Case report: Cerebral sinus vein thrombosis in VEXAS syndrome

Michael Zisapel, Estelle Seyman, Jeremy Molad, Hen Hallevi, Michal Mauda-Havakuk, Tali Jonas-Kimchi, Ori Elkayam, Tali Eviatar*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic) syndrome is a newly described hemato-inflammatory acquired monogenic entity that presents in adulthood. One of the main features of VEXAS syndrome is a high venous thromboembolism (VTE) burden, with approximately 30–40% experiencing lower extremity deep vein thrombosis and a lower incidence of pulmonary embolism at approximately 10%. To date, VEXAS syndrome has not been associated with rarer forms of VTE such as cerebral sinus vein thrombosis (CSVT) and Budd–Chiari syndrome, which are well-recognized vascular manifestations in Behcet’s disease, another autoinflammatory vasculitic disease. Herein, we describe a case of acute severe extensive and fatal CSVT in a patient with VEXAS syndrome. The event occurred during a period of apparently quiescent inflammatory status, while the patient was receiving tocilizumab and a low dose of glucocorticoids. Despite treatment with anticoagulation, high-dose glucocorticoids, endovascular thrombectomy, and intracranial pressure-lowering agents, the patient suffered severe neurologic damage and ultimately succumbed to the condition 3 weeks after the onset of CSVT. To the best of our knowledge, this is the first reported case of CVST in a patient with VEXAS syndrome.

Original languageEnglish
Article number1377768
JournalFrontiers in Medicine
StatePublished - 2024


  • VEAXS syndrome
  • autoinflammatory disease
  • case report
  • cerebral sinus vein thrombosis
  • tocilizumab (IL-6 inhibitor)
  • venous thromboembolism


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