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The following is a summary of “Clinical features and treatments of VEXAS syndrome in critical care: a scoping review,” published in the April 2025 issue of Critical Care by Satoh et al. 

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome was recently identified as a severe autoinflammatory condition affecting adult males, with limited awareness among critical care providers contributing to delayed diagnosis and treatment.  

Researchers conducted a retrospective study to address the knowledge gap by performing a scoping review of VEXAS syndrome in the critical care setting.  

They performed the scoping review following the Systematic Reviews and Meta-Analysis extension for Scoping Reviews guidelines (PRISMA-ScR) guidelines and the Joanna Briggs Institute (JBI) methodology. Data was analyzed from Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE via PubMed, EMBASE, and Web of Science, retrieved on May 19, 2024. Studies reported the clinical features and treatments of individuals with VEXAS syndrome requiring critical care.  

The results showed that 78 out of 1262 reports met the inclusion criteria, comprising 45 case reports/series, 17 observational studies, 15 reviews, and 1 systematic review. Analysis of 55 cases revealed a median age of 69, with a male predominance (54/55), Intensive Care Unit (ICU) admission rates ranged from 28% to 33%, and mortality ranged from 18% to 40%. Critical manifestations included shock, hemophagocytic lymphohistiocytosis (HLH), acute respiratory distress syndrome (ARDS), thrombosis, and airway edema. Sepsis was the primary cause of death, followed by VEXAS syndrome-related organ failure, cardiovascular events, and intestinal perforation. Treatment included conventional critical care alongside immunosuppressive and immunomodulatory therapies, though infectious complications were typically reported.  

Investigators concluded a significant gap in systematically analyzed studies on VEXAS syndrome in critical care, highlighting the need for further research to enhance recognition, treatment protocols, and patient outcomes. 

Source: ccforum.biomedcentral.com/articles/10.1186/s13054-025-05390-y