Background <p>Vacuolization, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is recently described, for which the diagnosis and management lack official guidelines.</p> Aims <p>To&#xa0;assess the diagnostic capabilities and disease management of VEXAS syndrome among physicians in the global context.</p> Methods <p>An electronic survey was sent to clinicians with expertise in VEXAS syndrome between January and February 2025 to gather real-life data on the management of VEXAS.</p> Results <p>Seventy-four clinicians completed the survey from Europe (<i>n</i> = 51, 68.9%), North America (<i>n</i> = 9, 12.2%), Australasia (<i>n</i> = 6, 8.1%), Asia (<i>n</i> = 6, 8.1%), Africa (<i>n</i> = 1, 1.4%), and South America (<i>n</i> = 1, 1.4%), mostly being hematologists (<i>n</i> = 24, 32.4%) and rheumatologists (<i>n</i> = 24, 32.4%). Majority of the clinicians were managing between 1 and 4 (<i>n</i> = 40, 54.1%) and 5 and 9 patients (<i>n</i> = 17, 23%) with VEXAS syndrome, with regular clinic review, typically under 7-weekly intervals (<i>n</i> = 44, 59.5%).</p> <p><i>UBA1</i>&#xa0;mutation testing was available for 76% of physicians and next-generation sequencing (NGS) of the entire&#xa0;gene was most common (<i>n</i> = 24, 32.9%) with a turnaround time within 12&#xa0;weeks. C-reactive protein (CRP) was selected by over half of the clinicians (<i>n</i> = 35, 55.4%) as a marker of disease relapse. Treatment with corticosteroids at&#xa0;1&#xa0;mg/kg (<i>n</i> = 48, 64.9%) was the most common initial dosing and upfront systemic immunomodulatory treatment was added by more than half of clinicians (<i>n</i> = 39, 52.7%). The most frequent treatments of choice (<i>n</i> = 66) were Janus kinase inhibitors (JAKi) and IL-6 targeted monoclonal antibodies (both <i>n</i> = 21, 31.8%). Azacitidine was mostly used in patients with concomitant myelodysplastic syndrome (MDS) (69.9%). Only 29.6% indicated that allogeneic hematopoietic stem cell transplant (allo-HSCT) had been successfully completed in their department.</p> Conclusions <p>This is the first clinician survey on VEXAS syndrome, encompassing a global representation of multiple specialties on current disease management, highlighting several unmet needs (longitudinal follow-up, lack of on-label drugs, financial toxicity) actionable for future research.<Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> <p><b>Keypoints</b></p> <p>• <i>Globally, VEXAS syndrome is being increasingly recognized, and this survey demonstrates real-life physician practices.</i></p> <p>•&#xa0;<i>The survey identified both hematologists and rheumatologists as the main care providers, amongst&#xa0;other specialties, identifying the need for a multidisciplinary approach&#xa0;in managing patients with VEXAS syndrome.</i></p> <p>•&#xa0;<i>Diagnostic UBA1 testing was available for more than 75% clinicians with turnaround time &lt; 12&#xa0;weeks and C-reactive protein was selected as a useful marker of disease relapse.</i></p> <p>•&#xa0;<i>Treatment modalities were heterogeneous, identifying the need for consensus guidelines.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>