Table 2.

Cox proportional hazards analysis of clinical factors associated with survival in VEXAS syndrome

UnivariableMultivariable*
Hazard ratio (95% CI)P valueHazard ratio (95% CI)P value
Ear chondritis 0.24 (0.09-0.61) <.01 0.32 (0.12-0.90) .03 
Val variant 3.49 (1.47-8.32) <.01 2.56 (1.01-6.47) .05 
Transfusion dependence 4.56 (1.87-11.08) .03 4.47 (1.79-11.18) <.01 
Myelodysplastic syndrome 2.44 (0.92-6.50) .07   
Nose chondritis 0.44 (0.15-1.13) .10   
Age at disease onset 1.00 (0.98-1.11) .12   
Unprovoked DVT 0.49 (0.17-1.25) .14   
Lower extremity edema 0.55 (0.20-1.38) .22   
Use of DMARDs 0.20 (0.11-1.56) .23   
Fever 3.19 (0.65-1.52) .25   
Arthritis 0.70 (0.28-1.70) .42   
Testicular pain/swelling 0.78 (0.28-1.74) .42   
Hearing loss 1.49 (0.44-4.23) .45   
UBA1 VAF 0.98 (0.94-1.03) .52   
Pleural effusion 1.28 (0.44-3.21) .63   
Periorbital edema 3.61 (0.36-2.92) .82   
Eye inflammation 1.32 (0.36-2.91) .85   
Skin involvement 1.05 (0.38-3.72) .92   
Pulmonary infiltrates 0.96 (0.39-2.48) .94   
UnivariableMultivariable*
Hazard ratio (95% CI)P valueHazard ratio (95% CI)P value
Ear chondritis 0.24 (0.09-0.61) <.01 0.32 (0.12-0.90) .03 
Val variant 3.49 (1.47-8.32) <.01 2.56 (1.01-6.47) .05 
Transfusion dependence 4.56 (1.87-11.08) .03 4.47 (1.79-11.18) <.01 
Myelodysplastic syndrome 2.44 (0.92-6.50) .07   
Nose chondritis 0.44 (0.15-1.13) .10   
Age at disease onset 1.00 (0.98-1.11) .12   
Unprovoked DVT 0.49 (0.17-1.25) .14   
Lower extremity edema 0.55 (0.20-1.38) .22   
Use of DMARDs 0.20 (0.11-1.56) .23   
Fever 3.19 (0.65-1.52) .25   
Arthritis 0.70 (0.28-1.70) .42   
Testicular pain/swelling 0.78 (0.28-1.74) .42   
Hearing loss 1.49 (0.44-4.23) .45   
UBA1 VAF 0.98 (0.94-1.03) .52   
Pleural effusion 1.28 (0.44-3.21) .63   
Periorbital edema 3.61 (0.36-2.92) .82   
Eye inflammation 1.32 (0.36-2.91) .85   
Skin involvement 1.05 (0.38-3.72) .92   
Pulmonary infiltrates 0.96 (0.39-2.48) .94   
*

Only variables with P < .05 on univariable analyses were carried forward to the multivariable model.

Modeled as time-varying predictor variables.

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