Lisa Lancaster

TL;DR: Pirfenidone, as compared with placebo, reduced disease progression, as reflected by lung function, exercise tolerance, and progression-free survival, in patients with idiopathic pulmonary fibrosis.

TL;DR: The primary objective in the Bosentan Use in Interstitial Lung Disease-3 trial was not met and bosentan was well tolerated.

TL;DR: Short telomeres are a signature in IIPs and likely play a role in their age-related onset, and the clustering of cryptogenic liver cirrhosis with IPF suggests that the telomere shortening can contribute to what appears clinically as idiopathic progressive organ failure in the lung and the liver.

TL;DR: The results suggest that genes involved in host defense, cell-cell adhesion and DNA repair contribute to risk of fibrotic IIPs.

TL;DR: FVC is a reliable, valid, and responsive measure of clinical status in patients with IPF, and a decline of 2-6%, although small, represents a clinically important difference.