Since mutations in NKX2-1 are extremely rare in the general population, the lack of identifiable mutations in NKX2-1 in our IPF and COPD cohorts may simply be a function of the limited sample size of our cohort, thus making it difficult to know the true prevalence of NKX2-1 mutations in patients with IPF. Here, NKX2-1 is linked to idiopathic interstitial pneumonia.