Patricia Inácio, PhD,  science writer—

The U.S. Food and Drug Administration (FDA) has granted orphan drug status to SM04646, a therapy developed by San Diego-based Samumed to treat idiopathic pulmonary fibrosis (IPF). This designation provides several benefits, including tax incentives, exemption from the FDA user fee and potential market exclusivity for seven years following approval. SM04646,…

Australia’s national registry of patients with idiopathic pulmonary fibrosis (IPF) includes patients of diverse ages, disease severity and co-morbidities, and shows that baseline lung function and GAP (gender, age, physiology) stage are important prognostic markers for mortality. The study, “Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the…

Esbriet (pirfenidone) stops the lung scarring in idiopathic pulmonary fibrosis (IPF) by improving the functioning of mitochondria, cell components that convert food to energy and regulate cell death, according to a study. The therapy centers on mitophagy, the process by which the body purges defective mitochondria. Mitophagy is a mitochondria quality…

Lung Therapeutics announced June 6 that it has completed a $14.3 million Series B financing round to support development of LTI-03, its lead therapy for idiopathic pulmonary fibrosis (IPF). LTI-03 has already showed promising results for other conditions including scleroderma and cardiac fibrosis. The funds will also support clinical trials, now underway in Australia and…

Natural gene variants, called polymorphisms, may explain why some patients with systemic sclerosis also develop pulmonary fibrosis (PF). The study, “The status of pulmonary fibrosis in systemic sclerosis is associated with IRF5, STAT4, IRAK1, and CTGF polymorphisms,” appeared in the journal Rheumatology International. PF is one of the leading…

An investigative therapy for idiopathic pulmonary fibrosis (IPF) based on inhibiting two proteins, including the cannabinoid CB1 receptor, shows potential for halting fibrosis progression, and greatly improved survival in a mouse model for human IPF. The study, “Cannabinoid CB1 receptor overactivity contributes to the pathogenesis of idiopathic pulmonary fibrosis,” was published…

Antacid therapy (AAT) may lead to major digestive problems and severe lung infections in idiopathic pulmonary fibrosis (IPF) patients who take Esbriet (pirfenidone), a study reports. The research, “Antacid Therapy and Disease Progression in Patients with Idiopathic Pulmonary Fibrosis Who Received Pirfenidone” was published in the journal…

A new imaging strategy shows promising results in improving the diagnosis and treatment of people with pulmonary fibrosis (PF). The study, “Type I collagen-targeted PET probe for pulmonary fibrosis detection and staging in preclinical models,” appeared in the journal Science Translational Medicine. Scientists at Massachusetts General Hospital (MGH) have developed…

Using Esbriet (pirfenidone) or Ofev (nintedanib) for a year reduces lung function decline in idiopathic pulmonary fibrosis (IPF) patients, according to a review of nine clinical trials. Esbriet may also improve patients’ survival. But N-acetylcysteine failed to improve lung function or decrease mortality, the comparative study indicated. The article, “…

The LOXL2 protein plays a critical role in the development of idiopathic pulmonary fibrosis (IPF) by helping promote lung tissue scarring, according to a study. LOXL2 is one of a family of proteins important to extracellular matrix (ECM), a non-cell component essential for providing structural and biochemical support to tissues and organs. LOXL2…