Nailfold capillaroscopy and autoimmune connective tissue diseases in patients from a Portuguese nailfold capillaroscopy clinic.

Raynaud's phenomenon (RP) is frequent in autoimmune connective tissue diseases (AICTD) and its approach includes nailfold capillaroscopy (NFC), as it is a non-invasive technique that permits direct visualization of the microcirculation. The aim of this study is to analyze and establish clinical correlations between NFC findings and particular aspects of autoimmune disorders. This is a retrospective study. Clinical data from patients attending our NFC clinic were reviewed. Inclusion criteria included AICTD previous diagnosis, which included systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), Sjögren syndrome, inflammatory idiopathic myopathies (IIM), rheumatoid arthritis, undifferentiated connective tissue disease and antiphospholipid syndrome (APS). Videocap® version 3.0 biomicroscope was used. NFC score was determined. For statistics, SPSS software was utilized. 384 patients were included; most of them were women, with mean age of 47 years. RP was present in 91% of the patients, with greater prevalence in SSc and MCTD. Scleroderma pattern was the most prevalent NFC pattern, mainly in SSc, MCTD and IIM. Mean capillary density was reduced in IIM, SSc and MCTD. NFC score was worse in SSc, IIM and MCTD. In patients with AICTD, RP is related to microvascular damage and worse NFC score. NFC scleroderma pattern correlates with SSc classification criteria score. In MCTD, scleroderma pattern relates to myositis. SLE and APS reveal significant hemorrhages, but not related to APS antibodies. This study highlights the possible role of NFC as biomarker of AICTD, particularly in SSc and IIM.

The Prevalence of COVID-19 Fog and the Impact on Quality of Life After SARS-CoV-2 Infection (QoL-COVID): A Cross Sectional Study.

INTRODUCTION: Coronavirus has an impact on both the physical and mental health of individuals. The literature regarding the patient's health status post-SARS-CoV-2 is still scarce with limited data on the prevalence of residual symptoms and quality of life (QoL) after the infection. The aim of this study was to understand the impact of SARS-CoV-2 on patient QoL, and remaining symptoms. MATERIAL AND METHODS: Single center cross-sectional study of patients who had been admitted to our COVID-19 ward between March 2020 and March 2021. By applying a QoL questionnaire (EQ-5D-5L) we assessed the overall sample, at three time points and in different groups of patients: those admitted to the intensive care unit (ICU) and the elderly. RESULTS: A total of 125 participants were included in our study. Most patients who were admitted had a severe course of disease (51%), with 22% of admissions to the ICU, with 8% requiring prone ventilation, 10% experiencing thrombotic complications and 18% of nosocomial infections throughout the admission. As for persistent symptoms related with COVID-19 fog, the most frequent were fatigue (57%), memory loss (52%) and insomnia (50%). Regarding QoL, the average decrease was 0.08 ± 0.2 in the index and 8.7 ± 19 in the Visual Analogue Scale (VAS). The QoL index decrease correlated with age, chronic obstructive pulmonary disease, asthma and heart failure, and all persistent symptoms, significantly. QoL VAS correlated significantly with fatigue, mood changes, difficulty concentrating and memory loss. The decrease in QoL and the persistent symptoms remained overall stable over the three time points. The ICU group showed no statistically significant difference in QoL, but the most frequently persistent symptoms were mood changes and attention disturbances. However, the elderly experienced a worsening in QoL expressed by index (0.69 ± 0.3 vs 0.8 ± 0.2, p-value = 0.01). CONCLUSION: A decrease in QoL was observed following SARS-CoV-2 infection, correlating with both chronic conditions and persistent symptoms. The lack of difference through time points of both QoL and persistent symptoms suggests a long-standing effect.

Absolute Hyperglycemia versus Stress Hyperglycemia Ratio for the Prognosis of Hospitalized Patients with COVID-19 in the First Months of the Pandemic: A Retrospective Study.

Diabetes is a risk factor for greater severity of coronavirus disease 2019 (COVID-19). The stress hyperglycemia ratio (SHR) is an independent predictor of critical illness, and it is reported to have a stronger association than absolute hyperglycemia. The aim of this study was to assess the relationship of absolute hyperglycemia and SHR with the severity of COVID-19, since there are no studies investigating SHR in patients with COVID-19. We conducted a retrospective observational study on hospitalized patients with COVID-19 in the first months of the pandemic, regarding absolute hyperglycemia, SHR, and severity outcomes. Of the 374 patients, 28.1% had a previous diagnosis of type 2 diabetes. Absolute hyperglycemia (64.8% versus 22.7%; p < 0.01) and SHR [1.1 (IQR 0.9-1.3) versus 1.0 (IQR 0.9-1.2); p < 0.001] showed a statistically significant association with previous diabetes. Absolute hyperglycemia showed a significant association with clinical severity of COVID-19 (79.0% versus 62.7%; p < 0.001), need for oxygen therapy (74.8% versus 54.4%; p < 0.001), invasive mechanical ventilation (28.6% versus 11.6%; p < 0.001), and intensive care unit (30.3% versus 14.9%; p = 0.002), but not with mortality; by contrast, there was no statistically significant association between SHR and all these parameters. Our results are in agreement with the literature regarding the impact of absolute hyperglycemia on COVID-19 severity outcomes, while SHR was not a significant marker. We therefore suggest that SHR should not be evaluated in all patients admitted in the hospital for COVID-19, and we encourage the standard measures at admission of blood glucose and HbA1c levels.

Non-bacterial Thrombotic Endocarditis as a Rare Manifestation of Early Stage Gastric Cancer.

Endocarditis is an inflammation of the endocardium and is characterized by the presence of vegetation, which may occur in the context of infectious or non-infectious diseases. Despite the higher rate of infective endocarditis diagnosis, it may also surge in other non-infectious conditions such as cancer or chronic inflammatory syndromes. Cancer defines a hypercoagulable state, and cancer-associated thrombophilia can have a diverse clinical presentation, most commonly venous thromboembolism and rarely non-bacterial thrombotic endocarditis (NBTE). The diagnosis of NBTE is difficult and requires a high level of suspicion. The treatment relies on anticoagulant therapy, control of underlying disease, and valve replacement when applied. Independently of the etiology, without treatment, endocarditis may lead to valve dysfunction and to the worst prognosis. In this paper, we describe a case of a patient with persistent fever and NBTE of the tricuspid valve, disclosing a rare presentation of gastric cancer.

Primary Anti-Phospholipid Antibody Syndrome: Real-World Defining Features of Rethrombosis in the Course of Disease.

Objective: We aimed to identify features that allow differentiation of primary antiphospholipid syndrome (PAPS) patients that suffer recurrent thrombotic events (RTE) despite anticoagulation, from the other diagnosed PAPS patients. Methods: This was an exploratory study of anticoagulated PAPS patients attending an Autoimmune Diseases Unit (1998-2018). From 2016, anti-phospholipid antibodies and lupus anticoagulant were determined for each patient at consecutive visits, collected together with retrospective clinical characteristics, laboratory, and therapeutic markers and compared according to the occurrence of thrombotic events during follow-up. Results: Overall, two thirds of the patients were female, 93% were Caucasian, with a median age of 40 years at diagnosis, for a median time of 11.5 years in follow-up. Out of 54 patients, 10 were identified with RTE. There were no significant differences among the RTE and non-RTE patients as far as classical risk factors for clotting disorders. The RTE group was characterized by a higher proportion of younger patients, male sex and positivity for all laboratory markers, and initially and over follow-up as well as a sustained high-risk profile based on APS laboratory markers. Anticardiolipin IgG at onset was the only statistically significant marker of the RTE group. At the end of follow-up, consistent reversion to negative status was a rare event, observed in 20% of RTE vs. 25% of non-RTE patients. Conclusions: Despite therapy, we were able to identify features associated to thrombotic events in patients with PAPS. Prospectively regular clinical and laboratory monitoring might be warranted in order to treat APS more assertively.

Does the Hyperglycemia Impact on COVID-19 Outcomes Depend upon the Presence of Diabetes?-An Observational Study.

Diabetes mellitus (DM) has emerged as a major risk factor for COVID-19 severity and SARS-CoV-2 infection can worsen glycemic control and may precipitate new-onset diabetes. At-admission hyperglycemia (AH) is a known predictor for worse outcomes in many diseases and seems to have a similar effect in COVID-19 patients. In this study, we aimed to assess the impact of AH regardless of pre-existing diabetes mellitus and new-onset diabetes diagnosis in the clinical severity of COVID-19 inpatients in the first months of the pandemic. A retrospective monocentric study on 374 COVID-19 inpatients (209 males) was developed to assess associations between AH (blood glucose levels in the Emergency Department or the first 24 h of hospitalization greater than 140 mg/dL) and severity outcomes (disease severity, respiratory support, admission to Intensive Care Unit (ICU) and mortality) in patients with and without diabetes. Considering diabetic patients with AH (N = 68;18.1%) there was a correlation with COVID-19 severity (p = 0.03), invasive mechanical ventilation (p = 0.008), and ICU admission (p = 0.026). No correlation was present with any severity outcomes in diabetic patients without AH (N = 33; 8.8%). All of the New-onset Diabetes patients (N = 15; 4%) had AH, and 12 had severe COVID-19; additionally, five patients were admitted to the ICU and three patients died. However, severity outcomes did not reach statistical correlation significance in this group. In nondiabetic patients with AH (N = 51; 13.6%), there was a statistically significant association with the need for oxygen therapy (p = 0.001), invasive mechanical ventilation (p = 0.01), and ICU admission (p = 0.03). Our results support data regarding the impact of AH on severity outcomes. It also suggests an effect of AH on the prognosis of COVID-19 inpatients, regardless of the presence of pre-existing diabetes or new-onset diabetes. We reinforce the importance to assess at admission glycemia in all patients admitted with COVID-19.

Patients' Perspectives on Determinants Avoidable Hospitalizations: Development and Validation of a Questionnaire.

Ambulatory care sensitive conditions (ACSC) can be avoided through effective care in the ambulatory setting. Patients are the most qualified individuals to express the social and individual contexts of their own experience. Thus, understanding why potentially preventable hospitalizations occur is important to develop patient-centred policies or interventions that may reduce them. This study aims to develop and validate a questionnaire to capture the patients' perspective on the causes of the hospitalizations for ACSC. The development of a new questionnaire involved four phases: a literature review, face validity, pre-test, and validation. We conducted a three-step face validity verification to confirm the relevance of the identified determinants and to collect determinants not previously identified by interviewing healthcare providers, representatives of patients' associations, and patients. Determinants were identified through the literature review predominantly in the "Healthcare Access", "Disease self-management", and "Social Support" domains. The validated resulting questionnaire comprises 25 questions, distributed by two dimensions (individual/contextual) covering seven domains and 20 determinants of ACSC hospitalization. Currently, there are no validated instruments as comprehensive and easy to use as the one described in this paper. This questionnaire should provide a base for further language/context validations.

Correction to the article "Implementing an Influenza Vaccine Effectiveness Study in a Hospital Context in Portugal: The EVA Hospital Project", Published on Acta Med Port 2021 Jan;34(1):20-27.

On page 23, Figure 1, where it reads: "Controls 157 2015-2016 season = 47" (area circled in the image bellow) It should read: (see new Figure 1 on pag. 62). On page 25, Table 2, third line of the last column, under "p-value", where it reads: 1 (see Table 2 in page 63) It should read: 1.000 (see new Table 2, page 64) Article published with errors: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13438.

Na página 23, Figura 1, onde se lê:"Controls 1572015-2016 season = 47"Deverá ler-se: (ver nova Figura 1 na página 62)Na página 25, Tabela 2, terceira linha da última coluna intitulada "p-value", onde se lê: 1 (ver Tabela 2 na pág. 63)Deverá ler-se: 1.000 (ver nova Tabela 2, pág. 64)Artigo publicado com erros: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13438.

Implementing an Influenza Vaccine Effectiveness Study in a Hospital Context in Portugal: The EVA Hospital Project.

INTRODUCTION: The project 'Integrated Monitoring of Vaccines in Europe' aimed to measure seasonal influenza vaccine effectiveness against hospitalised adults, aged 65 years and over, with influenza. We describe the protocol implementation in Portugal. MATERIAL AND METHODS: We implemented a test-negative design, targeting community-dwelling patients aged 65 years old and over hospitalised with severe acute respiratory illness. Patients were reverse transverse-polymerase chain reaction tested for influenza. Cases were those positive for influenza while others were controls. Most variables were collected using hospital medical records. Selection bias was evaluated by comparison with the laboratory influenza test requests database according to demographic characteristics. Crude, season-adjusted influenza vaccine effectiveness was estimated as = 1 - odds ratio, and 95% confidence intervals were obtained by conditional logistical regression, matched with the disease onset month. RESULTS: The recruitment rate was 37.8%. Most participants (n = 368) were female (55.8%) and aged 80 years old and over (55.8%). This was similar to values for potentially eligible severe acute respiratory illness patients (80 years old and over: 56.8%, female: 56.2%). The proportion of missing values was below 2.5% for 20 variables and above 5% (maximum 11.6%) for six variables. Influenza vaccine effectiveness estimates were 62.1% against AH1pdm09 (95% confidence intervals: -28.1 to 88.8), 14.9% against A(H3N2) (95% confidence intervals: -69.6 to 57.3), 43.6% against B/Yam (95% confidence intervals: -66.2 to 80.8). DISCUSSION: Given the non-existence of a coded admission database in either participating hospital the selection of severe acute respiratory illness due to clinical features was the feasible one. These results are only valid for the older adult population residing in the catchment area of the two participating hospitals who were admitted to a public hospital with severe influenza or SARI symptoms. CONCLUSION: Despite the low participation rate, we observed comparable characteristics of participants and eligible severe acute respiratory illness patients. Data quality was high, and influenza vaccine effectiveness results were in accordance with the results of meta-analyses and European season-specific estimates. The final sample size was low, which inhibited obtaining estimates with good precision.

Introdução: O projeto "Integrated Monitoring of Vaccines in Europe" pretende medir a efetividade da vacina antigripal nas hospitalizações por gripe nos adultos com mais de 65 anos. Este estudo pretende descrever a implementação do protocolo em Portugal. Material e Métodos: Implementou-se um estudo com desenho caso-controlo teste negativo. A população-alvo foram indivíduos com idade superior a 65 anos, hospitalizados com doença respiratória aguda grave. Os doentes foram testados para gripe por reverse transverse-polimerase chain reaction. Foram considerados casos aqueles com resultado positivo; os restantes foram controlos. Os dados foram obtidos através de registo clinicos. O potencial viés de seleção foi avaliado por comparação de características demográficas e enfermarias com dados das requisições laboratoriais. A efetividade da vacina, foi estimada em 1 ­ odds ratio por regressão logística condicional, emparelhada para o mês de início da doença. Resultados: A taxa de recrutamento foi de 37,8%. A maioria dos participantes (n = 368) era do sexo feminino (55,8%) e tinha idade superior a 80 anos (55,8%). Padrão similar foi verificado nos doentes elegíveis (idade superior a 80 anos: 56,8%; feminino: 56,2%). Os valores omissos foram inferiores a 2,5% em 20 variáveis e acima de 5% (máximo 11,6%) em seis variáveis. As estimativas da efetividade foram 62,1% contra AH1pdm09 (intervalo de confiança IC 95%: -28,1, 88,8); 14,9% contra A (H3) (intervalo de confiança 95%: -69,6; 57,3) e 43,6% contra B/yamagata (intervalo de confiança 95%: -66,2; 80,8). Discussão: Dada a inexistência de uma codificação em base de dados de admissão em qualquer um dos hospitais participantes, a abordagem de identificação e casos clínicos de doença respiratória aguda grave foi a exequível. Estes resultados são válidos para a população idosa residente na área de abrangência dos dois hospitais participantes que foram internados em um hospital público com gripe grave ou sintomas de doença respiratória aguda grave. Conclusão: Apesar da baixa taxa de participação, observámos características comparáveis entre os participantes e os doentes elegíveis. A qualidade dos dados foi elevada, e os resultados da efetividade concordantes com resultados de meta-análises e estimativas europeias. A reduzida dimensão da amostra impediu a obtenção de estimativas mais precisas.

Biological Therapy in Patients with Rheumatoid Arthritis in a Tertiary Center in Portugal: A Cross-Sectional Study.

INTRODUCTION: Clinical outcomes in rheumatoid arthritis have greatly improved with therapeutic advances. Despite the availability of substantial clinical trial evidence, there is a lack of real-life data. The aim of this study was to assess disease status and quality of life in an outpatient population treated with biological disease-modifying anti-rheumatic drugs. MATERIAL AND METHODS: Cross-sectional study recalling all patients ever treated in our unit with biological disease-modifying antirheumatic drugs. Clinical and demographic data, compliance, disease activity, functional status, joint deformities, and comorbidities were documented, and patients queried on occupational status, education, marital status and generic health related quality of life questionnaires. RESULTS: Recall was attended by 77 of the original 94 patients. At recall, median age was 63 years old, 82% of the patients were female and the median disease duration was 12 years. Biological therapy was started at a median of four years following disease onset. According to the disease activity score (DAS28), the percentage of patients with high, moderate, low disease activity or remission changed from 50, 45, 0 and 5 (pre-therapy) to 11, 37, 25 and 26 at recall, respectively; functional status was significantly improved. Seventy-five per cent of the patients retained the original treatment with good compliance. Lower Short Form-36 domain scores accompanied a low EQ-5D-3L score. Deceased patients (n = 6) had a lower estimated 10-year survival rate. In this group, biological therapy was discontinued at a higher frequency during follow-up. DISCUSSION: A high disease activity and a high HAQ disability index characterized most patients at pre-bDMARD onset. CONCLUSION: Despite therapy switches and regular follow-up, a significant percentage of patients still presented with moderate disease activity, functional impairment and a poor health-related quality of life.

Introdução: Avanços no tratamento da artrite reumatóide contribuiram para uma evolução favorável. Apesar de evidências substanciais provenientes de ensaios clínicos, são menos conhecidos os dados provenientes da vida real. O objetivo do estudo foi caracterizar a doença e a qualidade de vida em doentes sob fármacos biotecnológicos. Material e Métodos: Trata-se de um estudo transversal com recolha de dados clínicos relativos à adesão terapêutica, atividade da doença, capacidade funcional, deformidades articulares, comorbilidades e questionários de qualidade de vida relacionada com a saúde, estado civil, situação profissional e escolaridade. Resultados: Foram recrutados 77 doentes do grupo original de um total de 94. A mediana da idade foi 63 anos, 82% do sexo feminino e início de biológico cerca de quatro anos após o início da doença, com uma mediana de duração de 12 anos. De acordo com o disease activity score (DAS28), a percentagem de doentes com atividade alta, moderada, baixa ou em remissão mudou, respectivamente, de 50, 45, 0 e 5 pré- biológico para 11, 37, 25 e 26 na altura da re-avaliação, com melhoria funcional. Setenta e cinco por cento dos doentes mantiveram o tratamento original com boa adesão. Pontuações mais baixas do short form-36 associaram-se a uma baixa pontuação no EQ-5D-3L. No grupo de doentes que viriam a falecer (n = 6), foi observada uma menor esperança de vida aos 10 anos, assim como uma maior discontinuação da terapêutica biológica. Discussão: Pré-biológico, uma elevada percentagem dos doentes apresentava elevada atividade da doença e incapacidade funcional. Conclusão: Não obstante ajustes terapêuticos e seguimento regular, uma percentagem significativa de doentes mantinha atividade moderada e limitação funcional com baixa qualidade de vida relacionada com a saúde.

Clinical Presentation and Long-Term Outcomes of Systemic Sclerosis Portuguese Patients from a Single Centre Cohort: A EUSTAR Registration Initiative.

INTRODUCTION: Systemic sclerosis is a complex disorder that requires systematic screening. Our objective is to report the European Scleroderma Trials and Research group centre affiliation and its impact in our clinical practice. MATERIAL AND METHODS: The European Scleroderma Trials and Research group affiliation process, database update and current patient evaluation, with respect to demographic and clinical features. Cumulative mortality was analysed. RESULTS: We identified 19 female patients (which met all the American College of Rheumatology/ European League Against Rheumatism 2013 criteria for systemic sclerosis) under current follow-up, divided according to the LeRoy classification into diffuse cutaneous (n = 5), limited cutaneous (n = 11) and limited (n = 3) types, followed for a median period of 5, 12 and 6 years, respectively. Raynaud´s phenomenon and abnormal nailfold capillaries were universally present. Interstitial lung disease was absent in the limited cutaneous form but present in 100% of the diffuse subtype. Pitting scars were more common in the diffuse form. Active disease was also more frequent in the diffuse form, and most patients with active disease were treated with anti-endothelin receptor antagonists. Over 21 years (from 1994 to 2015) the mortality rate was 55% (n = 23/42). Age at time of death was significantly lower in the diffuse subtype. DISCUSSION: Our single centre cohort shares many features with larger and international reports and more specifically is in accordance with patient characteristics described in the European Scleroderma Trials and Research group registries. CONCLUSION: The European Scleroderma Trials and Research group registration motivated our systematic patient characterization and may be used as a tool for homogenous disease registries.

Introdução: A esclerose sistémica é uma doença complexa que requer uma vigilância regular e sistemática. Este estudo teve como objetivo divulgar a afiliação da nossa Unidade no registo Europeu Scleroderma Trials and Research group e o seu impacto na prática clínica. Material e Métodos: Processo de afiliação Scleroderma Trials and Research group, atualização da base de dados, avaliação sistemática das características demográficas e clínicas dos doentes em seguimento e análise da mortalidade cumulativa. Resultados: Foram identificados 19 doentes do sexo feminino (com preenchimento completo dos critérios de classificação de esclerose sistémica do American College of Rheumatology/ European League Against Rheumatism 2013), seguidas no momento atual e divididas pela classificação de LeRoy em três formas: cutânea difusa (n = 5), cutânea limitada (n = 11) e limitada (n = 3), com um período de seguimento com uma duração mediana de 5, 12 e 6 anos, respetivamente. O fenómeno de Raynaud e as anomalias nos capilares peri-ungueais estavam presentes em todas as doentes. Todas as doentes com a forma difusa apresentavam doença intersticial pulmonar, ausente na forma cutânea limitada. As pitting scars foram mais frequentes na forma difusa. A doença ativa foi mais frequente na forma difusa, na sua maioria tratada com antagonistas dos recetores da endotelina. Num período de 21 anos (de 1994 a 2015), a mortalidade foi de 55% (n = 23/42). A expectativa de vida nos doentes com a forma difusa estava significativamente reduzida quando comparada com a forma localizada. Discussão: O nosso grupo de doentes é semelhante a outros de maiores dimensões e cariz internacional e, mais especificamente, enquadra-se nas características dos doentes registados nos registos Scleroderma Trials and Research group. Conclusão: O registo Scleroderma Trials and Research group incentivou uma caracterização sistemática e pode revelar-se um veículo para a criação de registos mais homogéneos.

Leprosy and rheumatoid arthritis: consequence or association?

Leprosy or Hansen's disease is a chronic granulomatous infectious disease caused by Mycobacterium leprae with a high prevalence in some developing countries however, it is rarely seen in non-endemic regions. Arthritis has been described in all types of Hansen's disease. Chronic arthritis is known to exist even in paucibacillary forms, resolved or treated disease and in patients without reaction, suggesting a perpetuated inflammatory process. In these cases leprosy can mimic some autoimmune diseases such as rheumatoid arthritis. When a patient with a history of leprosy presents with a symmetric, distal, polyarthritis the diagnosis may not be linear. Possibly it is a rheumatoid-like leprous arthritis with M leprae acting as the trigger element for the chronic process or it is an overlap condition, with a concomitant rheumatoid arthritis? A case report of a patient with a chronic inflammatory arthritis with 10 years of evolution is presented. The differential diagnosis between leprous and rheumatoid arthritis is discussed.

Antiphospholipid syndrome and recurrent thrombosis--limitations of current treatment strategies.

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder that is characterised by the presence of antiphospholipid antibodies and a common cause of vascular thromboembolic phenomena. The management of patients with APS is currently directed to antithrombotic medications. The international therapeutic guidelines recommend oral anticoagulation with warfarin indefinitely after the first thrombotic episode. However, therapeutic guidelines lack for a minority group of patients - the patients appropriately anticoagulated with recurrent thromboembolic phenomena. The authors present a clinical report that reveals the therapeutic and diagnostic complexity of this specific group of patients. Regarding recent studies, APS has been revealed as a complex syndrome with multiple pathophysiological mechanisms previously unknown. In this context, new therapeutic approaches have been defended and empirically experienced, with potentially promising results.

Actinobacillus endocarditis associated with hypertrophic cardiomyopathy.

Infective endocarditis can be associated with complex clinical presentations, sometimes with a difficult multi-disciplinary management. Actinobacillus actinomycetemcomitans belongs to the Haemophilus species, Actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens and Kingella species group, responsible for 5% to 10% of infective endocarditis in native heart valves. These organisms have slow fastidious growth pattern, often associated with negative cultures, and cause systemic embolism with abscess formation. The authors present the case of a 59-year-old man, admitted due to fever of unknown origin, with a personal history of obstructive hypertrophic cardiomyopathy and recent dental manipulation. The diagnosis of mitral valve's endocarditis was established after a transoesophageal ecocardiography, with a late isolation of A actinomycetemcomitans in blood culture. Despite the institution of antibiotic therapy, the patient suffered from multiple episodes of septic embolism: skin, mucosae, cerebral abscesses, spondylodiscitis and uveitis. He was submitted to heart surgery with miectomy and replacement of the native mitral valve by a mechanical prosthesis, while on antibiotics.

The dark side of SAPHO syndrome.

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a relatively rare entity. The therapeutic approach of patients with SAPHO syndrome has included multiple drugs with varying success and incoherence responses. The therapy is still empirical today. SAPHO syndrome is commonly treated with non-steroidal anti-inflammatory drugs, bisphophonates and non-biologic disease modifying antirheumatic drugs. Recent reports showed successful treatment with tumour necrosis factor α (TNF α) antagonists, but there is still a dark side of SAPHO syndrome including a subgroup of patient's refractory to all the treatments that have been empirically experienced. A clinical report of a patient with SAPHO syndrome with 12 years of evolution is described. All the therapeutic approaches, including anti TNF α therapy, have not prevented the clinical and radiographic progression of the disease. Given that the disease affects mostly younger patients, new therapeutic strategies are necessary in order to avoid potentially irreversible joint and bone lesions.

Buerger's disease (Thromboangiitis obliterans): a diagnostic challenge.

Buerger's disease or Thromboangiitis obliterans is a segmental inflammatory disease that affects the vessels and nerves of the extremities. It usually affects men below 45 years old and correlates with tobacco, as a predisposing factor. The authors present the case of a 34-year-old male, with ulcers in the fingertips with progressive worsening: acrocyanosis, slow healing, necrosis and finally loss of substance. Dorsalis pedis and posterior tibial pulses were not palpable. Personal history of heavy smoking was (20 pack-years). The angiography revealed proximal occlusion of the left posterior tibial and interosseal arteries, with distal circulation by the anterior tibial artery. He was submitted to disarticulation of the second left toe and therapy with pentoxifyline and iloprost infusion, calcium antagonist, antiplatelet drugs, statin and low molecular weight heparin (later replaced by oral anticoagulation). Improvement was seen of active vascular lesions and pain symptoms.