Biological age instead of chronologic age as prognostic factor in IPF: clinical implications of telomere shortening

Extract

Introduction: Accelerated telomere shortening has been described in some degenerative diseases, such as idiopathic and familial pulmonary fibrosis (IPF/FPF), associating poor outcome. The aim of the study was to evaluate clinical implications of telomere shortening in an IPF/FPF cohort.

Methods: Cross-sectional study. Spanish IPF/FPF patients and first line relatives were included and divided by telomere shortening and chronological age. Telomere length was calculated from DNA of mouth epithelial cells and blood lymphocytes and through quantitative polymerase chain reaction and Z-score (mean length±SD) for age-matched controls. Outcome was assessed by mortality and lung transplant. Univariate regression logistic model was used for statistical analyze.

Results: 106 patients (whose characteristics are shown in Table 1) and 20 healthy relatives were included. 33 (31.1%) patients and 20 (100%) relatives were ≤60 years. Telomere shortness was present in 25/33 (75.8%) patients with ≤60 years, 32/73 (44.0%) patients with >60 years and 9 (45%) relatives. Subjects with telomere shortening had higher risk for disease (odds ratio:6.11; 95% confidence interval: 1.12-41.32, p = 0.02). 7/25 (28.0%) patients ≤60 years with telomere shortening underwent lung transplant in less than 2 years. 4 (12.1%) patients ≤60 years died: 3 (75%) had telomere shortening. 1 (11.1%) subclinical case was detected among relatives with telomere shortening.