INTRODUCTION VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is an X-linked, systemic, haemato-inflammatory syndrome that was first described in 2020.1 VEXAS is a clonal disease caused by somatic mutations in the UBA1 gene that originate in a haematopoietic stem and progenitor cell (HSPC) but mostly affects the myeloid precursor cells.2 The UBA1 gene encodes the ubiquitin-activating enzyme, E1, that is essential for ubiquitin signalling, a process involved in protein...