Medicina clinica
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Review
Paradigm shift in monogenic autoinflammatory diseases and systemic vasculitis: The VEXAS syndrome.
VEXAS syndrome was described by the end of 2020 as an autoinflammatory disease caused by post-zygotic variants in the UBA1 gene. VEXAS syndrome occurs in adult males with recurrent fever, arthralgia/arthritis, ear/nose chondritis, neutrophilic dermatosis, lung inflammation, venous thrombosis, and different types of vasculitis. Common laboratory changes include raised acute phase reactants and macrocytic anemia. ⋯ Azacitidine has been associated with a good response, especially in patients with accompanying myelodysplastic syndrome. Allogeneic hematopoietic stem cell transplantation appears to be the only curative therapy by now. VEXAS syndrome has become a paradigm shift in the diagnosis and treatment of autoinflammatory diseases and systemic vasculitis.
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SARS-CoV-2 is currently causing a persistent COVID-19 pandemic, which poses a risk of causing long-term cardiovascular sequels in the population. The viral mechanism of cell infection through the angiotensin 2 converter enzyme receptor and the limited antiviral innate immune response are the suspected causes for a more frequent cardiovascular damage in SARS-CoV-2 infection. Knowledge of the appearance during acute infection of other cardiac conditions beyond the classical myocarditis and pericarditis, the long-term cardiac manifestations (persistent COVID-19), and the increased incidence of myocarditis and pericarditis after vaccination is of special interest in order to offer our patients best practices based on current scientific evidence.