Outcomes of Lung Transplantation in Patients with Telomere Related Forms of Progressive Fibrosing Interstitial Lung Disease Pulmonary Fibrosis - a Systematic Review

Lung transplantation (LTX) is the last life-extending option for patients with progressive fibrosing interstitial lung diseases (fILD). Between 12 and 71% of patients with fILD are patients with underlying telomere-dysfunction (trILD) related to pathogenic telomere-related gene (TRG) variants and/or short telomere length. TrILD patients tend to have earlier disease onset, faster progression, and worse prognosis causing them to be referred for LTX more often.