Discovered in 2020, investigations, especially in the United States are ongoing. With 10 cases and two deaths, the new VEXAS syndrome has hit Portugal.
VEXAS is a condition that affects mainly men over 50 years, and of the ten confirmed cases in the country, seven were found in the Hospital Santo António do Porto, one in the Hospital Centre of Trás-os-Montes and Alto Douro, and another in Amadora-Sintra, the only case to date in a hospital in the Lisbon region.
The first case was discovered in October last year when a patient from the Vila Nova de Gaia/Espinho Hospital Centre, who received a bone marrow transplant, did not survive and died after contracting an infection.
According to several national media reports, this rare syndrome – which is non-hereditary, despite its genetic basis – has a high mortality rate, and current data indicates that about half of those diagnosed, mainly men, die within five years of diagnosis.
Symptoms include: fever, rashes, pain and swelling in the ears and nose, coughs and shortness of breath, joint pain, and inflammation of blood vessels.
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