Immune Suppression and Telomere Length on IPF Survival

The PANTHER-IPF trial was a large clinical study funded by the National Heart, Lung, and Blood Institute that examined the impact of prednisone, azathioprine and N-acetylcysteine (NAC) on IPF. Years ago, the NHLBI stopped the trial early due to safety concerns (higher mortality and serious side effects) and a lack of benefit.

The multi-institutional reseach study, "Telomere Length and Use of Immunosuppressive Medications in Idiopathic Pulmonary Fibrosis," published in the American Journal of Respiratory and Critical Care Medicine, aimed to determine whether the harm some IPF patients experienced from the immune-suppressive therapy may be related to telomere length in white blood cells. Telomere length was measured in white blood cells from people who participated in the PANTHER trial; those in the ACE-IPF trial, which studied anticoagulant effectiveness in IPF (also stopped early due to safety); and an independent IPF group. The researchers compared survival between groups.

A majority of patients in both the PANTHER and ACE groups had below-normal telomere lengths. This was associated with greater mortality, lung transplantation, hospitalization and lung function decline. The same was found for those IPF patients in the third group who had shorter telomeres. While it was observed that those with short telomeres who received immunosuppressants had bad outcomes, the harmful effect was not as strong in those with normal-length telomeres.

The researchers concluded that white blood cell telomere length may be useful for identifying IPF patients at risk for negative outcomes if given immune-suppressing treatment.

Adapted from pulmonaryfibrosisnews.com