Validation of a disease-specific health-related quality of life measure in adult Portuguese patients with systemic lupus erythematosus: LupusQoL-PT.

INTRODUCTION: LupusQoL is a questionnaire specifically designed to assess health-related quality of life (HRQoL) in SLE patients. We report on the translation and cross-cultural adaptation of LupusQoL into European Portuguese. METHODS: Translation and cultural adaptation were performed according to standard protocol described by the original developers. LupusQoL-PT was administered to patients during a routine visit from an outpatient clinic at a university hospital in Portugal. Content structure was validated using factorial analysis. Cronbach's alpha coefficient was computed for internal consistency. Sociodemographic were questioned during the visit and clinical data were collected during the visit and from the clinical files. Pearson's correlation, T-test, Mann-Whitney and one-way ANOVA were applied to test internal and external validation. RESULTS: Seventy-nine SLE patients (78 woman: 1 man) were evaluated. Most had Low disease activity (mean SLEDAI-2k = 3.49; standard deviation 4.80), 19% had moderate to severe activity and 38% had damage accrual (mean SDI = 0.75; standard deviation 1.05). Cronbach's alpha coefficient was at least 0.812, confirming good internal consistency. Correlation coefficient and test-retest correlation between the eight domains of LupusQoL-PT were strong in almost every domain (p < 0.01). External convergent analysis showed strong correlation between LupusQoL-PT and Medical Outcome Study Short Form 36-item version 2 and visual analogic scale. Current disease activity was negative correlated with "Body Image" domain. There was no correlation between other LupusQoL-PT domains and SLEDAI-2k on divergent validity. Patients with previous neuropsychiatric and DMARDs treatment had lower HRQoL in emotional domains, while patients with renal damage accrual had HRQoL impact in both physical and emotional domains. Portuguese SLE patients had lower HRQoL than French and Italian validation cohorts' patients, and higher than Spanish cohorts. CONCLUSION: LupusQoL-PT has shown adequate metric properties and should be considered an appropriate tool to evaluate HRQoL in Portuguese SLE patients.

Campylobacter jejuni Pericarditis: A Case Report.

Campylobacter jejuni is one of the most common causes of enteritis. In rare cases, extraintestinal infection can occur, with a handful of cases of cardiac involvement, of which the pathophysiological mechanism is unclear. We report a case of pericarditis in a patient with X-linked agammaglobulinemia presenting with chronic diarrhea and chest pain who evolved to cardiac tamponade, requiring a pericardial window and a long course of broad-spectrum antibiotics. To the best of our knowledge, this is the third case of pericarditis caused by Campylobacter jejuni reported in the literature, the second in a patient with X-linked agammaglobulinemia. Despite its rarity, this case serves as a reminder of Campylobacter as a potential cause of cardiac inflammation for clinicians treating pericarditis/myocarditis, especially in patients with a history of diarrhea or immunosuppression.

A Campylobacter jejuni é uma das causas mais comuns de enterite. A infeção extraintestinal pode ocorrer raramente, estando reportados alguns casos de atingimento cardíaco, de mecanismo fisiopatológico incerto. Reportamos um caso de pericardite num doente com agamaglobulinemia ligada ao X, que se apresentou como diarreia crónica e dor torácica, evoluindo para tamponamento cardíaco com necessidade de confeção de janela pericárdica e tratamento prolongado com antibióticos de largo espectro. Este é, tanto quanto é do nosso conhecimento, o terceiro caso de pericardite por Campylobacter jejuni reportado na literatura, o segundo em doente com agamaglobulinemia ligada ao X. Apesar da sua raridade, este caso serve para reforçar a importância do género Campylobacter como causa de inflamação cardíaca para médicos que tratem pericardite/miocardite, especialmente em doentes com história de diarreia ou imunossupressão.

Case Report: Anaphylactoid Shock Associated with Snakebite Envenoming in Portugal.

Although rare in Portugal, snakebite envenoming entails severe morbidity and mortality. We present the case of a 65-year-old woman bitten on her leg in a northern coastal region in Portugal, on a walk during the COVID-19 pandemic lockdown. Despite first looking for help at the nearest pharmacy, she developed anaphylactoid shock and was promptly driven to a tertiary hospital, where antivenom was administered in a timely manner under close monitoring. Prophylactic antibiotics were started and maintained based on elevated inflammatory markers and signs of wound inflammation. She evolved favorably, with rapid weaning of vasopressors and resolution of end-organ dysfunction. This case highlights the importance of prompt recognition and describes crucial steps in envenomation management in a country where snakebite is infrequent, but potentially fatal.

The Role of a Medical Intermediate Care Unit in the Management of Budd-Chiari Syndrome: Case Series.

Budd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). The therapeutic approach depends on disease severity and related etiology with patients with severe forms of presentation classically managed in intensive care units (ICUs). Here, we report a series of five BCS patients managed in a medical intermediate care unit (IntCU), with three of them presenting with acute liver injury. Progression to ALF was seen in three patients, two of whom died, with one being successfully submitted to liver transplantation. IntCUs allow a 24-h patient surveillance and a prompt management of BCS, with less economic impact when compared to ICUs. Mortality was related to the presence of associated comorbidities that limited therapeutic approach.

Ovarian teratoma: A case from 15th-18th century Lisbon, Portugal.

This paper discusses the differential diagnosis of an unusual calcified mass found in the pelvic cavity of 45+-year-old female excavated from 15th-18th century Lisbon (Portugal). The mass is relatively large, irregularly shaped, and exhibits a concave base with malformed teeth embedded within its inner surface. Considering its macroscopic and radiological characteristics, several conditions were considered in the differential diagnosis, namely eccyesis, fetus in fetu, lithopaedion, and ovarian teratoma. However, the morphological features of the specimen, such as its structure, morphology, and dimensions, are diagnostic of a teratoma. Its location and the sex of the individual are more specifically compatible with a calcified ovarian teratoma. With regional and temporal variations in the frequency of tumours, the report of new cases becomes imperative, especially from geographic regions where few cases have been identified. In fact, this appears to be the first case of ovarian teratoma detected in the Portuguese archaeological record and adds to the few palaeopathological cases described in the osteoarchaeological literature worldwide.

Subacute Hypophysitis with Panhypopituitarism as First Presentation of HIV and Syphilis Coinfection.

Infection by Treponema pallidum still represents a clinical challenge due to its various forms of presentation. HIV coinfection added diversity and changed the natural history of syphilis as a systemic infection. We present a rare case of subacute hypophysitis and panhypopituitarism due to an early active neurosyphilis in a previously unknown HIV coinfected patient.