Researchers at the Medical School of Nanjing University in China recently published in the journal Respirology findings that reveal how shorter telomeres in chromosomes are associated with a poor survival in patients with idiopathic pulmonary fibrosis (IPF). The study is entitled “Association between telomere length and survival in patients with idiopathic pulmonary fibrosis.”
IPF is a progressive fatal lung disease in which the alveoli and the lung tissue are damaged, becoming thick and scarred (fibrosis), leading to severe breathing difficulties and compromising oxygen transfer between the lungs and the bloodstream. IPF is characterized by a shortness of breath that gradually worsens, with respiratory failure being the main cause of death associated with the disease. There is no cure for IPF and it is estimated that 128,000 individuals in the United States suffer from the disease, with approximately 48,000 new cases diagnosed every year. IPF has a poor prognosis and around two-thirds of the patients die within five years after being diagnosed.
Telomeres are the caps at the end of each DNA strand in chromosomes, which protect the edge of the chromosomes from degradation and from fusion with other chromosomes. Short telomeres have been reported to be a risk factor for IPF development, although the possible link between telomere length and survival rate in IPF patients has been poorly studied. In this study, researchers assessed whether telomere length is associated with survival in IPF patients.
The team measured the telomere length of leukocytes (white blood cells) in 94 IPF patients enrolled in the study between January 2012 and June 2014. Researchers found that the average telomere length of IPF patients was significantly shorter in comparison to the one of healthy controls. During the follow-up period, 43 IPF patients died. Interestingly, the team found that the telomere length in the IPF patients who died during the study was significantly shorter in comparison to the telomere length in IPF patients who survived. The team reported that this association between telomere length and patient survival was independent of age, sex or forced vital capacity (the amount of air a person can forcibly exhale after taking a deep breath).
The research team concluded that telomere length is an independent predictor of survival in patients with IPF, where a shorter telomere length is associated with a poorer survival rate. The team suggests that future studies should address the molecular mechanisms behind the shortening of telomere length in IPF patients.