Patricia Inácio, PhD, science writer —

Researchers identified a molecular pathway called ER stress, and a molecular factor known as PI3K, as promoters of pulmonary fibrosis in mice. This research suggests that PI3K and ER stress inhibitors are potential therapeutic options for pulmonary fibrosis. The study reporting the findings, “Involvement of ER stress, PI3K/AKT activation,…

Health Canada has issued a safety warning on Boehringer Ingelheim’s idiopathic pulmonary fibrosis therapy Ofev (nintedanib) after patients in a number of countries developed liver injuries and one died. IPF patients should stop taking Ofev and seek medical assistance if they develop signs of liver damage, Canadian officials said. Those…

Bellerophon Therapeutics has enrolled the first pulmonary hypertension patient in a Phase 2b clinical trial of its INOpulse nitric oxide delivery system. The trial (NCT03267108) of the blood-vessel-widening device will cover people with pulmonary hypertension associated with interstitial lung disease, or PH-ILD. This includes people with idiopathic pulmonary fibrosis,…

Throughout 2017, Pulmonary Fibrosis News Today reported daily discoveries, therapeutic developments, and events related to pulmonary fibrosis. Here, we review the top 10 most-read articles of 2017, with a brief description of what made them interesting and relevant to PF patients, family members, and caregivers. No. 10 – Esbriet’s Efficacy…

A factor called Krüpple-like-factor 4, or KLF4, treated lung fibrosis in a mouse model of idiopathic pulmonary fibrosis (IPF) by preventing epithelial cells of the lung from moving into a fibrosis-promoting state, researchers in China report, suggesting its potential as an IPF therapy. The study “Krüpple-like-factor 4 Attenuates Lung…

A team of researchers discovered that a protein called RAGE can fix damage inflicted to DNA molecules, and also repair lung scarring in mice. The study reporting the findings, “Homeostatic nuclear RAGE-ATM interaction is essential for efficient DNA repair,” was published in the journal Nucleic Acids Research.

Radiation targeting the lungs damages alveolar type 2 cells, causing radiation-induced fibrosis — but a factor called Nrf2 is key in helping those cells recover, researchers at Nashville’s Vanderbilt University Medical Center found. Their study, “Loss of Nrf2 promotes alveolar type 2 cell loss in irradiated, fibrotic…

Esbriet (pirfenidone) seems to have diminishing therapeutic effect after six months of treatment and possibly no benefit after one year in idiopathic pulmonary fibrosis (IPF) patients with advanced disease, a Greek retrospective study shows. The study, “Safety and efficacy of pirfenidone in severe Idiopathic Pulmonary Fibrosis: A real-world observational…

Results from the RECAP clinical trial, which was conducted over five years, support the long-term safety of Esbriet (pirfenidone) in patients with idiopathic pulmonary fibrosis (IPF). The study, “An Open-Label Study of the Long-Term Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (RECAP),” was recently published in…

A small bacterial protein called R1R2 reduced fibrosis-promoting cells’ ability to go from the blood, where they normally reside, to lung tissue, a mouse study shows. The research, “R1R2 peptide ameliorates pulmonary fibrosis in mice through fibrocyte migration and differentiation,” was published in the journal PLOs One. Scientists…