Influence of contextual factors and reliability of ultrasound skin measures in persons with systemic sclerosis and healthy controls.

OBJECTIVES: To examine the influence of contextual factors upon the evaluation of skin thickness and stiffness by ultrasound and to assess the reliability of these parameters. METHODS: Ultrasound dermal thickness (by B-mode, 18MHz) and skin stiffness (by shear-wave elastography, 9MHz) were assessed in persons with systemic sclerosis (SSc) and in healthy controls. The influence of contextual factors upon repeated measures was evaluated: (i) room temperature (16-17ºC vs. 22-24ºC); (ii) time of day (morning vs. afternoon), and (iii) menstrual cycle phase (menstrual vs. ovulatory). Differences were analysed using the related-samples Wilcoxon signed-rank test. Inter- and intra-rater reliability of ultrasound skin thickness and stiffness were evaluated in the 17 skin Rodnan sites of 20 persons with SSc and 20 healthy controls, under stable contextual conditions. RESULTS: A significant increase in ultrasound dermal thickness was observed at the leg in the afternoon vs morning, in both patients and controls. Similar observations were made for skin stiffness at the leg (in SSc) and at the foot (in SSc and controls) in the afternoon. No significant changes were observed in association with room temperature and menstrual cycle. Intra- and inter-rater-reliability was good to excellent for ultrasound dermal thickness and stiffness, both in SSc and healthy controls. CONCLUSIONS: The timing of the ultrasound procedure within each day seems to influence the ultrasound measures at the legs and feet. Our study corroborates that ultrasound dermal thickness and skin stiffness are reliable domains to quantify skin involvement in SSc.

Intravenous immunoglobulin (IVIG) in the vanguard therapy of Systemic Sclerosis.

Systemic Sclerosis (SSc) is a rare autoimmune disease that is characterized by a progressive skin fibrosis, an obliteration of the microvasculature and an exaggerated extracellular matrix deposition, which lead to a multisystemic dysfunction. Various pathogenetic mechanisms were described. The lack of a successful therapy make SSc a disease with a poor prognosis. The intravenous immunoglobulin (IVIG) has been used for a long time in different autoimmune diseases, and firstly used in SSc patients in 2000. IVIG has multiple non-specific mechanisms of action and, beyond an impressive improvement in muscle symptoms, a French nationwide cohort demonstrated that IVIG ameliorates the skin disease and systemic inflammation, and helps the daily dose corticosteroid's tapering at the end of the treatment. The benefits on gastrointestinal symptoms of IVIG was reported by a recent English article, in which the patients consistently reported a decrease in the gastro-esophageal reflux disease symptoms and their frequencies. The impact on the lung involvement still remains unclear. One of the advantages of IVIG is its safe profile. Few adverse effects were reported and most of them are mild, and can be managed and usually they do not relapse. Harmful effects were described, but they can be avoid with cautious and judicious use of this therapy.

Orthorexia nervosa in a sample of Portuguese fitness participants.

PURPOSE: Orthorexia Nervosa (ON) is described as an obsessive behaviour motivated by great concern for following a healthy diet. Although it was first described in 1997, ON remains inconspicuous and poorly characterized in the literature. The aims of this study were to evaluate the prevalence of ON in a sample of ambulatory individuals and to investigate their common characteristics, attempting to better characterize this entity. METHODS: A non-random sample of 193 adult gym members answered a questionnaire based on a Portuguese version of ORTO-15, supplemented with additional questions. Possible associations between the different variables and pathological scores on ORTO-15 were analysed using logistic regression models. RESULTS: ON behaviour was documented in 51.8% of the sample, mean age 30.96 years (± 1.03 years), and this condition tended to be correlated with younger ages. Physical appearance and frequent exercising were also associated with ON, while no correlation between orthorexic tendencies and both gender and educational field was found. Furthermore, consumption of special food, dissatisfaction with physical appearance and frequent exercising could predict the presence of ON in the sample. CONCLUSIONS: Despite the use of a convenience sample, results place us in a different perspective, considering the idea that orthorexic individuals are exclusively concerned about pursuing a pure diet. The presence of ON was associated with other non-dietary behaviours allied to a healthy lifestyle and aesthetic concerns. LEVEL OF EVIDENCE: Level V: Descriptive study.

Löfgren Syndrome: A Case Report and Treatment Challenge.

Sarcoidosis is an inflammatory and immune-mediated multisystemic disorder of unknown etiology, characterized by the presence of non-caseating granulomas, impacting various organs. This indolent condition manifests with numerous nonspecific symptoms and lacks a definitive diagnostic test, typically requiring histopathologic confirmation. However, a distinct and more readily diagnosable form of sarcoidosis does exist. The Löfgren syndrome (LöS) is characterized by the triad of erythema nodosum (EN), bilateral hilar lymphadenopathy, and symmetrical inflammatory arthralgias or arthritis. The simultaneous presence of these elements obviates the necessity for a biopsy. Predominantly affecting women in their second and third decades of life, this syndrome generally carries a favorable prognosis with spontaneous resolution or the requirement for a nonsteroidal anti-inflammatory drug (NSAID) alone. Despite its rarity, in particular cases, the treatment can be more challenging. This article presents a case study of LöS in a young woman, whose more aggressive disease course led to the need for steroidal therapy.

Exercise-induced pulmonary hypertension in scleroderma patients: a common finding but with elusive pathophysiology.

BACKGROUND: The etiology of exercise-induced pulmonary hypertension (exPH) in systemic sclerosis (SSc) remains a complex task, as both left ventricle (LV) diastolic dysfunction and pulmonary vascular disease can contribute to its development. We determined the incidence of exPH in SSc and examined the association between pulmonary artery systolic pressure (PASP) and tissue Doppler-derived indexes of pulmonary capillary wedge pressure (PCWP). METHODS: Thirty-eight patients with SSc were studied, using a cycloergometer protocol; 10 were excluded due to resting PH or absence of tricuspid regurgitation (TR); TR and mitral E-wave velocities, LV outflow tract time-velocity integral and LV septal E'-wave were measured before and in peak exercise to calculate cardiac output (CO), PCWP and pulmonary vascular resistance (PVR). RESULTS: Mean age of diagnosis was 57.9 ± 8.9 years. At a mean workload of 64 ± 29 Watts, 48% of patients increased PASP ≥ 50 mmHg. PCWP, assessed by the E/e' ratio, did not change significantly during exercise (10.2 ± 3.1-10.0 ± 5.1; P = NS). Only 3 patients had elevations of the E/e' ratio ≥ 13 during exercise; 2 of them had an exercise PASP ≥ 50 mmHg, yielding a proportion of exPH due to elevated LV filling pressures of 2/11 (18%). Patients with exPH had lower DLCO and had more frequently the diffuse SSc. CONCLUSION: The elevation of PASP during exercise in most patients of this cohort seems to be related to a reduced pulmonary vascular reserve, and not to an increase in PCWP. Further studies are warranted to determine the therapeutic, as well as prognostic implications of these findings.

Advances in analytical methods to study cholesterol metabolism: the determination of serum noncholesterol sterols.

Cholesterol biosynthesis precursors and plant sterols are noncholesterol sterols currently used as relative surrogate markers of cholesterol synthesis and absorption, respectively. Its determination in serum samples is a way of diagnosing inherited disorders and also a tool for health evaluation during lipid-lowering lifestyle/drug therapy monitoring. This approach is the only one that can be used for large-scale clinical trials or population based studies, but, nevertheless, there is no reference method for the quantification of noncholesterol sterols in human serum samples and only analysis by GC-FID and GC-MS has been reported to be completely validated. Although there has been a wider use of noncholesterol sterols for the measurement and characterization of cholesterol metabolism, there is a lack of harmonization of measurements and of standardization of the methodology, which is essential for routine measurements of diagnostic utility. New recent advances in analytical methods for the determination of serum noncholesterol sterols are highlighted, focusing on the sample preparation, separation and detection techniques, which will enhance the range of applications in clinical practice.

Normocomplementemic urticarial vasculitis secondary to Lyme disease: A rare association with challenging treatment.

Urticarial vasculitis (UV) is a rare entity characterised by long-lasting recurrent episodes of urticarial lesions. Although frequently idiopathic, UV has been associated with multiple diseases, including infections. We present a case of Lyme disease (LD) as a trigger of normocomplementemic UV, a very rarely described association. The patient presented first with episodes of inflammatory polyarthritis and a positive serology for Borrelia burgdorferi, later followed by the appearance of long-lasting urticarial lesions, histologically suggestive of UV. Lyme arthritis resolved with doxycycline, but UV persisted. Response to cyclosporine was satisfactory but with side effects, and only methotrexate showed substantial and consistent improvement. This case reminds physicians that chronic urticaria with atypical characteristics should raise suspicion of UV. Possible triggers for this disease must be sought, even if rarely described, such as LD. Normocomplementemic UV frequently presents a therapeutic challenge, but methotrexate can be a particularly effective therapy in this setting.

Rare manifestations of refractory extrapulmonary sarcoidosis.

Sarcoidosis is a multisystemic disease, characterised by histopathologic presence of non-necrotising granulomas, primarily affecting the lungs (>90%). We present three cases predominantly characterised by uncommon extrapulmonary manifestations of sarcoidosis, where only one had pulmonary involvement. We describe three female patients, between the second and third decades of life, whose sarcoidosis was a diagnostic challenge due to their atypical extrapulmonary manifestations, from which we highlight: livedo reticularis and painful subcutaneous nodules with uncommon localisation, size and histology, being the first reported case of extensive subcutaneous nodules triggered by intramuscular penicillin; extensive symptomatic and refractory osseous involvement; and dispersed erythema nodosum affecting the entire body surface. All three patients required third-line treatment (antitumour necrosis factor agents) to achieve significant clinical and imagiological improvement. Through this case series, we highlight the importance of considering the rare and atypical presentations of sarcoidosis to avoid diagnostic delays and serious repercussions on the patient's prognosis.

Asymptomatic Elevation of Pancreatic Enzymes: A Case of Gullo's Syndrome.

Benign pancreatic hyperenzymemia (BPH) or Gullo's syndrome is a benign condition consisting of an oscillating elevation of pancreatic enzymes without the identification of pancreatic disease. Its diagnosis is usually incidental and by excluding other conditions that occur with elevated pancreatic enzymes. To the best of our knowledge, there are no reports of this diagnosis to this day in Portugal. A 65-year-old female was referred to an internal medicine consultation for complaints of xerostomia, xerophthalmia, and xeroderma with one year of evolution. From the study carried out by the patient prior to the consultation, an incidental elevation of amylase stands out. The sicca symptoms were attributed to sertraline since, after excluding other causes, its discontinuation resolved the symptoms. Regarding the elevation of pancreatic enzymes, the patient underwent an extensive diagnostic study for clarification without identifying any condition. The serial measurement of amylase and lipase revealed an oscillating increase in pancreatic enzymes with temporary normalization. After one year of follow-up, the diagnosis of Gullo's syndrome was established. The identification of Gullo's syndrome is extremely important as it avoids carrying out unnecessary tests in the future and allows the patient to be reassured in the face of this benign alteration of pancreatic enzymes. A follow-up of at least one year is crucial since some pancreatic tumors course with an asymptomatic increase in pancreatic enzymes.

Solvent-Induced Raynaud's Phenomenon.

Raynaud's phenomenon (RP) is a vasospastic disorder characterized by an exaggerated vasoconstrictive response to cold or emotional stress. It can be classified as primary (PRP) or secondary (SRP) depending on its association with an underlying condition. We present a case of a young female with severe RP, with trophic changes and abnormal capillaroscopy. After a detailed investigation, a diagnosis of secondary RP due to solvent exposure was made. The patient was treated with calcium channel blocker in low doses, due to hypotension, without improvement of symptoms. Vitamin C and pentoxifylline were added with an unsatisfactory response. Given the progressive worsening of RP and the appearance of trophic lesions, the patient's treatment was reviewed, and continuous intravenous iloprost infusion through an elastomeric pump was started. This resulted in significant symptom improvement and normalization on the capillaroscopic examination.

Primary Cardiac Tumor in the Left Atrium: A Diagnostic Challenge.

An elderly man presented to the emergency department with shortness of breath, peripheral edema, and significant weight loss. Blood tests revealed anemia and elevated inflammatory markers, and chest imaging showed a massive left pleural effusion. During hospitalization, he developed subacute cardiac tamponade, and pericardiocentesis was performed. Further imaging revealed a primary malignant cardiac tumor with extensive infiltration of the cardiac tissue, and biopsy was deemed impossible due to the tumor's location. The most likely diagnosis was angiosarcoma. The cardiac surgery team evaluated the case and considered it inoperable due to the tumor's extensive infiltration. The patient is currently under the regular care of a palliative care team. This case underscores the difficulties of diagnosing primary cardiac tumors, particularly in elderly patients with comorbidities. Despite advances in imaging and surgical techniques, the prognosis for malignant cardiac tumors remains poor.

Acute kidney injury: a strong risk factor for hypoglycaemia in hospitalized patients with type 2 diabetes.

AIMS: Acute kidney injury (AKI) is highly prevalent during hospitalization of patients with type 2 diabetes (T2D). We aimed to assess the impact of AKI and its severity and duration on the risk of hypoglycaemia in hospitalized patients with T2D. METHODS: Retrospective cohort analysis of patients with T2D, admitted at a University Hospital in 2018-2019. AKI was defined as an increase in serum creatinine by ≥ 0.3 mg/dl (48 h) or ≥ 1.5 times baseline (7 days), and hypoglycaemia as blood glucose concentration < 70 mg/dl. Patients with chronic kidney disease stage ≥ 4 were excluded. We registered 239 hospitalizations with AKI and randomly selected 239 without AKI (control). Multiple logistic regression was used to adjust for confounding factors and ROC curve analysis to determine a cutoff for AKI duration. RESULTS: The risk of hypoglycaemia was higher in the AKI group (crude OR 3.6, 95%CI 1.8-9.6), even after adjusting for covariates (OR 4.2, 95%CI 1.8-9.6). Each day of AKI duration was associated with a 14% increase in the risk of hypoglycaemia (95%CI 1.1-1.2), and a cutoff of 5.5 days of AKI duration was obtained for increased risk of hypoglycaemia and mortality. AKI severity was also associated with mortality, but showed no significant association with hypoglycaemia. Patients with hypoglycaemia had 4.4 times greater risk of mortality (95%CI 2.4-8.2). CONCLUSIONS: AKI increased the risk of hypoglycaemia during hospitalization of patients with T2D, and its duration was the main risk factor. These results highlight the need for specific protocols to avoid hypoglycaemia and its burden in patients with AKI.

Morvan's Syndrome: The Importance of Knowing Different Risk-Associated Phenotypes and Antibodies in Identifying the Correct Underlying Tumor.

Paraneoplastic neurologic syndromes (PNS) are neurologic disorders that can affect any part of the nervous system, occur in association with cancer, and have an immune-mediated mechanism that produces direct damage to the neural tissue. Neurological symptoms frequently precede, in months to years, the symptoms directly attributed to the primary tumor, requiring a high clinical suspicion for adequate investigation. We report the case of a man in his early 80s admitted for an altered level of consciousness, alternating between periods with stupor and drowsiness, short-term waking states and psychomotor agitation, respiratory failure and dysautonomia, resembling a Morvan's syndrome. Anti-leucine-rich glioma-inactivated 1 and anti-contactin-associated protein-like 2 antibodies were both positive and, after exclusion of infectious and autoimmune systemic causes, the possibility of PNS was raised. Screening for the primary tumor was pursued, and an 18F-fluorodeoxyglucose (18F-FDG)/PET showed only an intensely hypermetabolic, apparent parietal thickening of the lower rectum. Due to the frequent association of Morvan's syndrome to thymoma, a review of the CT of the thorax images was requested and a mediastinal image with features of thymoma was identified. PNS treatment and prognosis depend on finding and treating the underlying tumor, with benefits in both resolution of neurological symptoms and in the prognosis of the underlying tumor itself. Therefore, clinicians should be aware of this frequent but underdiagnosed and underreported condition, in order to improve the chances of better outcomes.

Host-derived mannose glycans trigger a pathogenic γδ T cell/IL-17a axis in autoimmunity.

Autoimmune diseases are life-threatening disorders that cause increasing disability over time. Systemic lupus erythematosus (SLE) and other autoimmune diseases arise when immune stimuli override mechanisms of self-tolerance. Accumulating evidence has demonstrated that protein glycosylation is substantially altered in autoimmune disease development, but the mechanisms by which glycans trigger these autoreactive immune responses are still largely unclear. In this study, we found that presence of microbial-associated mannose structures at the surface of the kidney triggers the recognition of DC-SIGN-expressing γδ T cells, inducing a pathogenic interleukin-17a (IL-17a)-mediated autoimmune response. Mice lacking Mgat5, which have a higher abundance of mannose structures in the kidney, displayed increased γδ T cell infiltration into the kidney that was associated with spontaneous development of lupus in older mice. N-acetylglucosamine supplementation, which promoted biosynthesis of tolerogenic branched N-glycans in the kidney, was found to inhibit γδ T cell infiltration and control disease development. Together, this work reveals a mannose-γδ T cell-IL-17a axis in SLE immunopathogenesis and highlights glycometabolic reprogramming as a therapeutic strategy for autoimmune disease treatment.

Biological therapy in systemic lupus erythematosus, antiphospholipid syndrome, and Sjögren's syndrome: evidence- and practice-based guidance.

Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and Sjögren's syndrome (SS) are heterogeneous autoimmune diseases. Severe manifestations and refractory/intolerance to conventional immunosuppressants demand other options, namely biological drugs, and small molecules. We aimed to define evidence and practice-based guidance for the off-label use of biologics in SLE, APS, and SS. Recommendations were made by an independent expert panel, following a comprehensive literature review and two consensus rounds. The panel included 17 internal medicine experts with recognized practice in autoimmune disease management. The literature review was systematic from 2014 until 2019 and later updated by cross-reference checking and experts' input until 2021. Preliminary recommendations were drafted by working groups for each disease. A revision meeting with all experts anticipated the consensus meeting held in June 2021. All experts voted (agree, disagree, neither agree nor disagree) during two rounds, and recommendations with at least 75% agreement were approved. A total of 32 final recommendations (20 for SLE treatment, 5 for APS, and 7 for SS) were approved by the experts. These recommendations consider organ involvement, manifestations, severity, and response to previous treatments. In these three autoimmune diseases, most recommendations refer to rituximab, which aligns with the higher number of studies and clinical experience with this biological agent. Belimumab sequential treatment after rituximab may also be used in severe cases of SLE and SS. Second-line therapy with baricitinib, bortezomib, eculizumab, secukinumab, or tocilizumab can be considered in SLE-specific manifestations. These evidence and practice-based recommendations may support treatment decision and, ultimately, improve the outcome of patients living with SLE, APS, or SS.

Biologic therapy in large and small vessels vasculitis, and Behçet's disease: Evidence- and practice-based guidance.

OBJECTIVE: Vasculitis are a very heterogenous group of systemic autoimmune diseases, affecting large vessels (LVV), small vessels or presenting as a multisystemic variable vessel vasculitis. We aimed to define evidence and practice-based recommendations for the use of biologics in large and small vessels vasculitis, and Behçet's disease (BD). METHODS: Recommendations were made by an independent expert panel, following a comprehensive literature review and two consensus rounds. The panel included 17 internal medicine experts with recognized practice on autoimmune diseases management. The literature review was systematic from 2014 until 2019 and later updated by cross-reference checking and experts' input until 2022. Preliminary recommendations were drafted by working groups for each disease and voted in two rounds, in June and September 2021. Recommendations with at least 75% agreement were approved. RESULTS: A total of 32 final recommendations (10 for LVV treatment, 7 for small vessels vasculitis and 15 for BD) were approved by the experts and several biologic drugs were considered with different supporting evidence. Among LVV treatment options, tocilizumab presents the higher level of supporting evidence. Rituximab is recommended for treatment of severe/refractory cryoglobulinemic vasculitis. Infliximab and adalimumab are most recommended in treatment of severe/refractory BD manifestations. Other biologic drugs can be considered is specific presentations. CONCLUSION: These evidence and practice-based recommendations are a contribute to treatment decision and may, ultimately, improve the outcome of patients living with these conditions.

An update on potato crisps contents of moisture, fat, salt and fatty acids (including trans-fatty acids) with special emphasis on new oils/fats used for frying.

Eighteen brands of potato crisps, frequently consumed, were analyzed to establish their nutritional value in relation to salt, fat and fatty acid (FA) composition. The purpose of the present study was to determine moisture, total fat, salt contents and FA profiles (including trans-FAs), and to identify the oil/fat used for frying of the 18 brands of potato crisps. Our results show that salt content ranged from 0.127 to 2.77 g/100 g and total fat content of potato crisps varied between 20.0 and 42.8 g/100 g. With respect to FAs analysis, palmitic acid (C16:0), oleic acid (C18:1) and linoleic acid (C18:2) were the major FAs found in the analyzed potato crisps. It is clear from our work that nowadays most potato crisps are currently produced using oils with high contents in unsaturated FAs, which can be considered as healthier from a nutritional point of view. Nevertheless, some brands of potato crisps still use palm oil or a blend of palm oil and other fats/oils, which are very rich in saturated FAs.

The impact of nutrition and lifestyle modification on health.

The main recommendations from public health entities include healthy diets and physical activity as the main lifestyle factors impacting the development of chronic diseases such as cardiovascular and metabolic diseases, cancer, and even neurological diseases. Randomized clinical trials have been designed to demonstrate those lifestyle modifications can change the pattern of chronic diseases development and progression. Among these, nutrition is one of the most impacting factors. Therefore, nutrition and diets were also included in different randomized clinical trials, and most of them showed a favorable impact of nutrition modification on the participant's health. Nevertheless, study designs were considerably different, and future studies are needed to support nutrition modifications further. The choice of a healthy considered diet, like the Mediterranean diet, was shown to impact chronic diseases, cardiovascular risk, and adult life expectancy mainly due to its anti-inflammatory and antioxidant properties. Furthermore, a high intake of fibers, fruits, and vegetables together with a low intake of fat and energy-dense, processed foods contribute to an inflammation reduction and a more robust immune system leading. Besides these well-known properties, all lifestyle modifications must be personalized according to the availability of foods, geographic localizations, and the healthy status of the patient.

Spontaneous Bacterial Peritonitis in Cirrhotic Patients: A Shift in the Microbial Pattern? A Retrospective Analysis.

Introduction: Over the last decade, a shift in the spontaneous bacterial peritonitis (SBP) microbial pattern toward an increasing incidence of gram-positive and multidrug-resistant (MDR) bacteria has been reported. Systematic surveillance of the local microbiological scenario and antibiotic resistance is crucial to SBP treatment success. The main objective of this study was to evaluate the microbiological profile and bacterial resistance of SBP pathogens in a Portuguese cohort to allow selection of the most appropriate empirical antibiotics. Methods: This is a single-center retrospective study including 63 adult cirrhotic patients with culture-positive SBP. Patients were identified using a hospital general diagnostic database and searching for all SBP events (neutrophil count in ascitic fluid ≥250/mm3) from January 1, 2012, to December 31, 2017. Patients were excluded if they had culture-negative SBP, secondary peritonitis, peritoneal dialysis, a liver transplant, or immunodeficiency. The site of SBP acquisition was classified as nosocomial if it was diagnosed 48 h or longer after hospitalization or as nonnosocomial if it was diagnosed within the first 48 h. MDR bacteria were those with an acquired resistance to at least 1 agent in 3 or more antimicrobial categories. All statistical analyses were carried out using IBM SPSS Statistics software version 22 (IBM, New York, USA). Results: The study cohort comprised 53 (84.1%) men. The mean age of the patients was 60.6 ± 11.2 years. Alcohol was the most common etiology (88.9%) and most patients had advanced liver cirrhosis (87.1%, Child C). Gram-negative bacteria were slightly more frequent than gram-positive bacteria (56.9 vs. 43.1%). Escherichia coli was the most common pathogen (33.8%). Nineteen (31.7%) bacteria were classified as MDR. Resistance to third-generation cephalosporins, quinolones, piperacillin-tazobactam, and carbapenems was found in 31.7, 35, 26.7, and 18.3% of the cases, respectively. The rates of gram-positive bacteria were similar between nosocomial and nonnosocomial episodes (45 vs. 42.2%; p = 0.835). MDR bacteria were more common in the nosocomial group (50 vs. 23.8%; p = 0.046). Resistance to third-generation cephalosporins (50 vs. 23.8%; p = 0.046), piperacillin-tazobactam (44.4 vs. 19.1%; p = 0.041), and carbapenems (33.3 vs. 11.9%; p = 0.049) occurred more frequently in nosocomial episodes. Resistance to first-line antibiotic occurred in 29.3% of the patients, being more common in the nosocomial group (44.4 vs. 22.5%; p = 0.089). Conclusion: Although gram-negative bacteria remain the most common causative microorganisms, our results emphasize the shift in SBP microbiological etiology, as almost half of the isolated microorganisms were gram positive. The emergence of bacteria resistant to traditionally recommended empirical antibiotics underlines the importance of basing this choice on local flora and antibiotic susceptibility data, allowing a more rational and successful use of antibiotics.

Introdução: Na última década assistiu-se a uma mudança no padrão microbiológico da peritonite bacteriana espontânea (PBE), com aumento da incidência de bactérias gram-positivas e multirresistentes. Uma vigilância sistemática do cenário microbiológico e da resistência antibiótica é crucial para o sucesso do tratamento da PBE. O principal objetivo deste estudo foi avaliar o perfil microbiológico e os padrões de resistência antibiótica dos agentes bacterianos responsáveis pelos casos de PBE numa coorte portuguesa de doentes cirróticos, de modo a permitir uma seleção mais apropriada da antibioterapia empírica. Métodos: Estudo retrospetivo unicêntrico, que incluiu 63 doentes adultos cirróticos com PBE culturapositiva. A identificação dos doentes foi efetuada a partir da base de dados eletrónica do centro hospitalar, pesquisando todos os internamentos por PBE (contagem de neutrófilos no líquido ascítico ≥250/mm3) entre 1 janeiro de 2012 e 31 de dezembro de 2017. Foram aplicados como critérios de exclusão: cultura de líquido ascítico negativa, peritonite secundária, diálise peritoneal, transplante hepático ou imunodeficiência. O local de aquisição da PBE foi classificado como nosocomial se diagnóstico após as primeiras 48 horas de hospitalização, e não-nosocomial se diagnóstico nas primeiras 48 horas. Multirresistência foi definida como resistência adquirida a pelo menos um agente em três ou mais categorias antimicrobianas. A análise estatística foi efetuada com recurso ao software IBM SPSS Statistics versão 22 (IBM, New York, USA). Resultados: A coorte incluiu 53 (84.1%) doentes cirróticos do género masculino. A idade média dos doentes foi de 60.6 ± 11.2 anos. O álcool constituiu a etiologia mais comum (88.9%) e a maioria dos doentes apresentava uma cirrose em estadio avançado (87.1%, Child-C). As bactérias gram-negativas revelaram-se ligeiramente mais frequentes que as gram-positivas (56.9 vs. 43.1%). Escherichia coli foi o microrganismo mais frequente (33.8%). Dezanove (31.7%) das bactérias isoladas foram classificadas como multirresistentes. As resistências às cefalosporinas de terceira-geração, às quinolonas, à piperacilina-tazobactam e aos carbapenemes foram de 31.7, 35, 26.7 e 18.3%, respetivamente. A taxa de bactérias gram-positivas foi similar entre PBE nosocomial e não-nosocomial (45 vs. 42.2%; p = 0.835). As bactérias multirresistentes foram mais frequentes no grupo nosocomial (50 vs. 23.8%; p = 0.046). A resistência às cefalosporinas de terceira-geração (50 vs. 23.8%; p = 0.046), à piperacilina-tazobactam (44.4 vs. 19.1%; p = 0.041) e aos carbapenemes (33.3 vs. 11.9%; p = 0.049) foi significativamente superior nas infeções nosocomiais. A resistência à antibioterapia empírica de primeira linha ocorreu em 29.3% dos doentes, sendo mais frequente no grupo nosocomial (44.4 vs. 22.5%; p = 0.089). Conclusão: Apesar de as bactérias gram-negativas constituírem, nesta coorte, a maioria dos microrganismos responsáveis pela PBE, os nossos resultados enfatizam a mudança na etiologia microbiológica da PBE, na medida em que, quase metade dos microrganismos isolados foram gram-positivos. A emergência de bactérias resistentes aos antibióticos empíricos tradicionalmente recomendados sublinha a importância de basear esta escolha nos dados locais sobre flora bacteriana e susceptibilidade antibiótica, permitindo uma escolha mais racional e um uso bem-sucedido dos antibióticos.

Lymphocyte-Depleted Hodgkin's Lymphoma in Coeliac Disease: A Diagnostic Challenge During the COVID-19 Pandemic.

A 51-year-old woman presented with constitutional symptoms, polydipsia, early satiety, nausea, vomiting, and a pruritic vesicular rash. On physical examination, she was febrile, had low peripheral oxygen saturation in room air (91%), hepatomegaly, lower limb edema, and palpable cervical adenopathies. She was hospitalized for diagnostic investigations and treatment. An autoimmune panel was requested which was positive for anti-parietal gastric cell, anti-endomysial, and anti-tissue transglutaminase antibodies, raising the suspicion for coeliac disease (CD). Gastric and duodenal biopsies were not diagnostic for CD, which was compatible with necrolytic migratory erythema similar to the vesicular rash biopsy. Thoracic-abdomino-pelvic computed tomography scan and fludeoxyglucose F18-positron emission tomography identified supra- and infra-diaphragmatic hypermetabolic adenopathies, with hypermetabolic activity in the lungs, suggestive of pulmonary lymphomatous involvement. Fine-needle aspiration of one supraclavicular adenopathy was performed but was not enough for histological diagnosis. The patient's respiratory insufficiency worsened and she died on day 63 of hospitalization. The final diagnosis was achieved on an anatomopathological autopsy that showed lymphocyte-depleted Hodgkin's lymphoma. The association of CD with other lymphomas besides enteropathy-type T-cell lymphoma is not clear. There is no clear relationship between CD and lymphocyte-depleted Hodgkin's lymphoma, which is the rarest subtype of classic Hodgkin's lymphoma and, by itself, has a very poor prognosis. This case highlights the challenge in diagnosis and significant delay due to isolation associated with coronavirus disease 2019 infection.