Fibrosis File

Exciting New IPF Research!

Reporting in Modern Pathology, Ohio State University researchers have found Idiopathic Pulmonary Fibrosis (IPF) might be caused by a virus. Evidence of the pathogen, herpesvirus saimiri, was found in all tissue samples from patients with IPF, but in none of those from patients with fibrosis with a known cause. Interventions aimed at stopping the growth and spread of herpesvirus saimiri might stabilize IPF. The scientists feel that discovering the origin of IPF gives us hope that both testing and treatments will be developed to help patients survive and live with IPF. Pulmonary fibrosis affects about one million people in the United States of which IPF represents about 20% of cases. There is currently no effective way to diagnose IPF prior to the symptoms appearing and treatments have not proven very effective.

In other news, TOLLIP, a gene called toll interacting protein, plays a role in regulating immunity to certain stimuli – meaning how well you can fight off disease and infection when exposed to things that may cause the problems. An abnormal form of this gene was found in the lungs of those with IPF by Chicago researchers who published their study in The Lancet Respiratory Medicine. This finding suggests that an abnormal immune response, possibly to infectious agents or even environmental injury, may be central to the disease. If an IPF patient has this variant, physicians might want to consider lung transplantation early in the course of the disease. The genetic markers would reveal who might need a transplant quickly. Dr. Joe Gacia, an author on this study, thinks researchers can now focus on understanding the role of the variants found in humans, and drug companies can assess whether they already have drugs that affect these pathways.

Dr. Marilyn Glassberg of the University of Miami has obtained approval from the FDA to launch the first U.S. clinical trial that will test whether mesenchymal stem cells given intravenously could be a therapy for IPF patients. Glassberg says, if successful, stem cells could be applied as a potential therapy for other, more common lung diseases such as asthma or emphysema. The first phase of the clinical trial in the next few months has nine patients receiving intravenous doses of donor stem cells during a hospital stay. The second phase will involve 16 patients, some of whom will receive the stem cell therapy while others will receive a placebo. The third and final phase is a larger study with about 300 patients worldwide. In tests with those with heart disease, it was found the stem cells led to a scar tissue reduction in the heart. Glassberg postulates it would make sense that the same effect would take place with fibrosis in the lungs.

The Coalition of Pulmonary Fibrosis (www.coalition has a program called Daughters of Pul­ monary Fibrosis.

image104They met recently and came up with ways to raise awareness which include having those with the problem, along with relatives and friends, speak out about their experiences with the disease in person, by mail and through social media. You might contact local media including newspapers, radio and television stations or organize an event in honor of someone with IPF such as a school basketball tournament or dinner. Talk to your co­ workers, elected officials and community organizations. If you would like advice on how to become an advocate or to join in the Daughters of PF program, please email Teresa Barnes at [email protected] or call (888) 222­8541, ext. 702.

We will bring you highlights from the Pulmonary Fibrosis Foundation’s PFF Summit (www.pulmonary in our next issue!