A novel VEXAS Syndrome
The medical world is developing, along with new pathology described. One of such recently described autoinflammatory disorders is VEXAS syndrome- first published in 2021 by David Beck and colleagues from the National Institutes of Health. This pathology is associated with hematological impairments, especially cytopenias. Patient clinical presentation include broad heterogeneous symptoms. Typically, it starts as a systemic inflammatory disease with unexplained episodes of fever, frequently involving lungs, skin, blood vessels and joints. Laboratory findings are described as cytopenias, macrocytic anemia (96%), and thromboembolic disorders (44%). To decrease high rates of morbidity and mortality in VEXAS patients, early diagnosis is a key of success and should be based on a multidisciplinary consultation (rheumatologists, dermatologists, clinical hematologists and biologists). Details by the link!