Clinical features and treatments of VEXAS syndrome in critical care: a scoping review

Abstract

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently discovered severe disorder that predominantly affects adult males, characterized by systemic inflammation and hematologic abnormalities. Despite its profound impact on patient outcomes, awareness of VEXAS syndrome among critical care providers remains severely limited, often leading to delayed recognition, diagnosis, and initiation of appropriate treatment. This study aims to address this knowledge gap by conducting a scoping review on VEXAS syndrome in the critical care setting. This scoping review followed the PRISMA-ScR guidelines and Joanna Briggs Institute methodology, analyzing data from Cochrane CENTRAL, MEDLINE via PubMed, EMBASE, and Web of Science on May 19, 2024. We included studies that reported clinical features and treatments of patients with VEXAS syndrome requiring critical care. Of the 1262 reports identified, 78 reports met the inclusion criteria, including 45 case reports/series, 17 observational studies, 15 reviews, and one systematic review. Analysis of 55 cases revealed a median age of 69 with a strong male predominance (54/55). ICU admission rates ranged from 28 to 33%, with mortality rates between 18 and 40%. Critical manifestations included shock, hemophagocytic lymphohistiocytosis, acute respiratory distress syndrome, thrombosis, and airway edema. Sepsis was the leading cause of death, followed by other causes including VEXAS syndrome related organ failure, cardiovascular events, and intestinal perforation. Treatment approaches combined conventional critical care measures with immunosuppressive and immunomodulatory therapies, although infectious complications were frequently reported. This review revealed the lack of systematically analyzed studies focusing on VEXAS syndrome in the critical care setting, suggesting a significant gap in understanding the clinical characteristics and optimal treatments for VEXAS syndrome. Further research focused on VEXAS syndrome in the critical care setting is essential to improve early recognition, develop standardized treatment protocols, and ultimately improve patient outcomes in this complex patient population.