Idiopathic Pulmonary Fibrosis and Telomeres
Alba Mulet, Jaime Signes‐Costa
Abstract
Idiopathic pulmonary fibrosis is an interstitial lung disease of unknown etiology with a highly compromised prognosis and a significant mortality rate within a few years of diagnosis. Despite being idiopathic, it has been shown that telomeric shortening could play an important role in its etiopathogenesis. Mutations in telomere-related genes have been identified, but they are not always present despite telomere shortening. On the other hand, this telomeric shortening has been linked to a worse prognosis of the disease independently of other clinical factors, implying it may serve as a biomarker.
Topics & Concepts
MedicineTelomereIdiopathic pulmonary fibrosisPulmonary fibrosisBiomarkerEtiologyDiseaseInterstitial lung diseaseFibrosisLungInternal medicinePathologyGeneOncologyGeneticsBiologyInterstitial Lung Diseases and Idiopathic Pulmonary FibrosisTelomeres, Telomerase, and SenescenceOccupational and environmental lung diseases