[VEXAS syndrome].

The VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) syndrome is an acquired autoinflammatory disease, which is based on somatic mutations in the UBA1 gene and described as an hematoinflammatory disease. It is clinically expressed as an inflammatory systemic disease with general vegetative symptoms and organ manifestations of the skin, lungs and the hematopoietic system. The symptoms are not uncommonly refractory to conventional anti-inflammatory therapeutic agents. The disease is associated with a clearly increased mortality and morbidity.