Viewpoint: Pre- and post-lung transplant considerations for patients with ultra-short telomere length

Extract Lung transplantation remains the only life-extending procedure for patients with idiopathic pulmonary fibrosis (IPF) and related progressive interstitial lung disease (ILD). Discoveries from recent decades have shown that mutations in telomerase and other telomere maintenance genes are their most common inherited risk factor, identifiable in up to 30–35% of families with pulmonary fibrosis [1]. Mutations in nine telomerase and telomere maintenance genes are confirmed to predispose to adult-onset pulmonary fibrosis by co-segregation in large families and functional studies (table 1) [2–13]. They compromise telomerase abundance, recruitment and function [1, 14].