control of cardiovascular risk factors is suboptimal in patients with atherosclerosis.
- Prognostic factors after liver resection for colorectal liver metastasisPublication . Matias, Margarida; Casa-Nova, Mafalda; Faria, Mariana; Pires, Ricardo; Tato-Costa, Joana; Ribeiro, Maria Leonor; Costa, LuisIntroduction: Surgery is the only potentially curative treatment for patients with colorectal liver metastases, resulting in 5-year survival rates of 36 - 58. Although many studies have been performed to determine prognostic factors for tumor recurrence and survival after resection of colorectal liver metastases, there are few prognostic scoring systems stratifying patients undergoing surgery for colorectal liver metastases into risk group models. Objectives: To identify, evaluate and compare the existing prognostic scores for survival after surgery for resection of colorectal liver metastases. Material and methods: Electronic search in PubMed, Cochrane and Embase from 1990 to 2013 using the terms ' hepatic resection', ' colorectal cancer' , 'liver metastasis', ' hepatectomy', ' prognostic' , and ' score' . Only studies proposing a prognostic model or risk stratification based on clinical and/or pathological variables were included. Results: From 1996 to June 2013, 19 scoring systems were identified, including one nomogram. Thirty prognostic factors were identified although none of the factors was common to all prognostic models. The 4 factors most often included were: number of liver metastases, regional lymph node metastization of primary tumor, preoperative CEA level and maximum size of metastases. The median study sample size was 305 patients (81-1 568 patients) and median follow-up was 33 months (16-54 months). All studies were retrospective and used the Cox proportional hazards model for multi-variable analysis. Conclusion: Several factors have been constantly reported as having prognostic value after liver resection of colorectal livermetastases, although there is no consensus on the ideal scoring system.
- Clinical and laboratory factors associated with prolonged hospital stay among patients with cellulitis/erysipelasPublication . Roda, Ângela; Pinto, Ana Marcos; Filipe, Ana Rita; Travassos, Ana Rita; Freitas, João Pedro; Filipe, PauloIntroduction: Cellulitis and erysipelas represent the most frequent cause of hospitalization in the dermatology department of Santa Maria Hospital in Lisbon, Portugal. The aim of this study was to investigate whether patient demographics, comorbidities, previous episodes of cellulitis/erysipelas, the presence of complications, laboratory markers at admission, microbial isolation or previous use of antibiotics, are associated with prolonged stays. Material and methods: Retrospective analysis, including patients admitted with cellulitis/erysipelas in the inpatient dermatology department of Santa Maria Hospital between July 1st 2012 and June 30th 2017. Results: There were 372 admissions, corresponding to 348 patients. The median length of stay was 11 days. Increased age (p = 0.002, OR 1.03, 95% CI 1.01 - 1.04), previous episode of cellulitis/erysipelas requiring hospitalization (p = 0.005, OR 4.81, 95% CI 1.63 - 14.23), the presence of cellulitis/erysipelas-associated complications (p = 0.001, OR 3.28, 95% CI 1.63 - 6.59), leukocytosis (p = 0.049, OR 1.81, 95% CI 1.00 - 3.30), high levels of C-reactive protein (p = 0.035, OR 1.03, 95% CI 1.00 - 1.06) and a positive culture result (p = 0.002, OR 2.59, 95% CI 1.41 - 4.79) were associated with prolonged hospitalization. Discussion: Prolonged hospitalization for cellulitis/erysipelas is associated with higher costs, additional clinical investigation, invasive treatments, prolonged courses of antibiotic therapy, risk of nosocomial infections, and delayed return to activities of daily living. Thus, the investigation of clinical-laboratory factors associated with prolonged hospitalization for cellulitis / erysipelas is essential and may be useful for the construction of a severity score. Conclusion: The knowledge of the characteristics that are associated with prolonged stay among patients with cellulitis/erysipelas may be relevant to improve health care, by reducing the length of hospital stay and associated risks and costs.