VEXAS Syndrome
New VEXAS syndrome research, summarized each month in plain language.
2026 so far: So far in 2026 (January 2026 to September 2026), allo-HSCT was linked to UBA1-clone clearance and more steroid stopping than drug approaches, while IL-6/JAK responses were often incomplete. Read the 2026 review
We also cover clinical trials.
Yearly update
2026 so far
- Transplant outcomes. Retrospective data increasingly compared clone clearance across approaches.
- How medicines worked. Cohorts clarified limits of cytokine blockers and steroid-sparing.
- Coordinated care. Field moved toward shared decision-making and shared measurement tools.
- Risks to track. Registries linked specific features to higher complication and outcome risk.
- Getting diagnosed. Reports emphasized targeted suspicion plus quicker genetic confirmation.
New
September 2026
- IL-6 and JAK drugs. IL-6 and JAK inhibitors sometimes lowered inflammation, but often incompletely.
- Steroid tapering. Some people reduced steroids, but long-term steroid-sparing was limited.
- Blood clot tendency. Blood tests suggested blood vessel activation and higher clotting tendency.
August 2026
- Blood clot frequency. Blood clots were common, including after VEXAS diagnosis.
- Clots on blood thinners. Some clots occurred despite blood thinners, often during flares.
- Screening results. Testing 21 men with unexplained clots and VEXAS-like features found no typical VEXAS mutations.
- NK cell signal. One NK cell pattern was linked to HLH risk, not proven.
July 2026
- Disease activity score. A new VEXAS activity score was agreed on, but needs testing.
- Eye inflammation. Eye inflammation often improved with steroids, but not always.
- Missed diagnosis risk. VEXAS can look like other inflammatory diseases or infections.
June 2026
- UBA1 mutation clearance. Donor stem cell transplant more often made UBA1 undetectable.
- Steroid stopping. Stopping steroid medicines was more common after transplant.
- Rapid UBA1 testing. A rapid PCR test quickly detected common UBA1 mutations.
May 2026
- Steroids: quick relief, hard taper. Steroids often reduced inflammation quickly, but many reports still described symptoms returning during tapering.
- Azacitidine: slower responses. Azacitidine responses were often slower to appear, and some reports described longer-lasting control.
- Testing in high-risk Sweet syndrome. In a small high-risk Sweet syndrome group, VEXAS was found more often among those tested.
April 2026
- UBA1 mutation burden. In one registry study, a higher fraction of blood cells with a UBA1 mutation was linked to a higher one-year risk of death or becoming dependent on blood transfusions.
- Starting steroid dose. In the same registry, a higher starting prednisolone dose was associated with worse one-year outcomes.
- PET/CT in remission. In a PET/CT study, scans often stayed abnormal even when VEXAS was thought to be in remission, and the meaning of this finding was unclear.
March 2026
- Fever and severity. Registry data linked recurrent fever with more severe overall illness in VEXAS.
- Treatment response signals. In one registry analysis, possible treatment-response patterns differed by fever level, but this was exploratory and not proven.
- Broader UBA1 variant range. Recent reports expanded the range of UBA1 gene variants associated with VEXAS-like illness.
- Lung imaging vs inflammation. One case report described improved lung imaging even while inflammation continued.
February 2026
- Coordinated care. More centers are using team-based discussions to choose steroid-sparing treatment options for VEXAS.
- Pacritinib trial underway. Pacritinib is being studied in a randomized trial, but results are not yet available.
- Cancer risk signal. Registry data suggest overall cancer risk may be higher in VEXAS than in some other autoinflammatory diseases.
- More organs reported. Recent case reports describe a wider range of organs that can be involved in VEXAS.
January 2026
- Diagnosis often needs UBA1 testing. When VEXAS looked like other inflammatory or bone marrow conditions, UBA1 genetic testing was repeatedly the step that confirmed the diagnosis, often prompted by bone marrow findings and abnormal blood counts.
- Treatment evidence is mostly case reports. Treatment details were mostly individual patient reports, including two cases where JAK inhibitors used with steroids were linked to reduced severe inflammation and other reports describing blood-disorder treatments when VEXAS overlapped with marrow disease.
- Orbital inflammation patterns. Across multiple patients, orbital inflammation was reported with swelling and pain, vision was usually preserved, and a small number had serious one-sided complications.
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