Marta Figueiredo, PhD, managing science editor —

People with rare disorders have a worse healthcare experience than those affected by chronic diseases, according to the results of an international survey conducted by Eurordis-Rare Diseases Europe. Indeed, rare disease patients overall give their healthcare experience a medium-low rating, of 2.5 on a scale of 1 to 5,…

The Pulmonary Fibrosis Foundation (PFF) is highlighting for smokers, and ex-smokers, the need to be aware of pulmonary fibrosis (PF) and their higher risk of developing this disease. It is well-established that tobacco smoke contains many chemicals harmful to health, and its use is linked to cancer, cardiovascular disease, and…

Suppressing MBD2, a DNA-binding protein, reduced the number of pro-fibrotic macrophages — a type of immune cell involved in pulmonary fibrosis (PF) progression — and lessened lung injury and scarring in a mouse model of PF, a…

A Phase 3 clinical trial evaluating Bellerophon Therapeutics’ investigational therapy INOpulse — inhaled nitric oxide — in patients with pulmonary fibrosis (PF) at risk of developing pulmonary hypertension (PH) has enrolled its first patient. Top-line data from the trial, called REBUILD (NCT03267108), are expected by 2022. “We…

The first wave of COVID-19 in Europe severely disrupted access to care and raised stress and anxiety in people with rare diseases, negatively affecting their health and well-being, according to a survey conducted by Eurordis-Rare Diseases Europe. “People living with rare diseases in Europe have found themselves caught as collateral…

FibroGen’s experimental antifibrotic therapy pamrevlumab may be more effective than Esbriet (pirfenidone) and Ofev (nintedanib) at slowing disease progression in people with idiopathic pulmonary fibrosis (IPF), according to a review study. Combined data from eight clinical trials showed that all three therapies significantly…

Treatment with inhalable bone morphogenic protein 4 (BMP4) — a soluble signaling molecule involved in the regulation of cellular identity — restored lung regeneration and improved lung function in a mouse model of idiopathic pulmonary fibrosis (IPF). The data showed that scar-forming cells in IPF lungs suppress BMP signaling…

Idiopathic pulmonary fibrosis (IPF) patients showing a lesser decline in lung function after antifibrotic treatment, especially with Ofev (nintedanib), live significantly longer, according to a study in Japan. Such decline prior to antifibrotic therapy and changes in its rate with treatment did not predict survival outcomes, the researchers noted,…

A cell therapy approach based on transplanting a type of lung cells called type II alveolar cells halted scarring (fibrosis) in a rat model of idiopathic pulmonary fibrosis (IPF) with established disease. This anti-fibrotic effect was associated with a drop in lung levels of several fibrotic markers, the researchers said.

Increasing the levels of a membrane-repair protein called tripartite motif containing 72 (TRIM72) protected against lung cell injury and lessened lung fibrosis in a mouse model of idiopathic pulmonary fibrosis (IPF). These data suggest that targeting TRIM72 or other membrane-repair molecules could be a treatment approach for IPF. The study,…