Cumulative incidence of thrombosis in VEXAS syndrome. Fine-Gray competing risk regression models using death as a competing risk. (A) The cumulative incidence of VTE was 33% at median follow-up of 890 days, 17% by 1 year, and 40% by 5 years. (B) The cumulative incidence of arterial thrombosis was 9% at median follow-up of 890 days, 6% at 1 year, and 11% at 5 years. (C) The cumulative incidence of PE was 9.7% at median follow-up of 980 days, 4% by 1 year, and 13% by 5 years. (D) The cumulative incidence of DVT was 29% at median follow-up of 890 days, 16% by 1 year, and 35% by 5 years. (E) The cumulative incidence of VTE by UBA1 variant was 7% for M41L, 22% for M41V, and 18% for M41T at 1 year and 46% for M41L, 37% for M41V, and 41% for M41T. No patients with splice site variants had VTE.