Idiopathic pulmonary fibrosis (IPF) is the most common manifestation of telomere-mediated
disorders. Germline mutations in the essential telomerase genes, hTERT and hTR, are the
causal genetic defect in up to one-sixth of pulmonary fibrosis families. The presence of
telomerase mutations in this subset is significant for clinical decisions as affected individuals
can develop extra-pulmonary complications related to telomere shortening such as bone
marrow failure and cryptogenic liver cirrhosis. There is also evidence that IPF is an ancestral …