Median time to death or lung transplant was reduced by 46 months in those with <i>CC</i> genotypes at rs4588 and 38 months in those with non-A-containing haplotypes at rs7041/4588 (p=0.014 and 0.008, respectively).The VTDB axis is associated with disease severity and outcome, and <i>GC</i> genotype could help predict transplant-free survival in LAM.