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February 21, 2005
New drug trial inspires hope for treating IPF lung disease

BY Tia Mccollors

Sooner or later, every breath becomes a struggle for people living with idiopathic pulmonary fibrosis (IPF). Lung transplantation has been the only definite treatment option for this progressive, incurable disease that eventually robs its victims of their ability to breathe.

But researchers at the Emory Center for the Treatment and Study of Interstitial Lung Disease (ILD Center) are participating in the multisite INSPIRE trial to help treat the disease and increase the six-year average life expectancy for IPF patients.

IPF is classified as an interstitial lung disease (ILD), a group of lung conditions characterized by the buildup of scar tissue in the lungs. The disease damages the air sacs of the lower respiratory tract as the scar tissue forms and thickens, irreversibly reducing the transfer of oxygen to the bloodstream. Although no definite cause has been discovered, IPF is thought to be caused by injury or inflammation induced by an unknown agent.

Pulmonary fibrosis is mostly attributed to inhaled environmental exposures, connective tissue, drug toxicity from chemotherapy radiation, diseases such as sclerodoma, lupus or sarcoidosis, and inherited causes. IPF patients face mortality threats from pulmonary hypertension and respiratory failure, according to Jesse Roman, associate professor and director of pulmonary allergy and critical care medicine and director of the ILD Center.

“Many patients are unable to perform their usual daily activities because of shortness of breath. Their lungs continue to deteriorate, and soon they’re also plagued with an unyielding dry cough and appetite loss,” Roman said. “However we hope treatments derived from studies like INSPIRE will improve their quality of life or, ultimately, successfully treat the disease.”

The INSPIRE trial, now under way in 70 centers in North America and Europe, tests the safety and efficacy of Actimmune (interferon gamma-1b), a disease-modifying drug with a low side-effect profile. Interferon gamma-1b is a cytokine produced naturally by the body and has anti-fibrotic properties (i.e., it helps prevent thickening or scarring of connective tissue). A number of treatment options for IPF are currently in development, but the Phase III trial testing interferon gamma-1b is currently the most advanced.

“We strive to provide patients and their physicians with advanced experimental treatment regimens,” Roman said. “We’re enthusiastic about discoveries that will allow us to develop new and better therapies for their lung condition.”

INSPIRE is a Phase III trial sponsored by InterMune. The study is recruiting 600 IPF patients between the ages of 40 and 79 for a two-year evaluation period. Patients are randomized at a 2:1 ratio into either a placebo group or a group receiving interferon-gamma subcutaneously three times weekly for two years. A physical examination, vital signs, electrocardiogram, hematology, urinalysis and blood chemistry profiles will evaluate safety, and a high-resolution computerized tomography of the chest will be performed. Adverse events, including infections and death, will also be recorded. The primary efficacy outcome measure of the study will be survival time from date of randomization in patients diagnosed with IPF.

According to the Coalition for Pulmonary Fibrosis, an estimated 31,000 cases of IPF are diagnosed each year, and approximately 83,000 Americans suffer from the disease. Some two-thirds of patients die within five years of diagnosis.

Anyone interested in participating in the trial should contact study coordinator Patricia Alvarez at 404-727-6821.

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