

Dergi
Arp Rheumatology
Cilt
5
ISSN
27954552
Yayın Tarihi
1 Temmuz 2026
VEXAS syndrome is an adult-onset autoinflammatory disease caused by somatic mutations in the UBA1 gene and is characterized by overlapping rheumatologic and hematologic clinical features. The most common clinical manifestations include recurrent fever, arthralgia/arthritis, pulmonary involvement, skin lesions, vasculitis, and/or thromboembolic events. Aortic–renal aneurysm in VEXAS syndrome is a rare entity and is associated with poor prognosis. Herein, we describe a 69-year-old male patient with VEXAS syndrome complicated by an aortic–renal aneurysm who responded well to tocilizumab treatment.