Chorea as a Manifestation of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystemic manifestations, including central nervous system involvement. Chorea is a hyperkinetic movement disorder, characterized by involuntary, dance-like and poorly coordinated movements. Acute-onset chorea is a rare neuropsychiatric inaugural manifestation of SLE. This presentation is frequently associated with positive antiphospholipid antibodies, and it usually improves with immunosuppressive treatment. We report the case of a 20-year-old female, who presented with acute onset left hemichorea and fever. Analysis showed active urine sediment. A detailed anamnesis and evaluation revealed several clinical manifestations suggestive of SLE with multiorgan involvement: neurological, renal, cardiac, hematological, joint and mucocutaneous. This case emphasizes the importance of keeping a high clinical awareness for rarer presentations of common autoimmune disorders, such as SLE, which can be severe and should be promptly treated. Furthermore, the relevance of SLE in the differential diagnosis of acute-onset movement disorders in young patients is highlighted in this report.

Awareness of sex and gender dimensions among physicians: the European federation of internal medicine assessment of gender differences in Europe (EFIM-IMAGINE) survey.

Sociocultural gender is a complex construct encompassing different aspects of individuals' life, whereas sex refers to biological factors. These terms are often misused, although they impact differently on individuals' health. Recognizing the role of sex and gender on health status is fundamental in the pursuit of a personalized medicine. Aim of the current study was to investigate the awareness in approaching clinical and research questions on the impact of sex and gender on health among European internists. Clinicians affiliated with the European Federation of Internal Medicine from 33 countries participated to the study on a voluntary basis between January 1st, 2018 and July 31st, 2019. Internists' awareness and knowledge on sex and gender issues in clinical medicine were measured by an online anonymized 7-item survey. A total of 1323 European internists responded to the survey of which 57% were women, mostly young or middle-aged (78%), and practicing in public general medicine services (74.5%). The majority (79%) recognized that sex and gender are not interchangeable terms, though a wide discrepancy exists on what clinicians think sex and gender concepts incorporate. Biological sex and sociocultural gender were recognized as determinants of health mainly in cardiovascular and autoimmune/rheumatic diseases. Up to 80% of respondents acknowledged the low participation of female individuals in trials and more than 60% the lack of sex-specific clinical guidelines. Internists also express the willingness of getting more knowledge on the impact of sex and gender in cerebrovascular/cognitive and inflammatory bowel diseases. Biological sex and sociocultural gender are factors influencing health and disease. Although awareness and knowledge remain suboptimal across European internists, most acknowledge the underrepresentation of female subjects in trials, the lack of sex-specific guidelines and the need of being more informed on sex and gender-based differences in diseases.

The UriCath study: characterization of the use of indwelling urinary catheters among hospitalized older patients in the Internal Medicine Departments of Portugal.

PURPOSE: Approximately 25% of older inpatients have an indwelling urinary catheter (IUC), 45-54% unnecessarily. This study aims to describe the prevalence, indications, and complications of IUC use in Portuguese older inpatients. METHODS: Multicentric, cross-sectional, observational study conducted in Portuguese internal medicine wards (UriCath). RESULTS: Of a total of 3135 inpatients from 39 hospitals, we included 628 patients with 65 years old or more using an IUC, mean age 82.0 ± 7.5. Prevalence of IUC use was 20.0%. The average Barthel Index was 44.0 ± 37.3 and Charlson comorbidity Index was 7.0 ± 2.8. The main reasons for IUC use were: urinary output monitoring (47.5%), urinary retention (22.5%), and pressure ulcers (11.0%). The IUC removal was attempted in 9.1% and 24.7% developed a complication. CONCLUSION: IUC use among older inpatients is prevalent and often inappropriate. Clinical awareness and development guidelines for restricted use of IUC are essential to reduce morbimortality and healthcare costs.

Serum markers of B-cell activation in pregnancy during late gestation, delivery, and the postpartum period.

PROBLEM: B cells are vital for the normal evolution of pregnancy due to their humoral and possible regulatory activities. Our group and others have documented that circulating B-cell subsets undergo changes from normal late pregnancy to the postpartum period. However, the underlying mechanisms are poorly understood. Therefore, this study examined the degree of B-cell activation in normal pregnancy by analyzing the levels of serum markers in healthy pregnant women during the third trimester of pregnancy, the day of delivery, and the postpartum period. METHOD OF STUDY: A prospective study including pregnant and non-pregnant women attending routine care was undertaken at a hospital clinic. Sociodemographic and clinical data were collected, along with peripheral blood samples. The serum levels of soluble CD23 (sCD23), B-cell-activating factor (BAFF), kappa (κ) and lambda (λ) free light chains (FLC), IgA, IgG, and IgM were quantified. RESULTS: Our study included 43 third trimester pregnant and 35 non-pregnant women. In the pregnant women, the median levels of sCD23, BAFF, IgG, and κ FLC were significantly higher during the postpartum period than during the third trimester of pregnancy. Compared to the non-pregnant women, the third trimester pregnant women had higher median BAFF levels and lower sCD23, IgA, IgG, and FLC levels. CONCLUSION: Changes in serum markers of B-cell kinetics that occur during pregnancy often persist into the postpartum period and affect the secretion of immunoglobulins from different classes. Further studies are needed to clarify the biological significance of our observations.

Looking for the Perfect Mentor.

INTRODUCTION: The authors established the profile of the Internal Medicine clinical teachers in Portugal aiming to define a future interventional strategy plan as adequate as possible to the target group and to the problems identified by the residents. MATERIAL AND METHODS: Observational, transversal, analytic study. An online anonymous questionnaire was defined, evaluating the demographic characteristics of the clinical teachers, their path in Internal Medicine and their involvement in the residents learning process. RESULTS: We collected 213 valid questionnaires, making for an estimated response rate of 28.4%. Median global satisfaction with the clinical teacher was 4.52 (± 1.33 points) and the classification of the relationship between resident and clinical teacher was 4.86 ± 1.04 points. The perfect clinical teacher is defined by high standards of dedication and responsibility (4.9 ± 1.37 points), practical (4.8 ± 1.12 points) and theoretical skills (4.8 ± 1.07 points). The multiple linear regression model allowed to determine predictors of the residentâs satisfaction with their clinical teacher, justifying 82,5% of the variation of satisfaction with the clinical teacher (R2 = 0.83; R2 a = 0.82). DISCUSSION: Postgraduate medical education consists of an interaction between several areas of knowledge and intervening variables in the learning process having the clinical teacher in the central role. Overall, the pedagogical abilities were the most valued by the Internal Medicine residents regarding their clinical teacher, as determinants of a quality residentship. CONCLUSION: This study demonstrates the critical relevance of the clinical teacher in the satisfaction of residents with their residentship. The established multiple linear regression model highlights the impact of the clinical and pedagogical relantionship with the clinical teacher in a relevant increase in the satisfaction with the latter.

Introdução: A formação médica pós-graduada consiste num processo complexo no qual os orientadores assumem um papel fundamental. Apesar da sua importância, pouco se sabe sobre os orientadores dos internos portugueses. O presente estudo pretende caracterizar os orientadores de formação em Medicina Interna em Portugal. Material e Métodos: Realizado um estudo observacional, transversal e de carácter analítico, através um questionário online anónimo onde foram avaliadas as características demográficas dos orientadores, o seu percurso na Medicina Interna e envolvimento com o processo formativo. Resultados: Dos 213 questionários válidos (taxa de resposta estimada de 28,4%), a média global da satisfação com o orientador encontra-se nos 4,52 pontos (± 1,33), sendo a relação entre interno e orientador classificada nos 4,86 ± 1,04 pontos. O orientador âidealâfoi caracterizado como dedicado e responsável (4,9 ± 1,37 pontos), com domínio de competências práticas (4,8 ± 1,12 pontos) e teóricas (4,8 ± 1,07 pontos). Foram identificados como preditores da satisfação dos internos com o orientador a relação estabelecidaentre ambos, o envolvimento do orientador na formação, o seu dinamismo, inovação e disponibilidade [modelo explicativo de 82,5% da satisfação (R2 = 0,83; R2 a = 0,82)]. Discussão: O orientador desempenha um papel preponderante no sucesso da formação pós-graduada. Os internos de Medicina Interna em Portugal encontram-se globalmente satisfeitos com os seus orientadores e valorizam preferencialmente as suas capacidades pedagógicas. Conclusão: Este estudo aponta para a importância do orientador de formação na satisfação do interno com o seu internato, alertando para a necessidade de investir nos orientadores como forma de investimento na formação médica pós-graduada.

Belimumab in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is one of the most challenging autoimmune disorders with a complex pathophysiology and diverse clinical presentation. Many drugs have been used to treat SLE with suboptimal results, especially in patients with moderate-to-severe disease. Belimumab is the first biological drug to be approved for the treatment of SLE in more than 50 years. This monoclonal antibody blocks B-cell activating factor, a cytokine important for B-cell differentiation and survival. In this review we focus on the activity of belimumab in patients with SLE and discuss the controversies of its use.

What can we learn from systemic lupus erythematosus pathophysiology to improve current therapy?

Systemic lupus erythematosus is an autoimmune disorder that can affect every organ system and cause a wide range of signs and symptoms. The precise pathogenic mechanism of disease remains uncertain, but it is clearly complex and involves the activation and deregulation of many components of the immune system. Certain well-characterized patterns of immune pathology are shared by a significant subset of patients and selective targeting of one or more of the key immune system molecules offers the prospect of more effective treatment. This review addresses the current and future treatment for patients with lupus, going from the lessons learnt with pathophysiologic studies to recent clinical trials with biological agents.

B-cell survival factors in autoimmune rheumatic disorders.

Autoimmune rheumatic disorders have complex etiopathogenetic mechanisms in which B cells play a central role. The importance of factors stimulating B cells, notably the B-cell activating factor (BAFF) and A proliferation inducing ligand (APRIL) axis is now recognized. BAFF and APRIL are cytokines essential for B-cell proliferation and survival from the immature stages to the development of plasma cells. Their levels are increased in some subsets of patients with autoimmune disorders. Several recent biologic drugs have been developed to block this axis, namely belimumab [already licensed for systemic lupus erythematosus (SLE) treatment], tabalumab, atacicept and blisibimod. Many clinical trials to evaluate the safety and efficacy of these drugs in several autoimmune disorders are ongoing, or have been completed recently. This review updates the information on the use of biologic agents blocking BAFF/APRIL for patients with SLE, rheumatoid arthritis, Sjögren's syndrome and myositis.