UBA1 and VEXAS syndrome: Fever, inflammation and vacuoles in haematopoietic cells are the main characteristics associated with VEXAS syndrome, VEXAS is a novel autoimmune disease prototype, characterized by somatic mutations in the UBA1 gene encoding the enzyme 1 (E1) activator required for ubiquitin signalling, VEXAS syndrome patients exhibit a systemic autoimmune syndrome associated with haematological damage, particularly cell loss, whose pathophysiology has yet to be elucidated.4