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BackgroundAnti-CD20 monoclonal antibodies are a class of immunosuppressive drugs widely used in the treatment of central nervous system (CNS) inflammatory diseases, with well-established efficacy and safety. Although rare, these therapies can be associated with serious adverse events including hematological and infectious complications. This study aims to evaluate their safety and tolerability profile in real-world clinical practice.MethodsWe conducted a retrospective cohort study comprising patients diagnosed with multiple sclerosis (MS) treated with anti-CD20 drugs since 2016 followed in the Demyelinating Diseases clinic of a tertiary center. Clinical and paraclinical parameters were evaluated, including complete blood count and immunoglobulins measurements.ResultsA total of 160 with multiple sclerosis (pwMS) were included in our study, of whom 110 (68.8%) were female and 147 are currently receiving anti-CD20 therapies. Half of the patients were diagnosed with relapsing–remitting MS, while the remaining had progressive forms, including 23 with primary-progressive MS and 57 with secondary-progressive MS. Eighty-three patients were on ocrelizumab, 48 on rituximab, and 29 on ofatumumab. The mean follow-up duration from the start of anti-CD20 therapy was 30.5 ± 21.3 months. During this period, serious adverse events were observed in 9 patients, including SARS-CoV-2 infection (resulting in one death), urinary tract infection, febrile neutropenia, severe diarrhea, and acute hepatitis. The rate of serious infections in the ocrelizumab subgroup was consistent with the literature, although a higher rate was observed in the rituximab subgroup. A positive correlation was found between serious infectious complications and lower IgG levels. Additionally, longer exposure to anti-CD20 therapy in our cohort was associated with an increased risk of IgG deficiency and a higher incidence of serious infections. Lymphopenia was detected in 25 patients, though it was not directly linked to the occurrence of serious infections.ConclusionOur work confirms the tolerability and safety of anti-CD20 drugs in a real-world clinical practice MS cohort, despite their frequent association with analytical changes such as lymphopenia and hypogammaglobulinemia. To better understand the clinical significance of hypogammaglobulinemia secondary to anti-CD20 treatment and to develop strategies for mitigating the associated potential infection risk, future studies with larger populations are essential.

Diffuse idiopathic skeletal hyperostosis (DISH) and axial spondyloarthritis (axial SpA) are differential diagnoses of lower back pain. While the latter is considered to be an inflammatory disease, DISH is thought to be a metabolic condition. The authors report a case of a 34-year-old man who presented with a one-year history of axial lower back pain associated to migratory polyarthritis, buttock and heel pain. Imaging revealed contiguous calcification of the anterior longitudinal ligament of the cervical segment, meeting major criteria for DISH. However, he also exhibited signs of bilateral sacroiliitis highly suggestive of axial SpA for which he initiated biological therapy.

Classical Ehlers-Danlos syndrome (cEDS) is one of the 13 subtypes of Ehlers-Danlos syndrome, which has the major clinical criteria of hyperextensibility skin, atrophic scars, and generalised joint hypermobility. The occurrence of aortic dissection has been described in some subtypes of Ehlers-Danlos, but it has a rare association with the cEDS subtype. This case report discusses a 39-year-old female with a past medical history of transposition of great arteries with a Senning repair at the age of 18 months and controlled hypertension with medication, who presents a spontaneous distal aortic dissection. The diagnosis of cEDS was made using the major criteria, and a novel frameshift mutation in COL5A1 was discovered. The reported case emphasises that in patients with cEDS, vascular fragility may be a complication.

Eosinophilic gastroenteritis (EGE) is a rare gastrointestinal disease characterized by generalized abdominal pain, nausea, vomiting, diarrhea, gastrointestinal bleeding and weight loss. The etiology and pathogenesis are not well understood and mostly based on case reports. Clinical presentation may vary depending on sites and depth of involvement of the gastrointestinal tract. The diagnosis is usually confirmed by histology that shows eosinophilic infiltration and the absence of secondary cause of eosinophilia. The authors present a case report of a 47-year-old woman that was diagnosed with dyslipidemia, obesity and allergic rhinitis. She had no significant family history. She was admitted in internal medicine department with 2 weeks of abdominal pain, nausea, vomiting, diarrhea and weight loss (> 10%). Further study revealed increased eosinophil count, normocytic anemia, elevated erythrocyte sedimentation rate and normal Ig E levels. Digestive endoscopic study was performed, and multiple biopsy specimens showed moderate eosinophilic infiltration in stomach and duodenal mucosa. The patient was started on prednisolone, with clinical improvement. Follow-up go the patient twenty years after the beginning of this treatment showed stabilization of clinical symptoms and laboratory tests. So, eosinophilic gastroenteritis is characterized by three criteria - presence of gastrointestinal symptoms, histologic evidence of eosinophilic infiltration and exclusion of other causes of tissue eosinophilia. The treatment is based on the severity of symptoms. Corticosteroids are the mainstay of therapy, so the usual dose of prednisolone/prednisone is 20-40 mg daily for two weeks with tapering thereafter. In the literature, the use of montelukast and biologic agents have been reported.

Leucocytoclastic vasculitis refers to a small vessel vasculitis caused by immune complexes, infections and medications. IgA or IgM/IgG immune complexes can be found in direct immunofluorescence studies suggesting specific forms of leucocytoclastic vasculitis. Some authors suggested that IgA vasculitis and IgA nephropathy were two clinical mani- festations of the same disease. From a histological point of view, it is not possible to distinguish a glomerulonephritis as part of an IgA vasculitis from an IgA nephropathy. The authors present a case of a 50-year-old woman diagnosed with vitiligo ad immune thrombocytopenic purpura (ITP). ITP was diagnosed 2 years before the present case. She presented to the autoimmune diseases’ appointment with a pruriginous rash of the lower extremities over the last 3 months. Skin biopsy was suggestive of leukocytoclastic vasculitis, revealing deposits of C3, IgG (less intensity) and IgA. IgA vasculitis was then assumed. After a few weeks, she kept peripheral edema, but an increasing decline in renal function was detected. Therefore, a renal biopsy was performed, which revealed endocapilar proliferative glomerulonephritis and predominantly C3 mesangial deposits, with IgA and vestigial IgM. These results were compatible with a C3 glomerulonephritis. The patient was started on systemic steroid treatment with prednisolone 1 mg/kg/day and ramipril 2.5 mg/day with progressive normalization of renal function. With this case, the authors emphasize the possibility that all these manifestations could be part of the same disease spectrum, but also, the importance of complement activation. So, this case may constitute additional evidence of the complement activation in pathogenesis of this vasculitis, however, further investigation is need, particularly to understand C3 glomerulonephritis, a rare kidney disease.

A pandemia COVID‑19 foi declarada uma emergência de saúde mundial. Estima‑se que poderá originar um elevado nível de sofrimento, prevendo‑se que o período de crise origine variados desafios na esfera da saúde mental, tanto na população geral como nas pessoas com perturbação mental prévia. O sofrimento psicológico e angústia generalizada devido ao impacto imediato do vírus na saúde, mas também a necessidade de implementar medidas de quarentena, de isolamento físico associado à perda nas atividades educativas e laborais, adivinham o medo da doença, o receio da morte e incerteza quanto ao futuro. Por outro lado, estima‑se que a pandemia COVID‑19 poderá afetar de modo desproporcional populações mais vulneráveis, nomeadamente os profissionais de saúde e aqueles com antecedentes prévios de perturbação mental. Pela ameaça de problemas da saúde mental que a população em geral pode enfrentar e a possível agudização da condição de base nos doentes com perturbação mental prévia éimportante uma avaliação dos vários problemas emergentes. Com efeito, os serviços de saúde mental necessitam de uma adaptação estrutural de modo a lidar com o risco acrescido das morbidades psiquiátricas, desenvolvendo e implementando programas de rastreio e intervenção precoce, que visem não apenas os efeitos agudos, mas também os efeitos a longo prazo ou crónicos desta pandemia. A elaboração deste artigo de revisão tem por objetivo reunir, de forma rigorosa, a informação dispersa em variadas fontes científicas atualmente existentes sobre o impacto da infeção por COVID‑19 na saúde mental, assim como a experiência clínica no terreno. Deste modo, os autores realizaram uma revisão da literatura, pela pesquisa através da PubMed e Medscape usando as palavras‑chave “Pandemic”, “COVID‑19”, “Mental Health”, “Mental Disorder”. O artigo propõe‑se a desenvolver de forma concisa e sumária algumas das problemáticas particulares inerentes à pandemia COVID‑19 e saúde mental, propondo ainda algumas estratégias e intervenções para fazer face à situação de crise vivida. Considera‑se, no entanto, a necessidade futura da contínua realização de estudos e avaliação da experiência prática, de modo a reorientar as políticas e medidas de intervenção na saúde mental.

Fasciitis with eosinophilia (FE) is a rare connective tissue disease. Due to its rarity, large-scale studies are lacking, which makes its treatment challenging. Systemic corticosteroids (SCSs) are the cornerstone of treatment; however, additional immunosuppressive drugs (ISDs) are frequently necessary (usually methotrexate). We report 2 patients, for whom an SCS and methotrexate were not a viable long-term option. In the first case, we were unable to taper the SCS dose without symptom relapse, the patient showed only a partial response to methotrexate and presented side effects. The second case never fully responded to the SCS and methotrexate and demonstrated serious SCS adverse effects. Both patients were started on tocilizumab with extremely favourable results, making this drug a potential therapeutic weapon for these patients.

A sarcoidose é uma doença granulomatosa que pode afetar qualquer órgão de etiologia desconhecida. Apresentamos o caso de um homem, de 49 anos, com antecedentes pessoais de um episódio de eritema nodoso. O estudo imagiológico do tórax mostrou múltiplas adenopatias mediastínicas e justa-hilares bilaterais, apesar de não apresentar queixas respiratórias. O lavado broncoalveolar revelou uma relação de células CD4/CD8 de 9,98 e o estudo analítico revelou uma enzima conversora da angiotensina elevada, sem outros achados. O doente iniciou corticoterapia verificando-se uma melhoria das manifestações cutâneas. Após dois anos, apresenta-se na consulta de Medicina Interna com queixas de emagrecimento, anorexia, fadiga, hipoacusia, síndrome vertiginosa e hipostesia dorsal em banda. Este caso mostra o envolvimento multissistémico da sarcoidose, tendo como ponto de partida para o seu diagnóstico uma manifestação cutânea e realça a importância da vigilância e do tratamento destes doentes a fim de um controlo atempado da doença.

The gastrointestinal system is commonly implicated in Systemic Lupus Erythematosus (SLE). Ascites, produced by several mechanisms has been reported as a systemic manifestation of lupus, but only rarely as an initial presentation of the disease. Its appearance is often insidious and without abdominal pain. Chylous ascites (such as chyle in the peritoneal cavity) has seldom been reported in SLE. We describe a case of chylous ascites in an SLE patient, reviewing the other published cases, its pathophysiology and its management.

O Inventário de Fontes de Estresse Acadêmico no Curso de Medicina (IFSAM) foi desenvolvido para caracterizar as principais fontes de estresse acadêmico dos estudantes da Faculdade de Medicina da Universidade do Porto (FMUP) e a intensidade com que são experienciadas. O inventário (31 itens) foi construído por meio de entrevistas semiestruturadas a estudantes (n = 80) dos seis anos da licenciatura. Suas propriedades psicométricas foram testadas num estudo com 251 estudantes dos seis anos do curso (160 do sexo feminino e 91 do sexo masculino). A validade de constructo foi avaliada mediante análise fatorial que resultou numa solução de cinco fatores ("exigências do curso", "exigências humanas", "estilos de vida", "competição" e "adaptação") com 54,8% da variância explicada e boa representatividade do constructo. O IFSAM total evidencia ainda uma boa fidelidade, com um coeficiente de consistência interna de.88. Os resultados revelam que o IFSAM apresenta boas características psicométricas na amostra, podendo constituir um instrumento útil na avaliação das fontes e intensidade de estresse acadêmico do curso de Medicina noutras instituições.