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

Summary

PRISMA-ScR-compliant scoping review synthesizing 78 reports found ICU admission 28–33% and mortality 18–40% in VEXAS; critical manifestations included shock, HLH, ARDS, thrombosis, and airway edema, with sepsis the leading cause of death. Treatments combined critical care with immunosuppressive/immunomodulatory therapy but were frequently complicated by infections.

Key Findings

• Across 78 reports, ICU admission ranged 28–33% and mortality 18–40% for VEXAS cases.
• Critical manifestations included shock, HLH, ARDS, thrombosis, and airway edema; sepsis was the leading cause of death.
• Treatment combined critical care with immunosuppressive/immunomodulatory agents, but infectious complications were common.

Clinical Implications

Increase ICU awareness of VEXAS in older men with systemic inflammation and cytopenias; incorporate ARDS and sepsis vigilance and tailor immunosuppression while mitigating infectious risks.

Limitations

• Predominant reliance on case reports/series with heterogeneity and publication bias
• No quantitative meta-analysis; limited standardization of ICU management data

Future Directions

Prospective ICU cohorts to define organ failure trajectories, diagnostic criteria, and optimal immunomodulation; strategies to mitigate infectious complications.