Boehringer Ingelheim Pharmaceuticals, Inc., recently released a report highlighting the first batch of findings based on 49 idiopathic pulmonary fibrosis patients enrolled in the IPF-PRO Registry — an academic-industry partnership between Boehringer and the Duke Clinical Research Institute designed to better understand IPF patients’ health characteristics once diagnosed with the disease. The preliminary results are being presented at the American College of Chest Physicians Annual Meeting (CHEST 2015) on October 26 in Montreal, Canada.

“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, MD, 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.”

According to a press release, observations so far of the first 49 IPF patients in the registry (NCT01915511; abstract 362A) have found that:

• It took most people with IPF symptoms more than a year to receive an accurate diagnosis.
• Many of the IPF patients were at the time of enrollment already experiencing significantly 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, MD, vice president, Clinical Development and Medical Affairs, Respiratory, with Boehringer Ingelheim. “The Registry emphasizes the critical need to recognize IPF earlier and send patients to a specialist faster to determine diagnosis and care.”

The five-year registry is now actively recruiting 300 people with a diagnosis of IPF at 18 centers specialized in the treatment of  lung disease.