Satoh Kasumi, Tsujimoto Yasushi, Kasugai Daisuke, Okura Kazuki, Luthe Sarah Kyuragi, Ono Takao, Miyamoto Yuki, Matsuyama Tasuku, Okuyama Manabu, Watase Taketo, Nakae Hajime, Goto Tadahiro
Department of Emergency and Critical Care Medicine, Akita University Graduate School of Medicine, 1 - 1- 1 Hondo, Akita, 010 - 8543, Japan.
Department of Health Promotion and Human Behavior, Kyoto University Graduate School of Medicine/School of Public Health, Kyoto, Japan.
Crit Care. 2025 Apr 17;29(1):154. doi: 10.1186/s13054-025-05390-y.
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.
空泡、E1酶、X连锁、自身炎症性、体细胞（VEXAS）综合征是一种最近发现的严重疾病，主要影响成年男性，其特征为全身性炎症和血液学异常。尽管它对患者预后有深远影响，但重症监护人员对VEXAS综合征的认识仍然极为有限，常常导致识别、诊断延迟以及未能及时开始适当治疗。本研究旨在通过对重症监护环境下的VEXAS综合征进行范围综述来填补这一知识空白。
本范围综述遵循PRISMA-ScR指南和乔安娜·布里格斯研究所方法，于2024年5月19日分析来自Cochrane CENTRAL、通过PubMed的MEDLINE、EMBASE和科学网的数据。我们纳入了报告需要重症监护的VEXAS综合征患者临床特征和治疗的研究。
在1262份识别出的报告中，78份报告符合纳入标准，包括45份病例报告/系列、17项观察性研究、15篇综述和1项系统综述。对55例病例的分析显示，中位年龄为69岁，男性占主导（54/55）。ICU入院率在28%至33%之间，死亡率在18%至40%之间。关键表现包括休克、噬血细胞性淋巴组织细胞增生症、急性呼吸窘迫综合征、血栓形成和气道水肿。脓毒症是主要死因，其次是其他原因，包括VEXAS综合征相关器官衰竭、心血管事件和肠穿孔。治疗方法将传统重症监护措施与免疫抑制和免疫调节疗法相结合，不过感染并发症经常被报告。
本综述揭示了在重症监护环境下缺乏针对VEXAS综合征的系统分析研究，这表明在理解VEXAS综合征的临床特征和最佳治疗方法方面存在重大差距。针对重症监护环境下的VEXAS综合征进行进一步研究对于提高早期识别、制定标准化治疗方案以及最终改善这一复杂患者群体的患者预后至关重要。
