IPF-PRO Registry data sheds light on characteristics of people with idiopathic pulmonary fibrosis

Oct 23 2015

Boehringer Ingelheim Pharmaceutical, Inc. today announced initial results of the first 49 people enrolled in the IPF-PRO Registry, which shed light on characteristics of people with IPF at the time of diagnosis. Results will be presented at the American College of Chest Physicians Annual Meeting (CHEST 2015) on October 26 in Montreal, Canada.

The IPF-PRO Registry is an academic-industry alliance between Boehringer Ingelheim Pharmaceuticals, Inc. and the Duke Clinical Research Institute to better understand outcomes and disease progression for people with IPF, a rare and fatal lung disease.

“We are very excited to share this first look at real-world patients with IPF across 18 IPF academic centers in the United States. Over time, we look forward to helping the IPF community learn more about disease progression, quality of life and other outcomes that are important to patients,” said lead study author Michael Durheim, M.D., Medical Instructor, Department of Medicine, Duke Clinical Research Institute. “As this alliance continues, our objective is to advance the understanding of this devastating disease, through additional findings about diagnosis, treatment patterns and whether blood or genetic markers may impact patient outcomes.”

From an evaluation of the first 49 people with IPF enrolled in the registry (NCT01915511; abstract 362A), the results showed:

• Most people exhibited symptoms of IPF for more than a year before being diagnosed.
• By the time of enrollment, many people exhibited considerably impaired lung function, with a median forced vital capacity (FVC, or the amount of air that can be exhaled after maximum inhalation) of 72 percent (61 to 81 percent) predicted and diffusing capacity of carbon monoxide (DLCO, or the measure of the lungs’ ability to transfer oxygen to red blood cells) of 39 percent (34 to 48 percent) predicted.
• Twenty-nine percent (14 patients) required supplemental oxygen when resting, and 45 percent (22 patients) required supplemental oxygen during activities.
• Nearly all patients (98 percent, 48 patients) received an imaging test known as high resolution CT scan as part of their diagnosis, while 20 percent (10 patients) also underwent surgical biopsy to examine for signs of IPF.
• The most commonly reported comorbidities were gastroesophageal reflux disease (GERD) (69 percent), coronary artery disease (31 percent) and sleep apnea (29 percent).

“These results showed us that many patients already have significant respiratory impairment by the time they are diagnosed by a pulmonologist, which reinforces what we know from ongoing research,” said Danny McBryan, M.D., vice president, Clinical Development and Medical Affairs, Respiratory, Boehringer Ingelheim Pharmaceuticals, Inc. “The Registry emphasizes the critical need to recognize IPF earlier and send patients to a specialist faster to determine diagnosis and care.”