Affecting some 5 million people worldwide, pulmonary fibrosis is a progressive, fatal lung disease that few survive more than 3-5 years after diagnosis. A new study suggests a protein molecule may slow the disease down. It found patients with high blood levels of the protein – called LYCAT – had better survival and lung function than patients with lower levels.
The team behind the study, from the University of Illinois at Chicago (UIC) College of Medicine, reports the findings in the American Journal of Respiratory and Critical Care Medicine.
Pulmonary fibrosis is a disease that causes the lungs to be covered in scar tissue. As the disease progresses, the shortness of breath that accompanies it gets worse.
The disease can develop after exposure to asbestos and toxic gas, and even radiation treatment for lung cancer. Chronic inflammatory and autoimmune diseases can also lead to it.
Unfortunately, it is often not diagnosed until symptoms emerge, by which time permanent scarring has already occurred.
First author Dr. Long Shuang Huang, postdoctoral research associate in pharmacology at UIC, says the study offers a new target for drugs to treat the disease. Current treatments for slowing the disease down only work in very few patients.
Previous studies have found several genes may be involved in the development of idiopathic pulmonary fibrosis (IPF) – a form of the disease where no cause can be identified.
Dr. Huang and colleagues decided to investigate the role of one of these genes – that codes for a protein called lysocardiolipin acyltransferase (LYCAT) – in the development of IPF.
They measured levels of LYCAT in the blood of IPF patients and found the highest levels were in patients with significantly better lung function and more likely to survive more than 3 years after diagnosis.
Dr. Huang says they wonder if LYCAT is either playing a protective role, or slowing progress of the disease, and perhaps “boosting LYCAT levels in patients with pulmonary fibrosis may be a viable new therapeutic approach to treating the disease.”
The study also examined how LYCAT behaves in mice bred to develop lung tissue scarring. In mice without the gene, scar tissue developed more quickly compared to mice with the gene. And scar tissue developed even more slowly in mice engineered to have higher than normal levels of LYCAT.
The team now wants to look for molecules that spur production of LYCAT.
Medical News Today recently reported a leading respiratory expert, referring to the dramatic rise in cases of idiopathic pulmonary fibrosis, warning that the UK is sitting on a lung disease “time bomb,” and there is an urgent need to develop a quick and easy way to diagnose it.