In up to 15–20% of the familial IPF cases, causative mutations have been identified in genes involved in regulating telomere function—telomerase reverse transcriptase, TERT; telomerase RNA component, TERC; dyskerin, DKC1; telomere interacting factor 2, TINF2; and regulator of telomere elongation helicase, RTEL1. Here, DKC1 is linked to idiopathic pulmonary fibrosis.