Biologics Drugs in Behçet’s Disease: A Single Centre Experience

Introduction: Behçet´s disease (BD) is a systemic vasculitis of unknown cause. Several cytokines, such as tumor necrosis factor-alpha (TNF-α), appear to play a substantial role. Therefore, biologics such as anti-TNF-α agents are rising to control severe or refractory BD´s manifestations. We aimed to describe the biological therapy´s outcomes in BD patients. Methods: A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic. We collected data regarding BD´s manifestations, treatments, and outcomes during follow-up. Results: Our cohort includes 243 patients, of whom 31% were male. During follow-up, 20 patients (8%) were treated with biological drugs. Patients who received biological therapies were younger (p = 0.030), had less frequently genital aphthosis (p = 0.009), and more frequently erythema nodosum (p = 0.009), polyarthritis (p = 0.002), spondyloarthritis (p = 0.024), retinal vasculitis (p = 0.011) and gastrointestinal manifestations (p = 0.024), namely gastroduodenal ulcer (p = 0.035), digestive bleeding from ulcers (p = 0.002), and bowel perforation (p = 0.004). Anti-TNF-α agents were used in all of these patients, most frequently infliximab. Patients started biologicals after classical immunosuppressors failure, and most went into remission (93%). Three patients developed tuberculosis during treatment, regardless of regular screening tests. It was possible to stop biological therapy in five patients, so far, without recurrence, with 33 months of mean follow-up time after suspension. Discussion: Anti-TNF-α agents are highly effective for refractory BD´s manifestations, although they are not innocuous. Little is known about the optimal duration of these therapies, regarding when and how to stop these drugs. This issue is essential not only to avoid relapses but also to reduce therapy side-effects.

Síndrome de Quilomicronemia Familiar: Algoritmo Diagnóstico

A hipertrigliceridemia, a par do aumento do risco cardiovascular pode, em níveis muito elevados, ter consequências como a pancreatite recorrente e dor abdominal. A síndrome da quilomicronemia familiar (SQF) é uma doença autossómica recessiva rara do metabolismo dos quilomicra, causada mais frequentemente por mutação do gene da lipoproteína lipase. A concentração plasmática de triglicéridos (TG) pode ser dez vezes superior ao normal. Distinguir esta condição da quilomicronemia multifactorial (QMM) pode tornar-se difícil pela semelhança fenotípica, sendo necessário realizar o teste genético. O tratamento da hipertrigliceridemia foca-se na prevenção da pancreatite em doentes com hipertrigliceridemia grave e na redução do risco cardiovascular global. O volanesorseno é um fármaco indicado na SQF que actua sobretudo pela clearance dos TG por vias independentes da LPL, atingindo em estudos de fase 2 reduções da apoC-III e de TG de 79,6% e 70,9% respectivamente; em estudos de fase 3, a redução de TG foi de 77%, com efeitos adversos minor. Os dados acerca da redução de risco cardiovascular são mais limitados. Tendo em conta a realidade nacional, os autores propõem um algoritmo de abordagem da hipertrigliceridemia que se inicia nos Cuidados de Saúde Primários com a exclusão de causas secundárias de hipertrigliceridemia e determinação da necessidade de referenciação a uma consulta de especialidade para confirmação do diagnóstico, com base no score do SQF sugerido por Moulin et al em 2018.

A Rare Case of Drug Induced Liver Injury Secondary to Empagliflozin

Drug induced liver injury (DILI) is a condition with a wide clinical spectrum. The diagnosis represents a challenge not only due to the large number of known hepatotoxic products but especially when the substance involved is not known to induce liver damage. Empagliflozin is linked to several adverse effects but has not been convincingly associated to DILI. We report a case of a 70-year-old type 2 diabetic woman that presented with gastrointestinal symptoms 1 month after empagliflozin introduction. Elevated hepatic enzymes were found and despite ultrasound evidence of vesicular microlithiasis, no biliary obstruction was confirmed. Other causes of liver injury were excluded and the diagnosis of DILI secondary to empagliflozin was made after liver biopsy. Complete clinical and laboratorial resolution was verified after empagliflozin withdrawal. Only two cases of DILI were reported since empagliflozin licensure which makes this case more interesting, alerting clinicians to an early diagnosis and appropriate treatment.

Estarão os Doentes com Fibrilhação Auricular Correctamente Anticoagulados? Um Retrato de um Hospital Português do Interior

Introdução: Os anticoagulantes orais (ACOs) demonstraram reduzir o risco de acidente vascular cerebral isquémico em doentes com fibrilhação auricular (FA). Contudo, a proporção de doentes que não está anticoagulada e tem indicação para estar é ainda elevada. O nosso objectivo é avaliar a proporção de doentes com FA que estão sob anticoagulação e avaliar a qualidade da prescrição de ACOs. Material e Métodos: Foi realizado um estudo observacional retrospectivo no Serviço de Medicina de um hospital do Alentejo (01-04/2019). Foram incluídos doentes com ≥18 anos, com história prévia de FA não-valvular e com CHA₂DS₂-VASc ≥ 2 (homens) ou ≥ 3 (mulheres). Extraiu-se o número de doentes que estava sob ACO e o número de doentes correctamente anticoagulado. Realizou-se análise estatística uni e bi-variada (IBM SPSS v.27.0). Resultados: Foram incluídos 203 doentes: 51,7% (n = 105) eram mulheres e a idade média foi 80,2 ± 9,4 anos. Cerca de 40% (n = 82) dos doentes não estavam sob ACO. Cinquenta e sete porcento (n = 20) dos doentes que tinham história prévia de eventos cerebrovasculares não estavam sob ACOs. Um quarto da amostra (n = 31) estava incorrectamente anticoagulada. Os doentes sob ACOs tendiam a ser mais novos e estar medicados um maior número de fármacos (p = 0,001 e p = 0,027, respectivamente). Conclusão: Os resultados sugerem que uma elevada proporção de doentes elegíveis para ACOs não estava medicada. Dos doentes medicados, um quarto estava incorrectamente anticoagulado. De forma a melhorar o padrão de prescrição, poder-se-ia considerar a optimização da formação contínua aos profissionais de saúde e o desenvolvimento de novos indicadores relativos à performance do sistema de saúde.

Impact of Vitamin D Supplementation in a Heart Failure Population

Introduction: Vitamin D (VD) deficiency is a major comorbidity, frequently associated to heart failure (HF). VD supplementation effects in these patients remain unknown. Therefore, this study aims to evaluate the impact of VD deficiency treatment or therapy correction in HF patients´ cohort. Material and Methods: Observational retrospective single-center study enrolling patients admitted to a HF clinic with VD deficiency. VD was prescribed to these patients with reassessment of its levels 12 months later. Study population was divided in: [VD (+) group: corrected VD deficiency] and [VD (-): maintained VD deficiency]. Variables were analysed in both groups. Results and Conclusion: Eighty-seven patients were included with no difference of baseline characteristics between the groups. Poor compliance was reported in 40% of VD (-) patients. After treatment, there were no statistically difference in variables analysed between the two groups: NYHA class I, NT-proBNP, HF hospitalizations in the previous year, Duke Activity Score Index score and 6-minute Walking Distance. VD (+) group had a statistically significant decrease of NT-proBNP level over time (1740 ± 2761 pg/mL to 851 ± 1436 pg/mL, p = 0.001). Statistically, both groups had a significant reduction of the number of HF hospitalizations, between baseline and 12 months later (1.02 ± 0.67 to 0.29 ± 0.82, p< 0.001 and 1.03 ± 1.04 to 0.40 ± 0.81, p = 0.001, for VD (+) and VD (-), respectively). Therefore, the correction of VD deficiency did not have impact in the variables analysed. The improvements reported within both groups may reflect the impact of the HF clinic optimized care.