In a recent study published in the journal Respirology, a team of researchers found that shorter telomeres — which are the protective caps at the end of a cell’s chromosomes — are associated with worse survival in patients with idiopathic pulmonary fibrosis (IPF). This newly-discovered feature could have future implications in designing IPF therapies.
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disease of unknown etiology with a variable and unpredictable course. Patients may remain stable for several years with a slow and gradual decline in pulmonary function, or experience an accelerated, often fatal course known as “acute exacerbations.” Short telomeres have been found to be a crucial risk factor for IPF. However, little is known about the association between baseline telomere length and survival in IPF.
In an attempt to determine whether telomere length is associated with survival in IPF, in the study titled “Association between telomere length and survival in patients with idiopathic pulmonary fibrosis”, Jinghong Dai from the Department of Respiratory Medicine at the Medical School of Nanjing University in China measured Leukocyte telomere lengths in ninety-four IPF patients. The primary endpoint was the survival of the IPF patients from the time of their initial enrollment assessment.
The results showed that the association between telomere length (hazard ratio (HR) 0.470 (95% confidence interval (CI): 0.25–0. 89);P = 0·019) and survival in patients with IPF was independent of age, sex, forced vital capacity or diffusing capacity of carbon monoxide. After excluding the six patients with telomerase gene mutations, telomere length (HR 0.46 (95% CI: 0.24–0.88); P = 0·018) remained an independent predictor of survival time in patients with IPF.
Based on these results, the researchers concluded that short telomere length is independently associated with worse survival in IPF, and the findings support the theory that mutations in the enzyme that ensures the presence of full-length telomeres in each cell play an important role in IPF.
Based on the findings, the team of researchers suggest that future research should focus on the molecular mechanism underlying the shortening of telomere length in IPF. If this mechanism can be targeted, then patients with short telomeres can be more effectively treated, prolonging survival.