Recently, somatic mutations in UBA1 have been associated with VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, a monogenic form of RP with a unique phenotype that includes chondritis, hematologic abnormalities, poor response to immunosuppressive therapy, and high mortality rate (3). This evidence concerns the gene UBA1 and retinitis pigmentosa 1.