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  • Primary Antiphospholipid Syndrome Presenting With Life-Threatening Diffuse Alveolar Hemorrhage
    Publication . Casanova Pinto, J; Costa, MG; Fernandes, B; Ramalheira, C
    Antiphospholipid syndrome (APS)-associated diffuse alveolar hemorrhage (DAH) results from antiphospholipid-induced endothelial dysfunction, microvascular thrombosis, and complement activation. Management requires balancing thrombosis prevention and hemorrhage control. High-dose corticosteroids are the first-line therapy. We report a case of a 33-year-old male presenting with progressive dyspnea, hemoptysis, and hypoxemia. Initial evaluation revealed thrombocytopenia, anemia, elevated inflammatory markers, and diffuse bilateral ground-glass opacities on a thoracic computed tomography, consistent with DAH. Autoimmune serology confirmed persistent positivity for lupus anticoagulant, anti-cardiolipin IgM, and anti-β2-glycoprotein-I IgM, fulfilling APS classification criteria. The patient developed respiratory failure, requiring mechanical ventilation and venovenous extracorporeal membrane oxygenation (VV-ECMO). High-dose corticosteroids were initiated, leading to gradual improvement. He was successfully extubated and discharged on long-term anticoagulation with warfarin. At four years of follow-up, he remains free of APS-related complications. This case highlights the importance of recognizing APS as a cause of DAH, particularly in patients with unexplained pulmonary hemorrhage. Early diagnosis and aggressive therapy can improve outcomes.
  • Long-term cardiovascular events in individuals hospitalised with COVID-19: a retrospective cohort
    Publication . Soares, P; Ruivinho, C; Silva, J; Lobão, MJ; Santos, L; Paixão, J; Ramalho, AR; Henriques, A; Simões, I; Guimarães, LE; Moça, R; Costa, A; Atanásio, G; Nóbrega, S; da Luz Brazão, M; Goes, AR; Leite, A
    Background: Post-COVID condition encompasses a spectrum of persistent or emerging symptoms affecting multiple organ systems, including a heightened risk of cardiovascular complications. Despite growing recognition of this phenomenon, there remains a lack of comprehensive data regarding the incidence and risk factors associated with cardiovascular events during the post-acute phase in patients previously hospitalised for COVID-19. Thus, we aimed to estimate the incidence of cardiovascular events among patients hospitalised for COVID-19 in Portugal and assess the association between patient and infection characteristics and cardiovascular events in the COVID-19 post-acute phase. Methods: We conducted a registry-based retrospective cohort study from seven hospitals across Portugal. Data was retrospectively collected from the electronic medical record of each patient. We included individuals hospitalised due to COVID-19 between March 2020 and March 2021. Our outcome of interest was cardiovascular events in the post-acute phase of COVID-19, occurring at least 30 days after a positive SARS-CoV-2 test. The variables of interest considered were the severity of the episode, existing cardiovascular risk and vaccination status before the SARS-CoV-2 test. Person-years was estimated for each individual, and incidence rates were estimated. A Cox proportional hazard regression model was employed to assess risk factors. Results: We included 1,803 patients in the analysis, of which 143 (7.9%) experienced at least one cardiovascular event following COVID-19 hospitalisation. The overall incidence rate of having at least one cardiovascular event was 34.65 per 1,000 person-years (95% confidence interval (CI): 29.20; 40.82). We found higher risk of cardiovascular events for individuals with pre-existing cardiovascular risk (adjusted hazard ratio (aHR): 3.76, 95% CI: 1.53; 9.24) and lower risk for individuals with at least one vaccine dose before the SARS-CoV-2 test (partial vaccination - aHR: 0.44, 95%CI: 0.30; 0.64, complete vaccination - aHR: 0.46, 95%CI: 0.2; 0.80). We did not find a significant difference between the severity of the COVID-19 episode and the risk of having cardiovascular events post-COVID-19. Conclusion: Our findings suggest a substantial burden of cardiovascular complications post-COVID-19, underscoring the need for health services to be prepared and commence screening and preventive measures for individuals at higher risk.
  • Giant Cell Arteritis With Central Nervous System Vasculitis Presenting As Binocular Diplopia and Ptosis due to Third Cranial Nerve Palsy
    Publication . Casanova Pinto, J; Costa, MG; Fernandes, B; Ramalheira, C
    We report the case of a woman in her 60s with no notable comorbidities presented with a four-week history of bilateral temporal headache, scalp tenderness, jaw claudication, and sporadic fever. She also reported binocular diplopia for the previous 24 hours. Examination revealed tender superficial temporal arteries (TA) and right-sided third cranial nerve palsy with ptosis. Laboratory tests showed elevated inflammation markers. Cranial and cervical computed tomography (CT) and CT angiography (CTA) were unremarkable. She was treated with a single dose of intravenous methylprednisolone, followed by oral prednisolone. Subsequent TA duplex ultrasound demonstrated artery wall thickness, and TA biopsy confirmed chronic inflammation with disruption of the internal elastic lamina, both consistent with giant cell arteritis. Cranial magnetic resonance imaging (MRI) revealed scattered punctate areas on T2-weighted fluid-attenuated inversion recovery (FLAIR) sequences, consistent with small-vessel vasculitis. Under corticosteroid treatment, the patient achieved full clinical remission at the four-month follow-up. This case illustrates an uncommon neurological presentation of giant cell arteritis with oculomotor nerve involvement, associated with findings suggestive of central nervous system vasculitis, and highlights the importance of early recognition and prompt corticosteroid treatment to prevent irreversible complications.
  • Lupus Enteritis: An Unusual Flare of Systemic Lupus Erythematosus
    Publication . Casanova Pinto, J; Costa, MG; Fernandes, B; Ramalheira, C
    A woman in her 20s with previously diagnosed systemic lupus erythematosus (SLE) presented with a year-long history of chronic watery diarrhea, significant weight loss, and additive symmetrical inflammatory polyarthritis. She had been receiving hydroxychloroquine, azathioprine, and low-dose prednisolone but had discontinued treatment shortly before admission due to persistent vomiting. Laboratory evaluation showed new-onset proteinuria, hypokalemia, hypomagnesemia, microcytic anemia, lymphopenia, low complement levels, and markedly elevated antinuclear and anti-double-stranded deoxyribonucleic acid (anti-dsDNA) antibodies. Imaging demonstrated diffuse mural thickening and submucosal edema of the small bowel, a large left pleural effusion, and peritoneal fluid. The patient was treated with intravenous methylprednisolone pulses, followed by high-dose oral prednisolone and hydroxychloroquine. Renal biopsy revealed class III lupus nephritis, leading to the initiation of mycophenolate mofetil (MMF). Under this immunosuppressive regimen, gastrointestinal symptoms resolved, pleural effusion regressed, and renal parameters progressively improved. This case illustrates lupus enteritis as a rare but clinically significant manifestation of SLE, presenting with chronic diarrhea in association with other systemic features, including inflammatory polyarthritis, hematological involvement, and lupus nephritis. This report emphasizes the importance of considering lupus enteritis in patients with SLE who present with persistent gastrointestinal symptoms and of early recognition and prompt initiation of appropriate immunosuppressive therapy to prevent severe complications such as ischemia and perforation.
  • Role of Stress as a Precipitating Factor of Creutzfeldt-Jakob Disease: A Case Series
    Publication . Relvas, R; Vasconcelos, C; Buinho, A; Monteiro, N; Castro Sousa, S
    Easily mistaken for multiple neurological and psychiatric pathologies, Creutzfeldt-Jakob disease (CJD) is still underdiagnosed, with a difficult, late, and often retrospective recognition. Furthermore, its pathophysiology is poorly understood, and to date, no intervention has proven effective in its prevention or associated morbidity and mortality. The authors present a series of two clinical cases that not only expose its natural history and challenging differential diagnosis but also warn of a potential impact of psychological and stressful factors in its predisposition and/or precipitation.
  • Recurrent optic neuropathy: a case of Harding’s disease?
    Publication . Miranda, M; Andrade, MM; Silva, MI; Sousa, SC; Carmona, C
  • Subacute Meningitis: A Rare Presentation of Brucellosis
    Publication . Duarte, M; Tiago, V; Sousa, R; Aldomiro, F
    A young male adult presented with a two-week-long history of myoarthralgia, vomiting, febrile sensation, holocranial headache with photophobia, neck stiffness, anorexia, and weight loss. He worked as a butcher in a local slaughterhouse, and two of his coworkers had recently been diagnosed with brucellosis. On his fourth visit to the Emergency Department (ED), fever was observed for the first time. He was admitted for subacute meningitis. A lumbar puncture revealed high protein concentration, low glucose concentration, and pleocytosis without predominance. He was started on empirical treatment for acute meningitis and neurobrucellosis with ceftriaxone, doxycycline, and rifampin. There was a complete symptom remission over one week. The etiological investigation yielded a positive cerebrospinal fluid (CSF) and serum Rose Bengal Tests (RBTs), as well as a positive anti-Brucella IgG titer, leading to a final diagnosis of neurobrucellosis. After nine months of antimicrobial therapy and two years of follow-up, the patient remained asymptomatic.
  • Epidemiological and Clinical Aspects of Cutaneous and Mucosal Leishmaniases in Portugal: Retrospective Analysis of Cases Diagnosed in Public Hospitals and Reported in the Literature between 2010 and 2020
    Publication . Rocha, R; Conceição, C; Gonçalves, L; Carvalho, AC; Maia, A; Martins, A; Carujo, A; Maio, A; Forra, C; Melita, C; Couto, D; Fernandes, D; Pereira, D; Leal, E; Sarmento, H; Sousa, I; Gonçalves, JP; Marinho, J; Vasconcelos, J; Cunha, J; Rodrigues, J; Silva, JM; Caley, L; Malheiro, L; Santos, L; Garcia, M; Cunha, M; Lima, M; Andrade, MM; Marques, M; Alpalhão, M; Silva, M; Ferraz, R; Soares, R; Fernandes, S; Llobet, S; Cruz, S; Guimarães, T; Branco, T; Robalo-Nunes, T; Almeida, V; Maia, C
    Leishmania infantum, a zoonotic vector-born parasite, is endemic in the Mediterranean region, presenting mostly as visceral (VL), but also as cutaneous (CL) and mucosal leishmaniasis (ML). This study aimed to describe the epidemiological and clinical aspects of the CL and ML cases diagnosed in mainland Portugal between 2010 and 2020. Collaboration was requested from every hospital of the Portuguese National Health System. Cases were screened through a search of diagnostic discharge codes or positive laboratory results for Leishmania infection. Simultaneously, a comprehensive literature search was performed. Descriptive statistics and hypothesis testing were performed using IBM® SPSS® Statistics. A total of 43 CL and 7 ML cases were identified, with a predominance of autochthonous cases (86%). In CL, immunosuppressed individuals constituted a significant proportion of patients (48%), and in this group, disseminated CL (22%) and simultaneous VL (54%) were common. In autochthonous cases, lesions, mostly papules/nodules (62%), were frequently observed on the head (48%). The approach to treatment was very heterogeneous. ML cases were all autochthonous, were diagnosed primarily in older immunosuppressed individuals, and were generally treated with liposomal amphotericin B. The findings suggest a need for enhanced surveillance and reporting, clinical awareness, and diagnostic capacity of these forms of leishmaniasis to mitigate underdiagnosis and improve patient outcomes. A holistic One Health approach is advocated to address the multifaceted challenges posed by leishmaniases in Portugal and beyond.
  • Treatment patterns and preferences of people living with HIV starting or switching antiretroviral therapy: Real-world evidence from Portugal
    Publication . Pedro, L; Zagalo, A; Tavares, R; Pacheco, P; Oliveira, J; Vaz Pinto, I; Serrão, R; Tavares, S; Brito, P; Maltez, F; Neves, I; Carvalho, A; Teófilo, E; Almeida, J; Lains, I
    Background: There is a lack of up-to-date real-life evidence on antiretroviral therapy (ART) strategies among people living with HIV (PLWH) in Portugal. This study aimed to describe the treatment strategy used in PLWH either initiating or switching ART. Methods: Non-interventional, cross-sectional, multicenter study carried out between December 2019 and October 2021 in Portugal. Results: A total of 237 PLWH were included in this study, 171 of whom were ART-experienced and 66 were ART-naïve. The study showed that triple regimens were the most common ART strategy and integrase strand transfer inhibitors-based therapy was the most frequently used therapeutic class in both ART-naïve and ART-experienced PLWH. Nevertheless, about a third of PLWH who started a triple regimen transitioned to a dual regimen. Patient-reported outcomes revealed high HIV literacy and similar ART preferences in both groups. Conclusions: This real-world study showed that triple regimens were the most widely used ART strategy, even after the European AIDS Clinical Society guidelines introduced the recommendation of a dual regimen for naïve patients. The cohorts of this study presented a high level of HIV literacy at the time of inclusion. Our findings highlighted that taking pills only once a day is considered a very important feature for most patients.
  • Thrombocytopenia induced by clopidogrel: a rare adverse effect
    Publication . Cruz, D; Rodrigues dos Santos, J; Távora, C
    Introduction: Immune thrombocytopenic purpura (ITP) can be induced by several drugs but there are few case reports of ITP induced by clopidogrel. Second-line treatment with thrombopoietin receptor agonists (TPO-RA) presents solid evidence and should be considered in patients in need of elective surgery who are poor responders to steroids. Case description: We report the case of a 79-year-old male who developed severe immune thrombocytopenic purpura after initiating treatment with clopidogrel. Because he needed elective orthopaedic surgery and he did not respond to corticotherapy and immunoglobulin, second-line treatment with romiplostim was initiated with a significant increase in platelet count. Discussion and conclusion: Clopidogrel can induce ITP and this diagnosis should be considered in patients who present with isolated thrombocytopenia. First-line therapy of ITP is not always successful; second-line treatment with TPO-RA has a high response rate and should be considered in patients in need of elective surgery who have failed to respond to first-line therapy. Learning points: Clopidogrel can cause immune thrombocytopenic purpura (ITP); although there are some published cases in literature, it is a rare adverse effect.ITP induced by clopidogrel should be considered in the differential diagnosis of patients experiencing isolated thrombocytopenia.Second-line treatment of ITP with thrombopoietin receptor agonists (TPO-RA) presents solid evidence and should be considered in patients in need of elective surgery who are poor responders to steroids.