Survival analysis in VEXAS syndrome. (A) Frequencies of common clinical diagnoses assigned to patients with VEXAS syndrome are shown stratified by genotype. (B) Kaplan-Meier curves demonstrate survival in the entire cohort and (C) are stratified by genotype. (D) Forest plot showing the results of a multivariable Cox proportional hazards model where the Val variant (c.121 A>G, p.Met41Val), transfusion dependence (time-varying predictor), and ear chondritis were significantly associated with mortality in VEXAS syndrome. *, P < .05; #, P < .01 for survival difference between all groups.