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

Critical Care, Journal Year: 2025, Volume and Issue: 29(1)

Published: April 17, 2025

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

Language: Английский

A Vexing diagnosis: A case report on VEXAS syndrome

Canadian Journal of Respiratory Critical Care and Sleep Medicine, Journal Year: 2025, Volume and Issue: unknown, P. 1 - 4

Published: March 6, 2025

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: A comprehensive review of cases across different ethnicities

European Journal of Internal Medicine, Journal Year: 2025, Volume and Issue: unknown

Published: May 1, 2025

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) is an autoinflammatory disease associated with mutations in the UBA1 gene. Although has been described many different countries, no studies have investigated origin of patients to determine if universal across ancestries. The aim this study investigate distribution VEXAS syndrome continents and ethnicities. A literature review all reported cases was conducted between October 2020 April 2025 using term 'VEXAS' all-field filter Pubmed Web Science databases. Epidemiological clinical data were collected for included patients. If country not described, it assumed be same as evaluation. subgroup analysis performed whose or ethnicity documented by authors. 674 collected, from four 32 countries. Considering origin, 451 19 Of these, recorded 372 presence Caucasian, Central East Asian, South Middle Eastern, American results support a broad global highlight importance investigating regardless patient's compatible symptoms.

Language: Английский