New results from patient registry reveal insights about IPF

October 22, 2015 – Initial results from the IPF-PRO Registry will be presented at the American College of Chest Physicians Annual Meeting later this month.

Boehringer Ingelheim Pharmaceutical, Inc. today announced initial results of the first 49 people enrolled in the Idiopathic Pulmonary Fibrosis – PROspective Outcomes (IPF-PRO) Registry, which shed light on characteristics of people with idiopathic pulmonary fibrosis (IPF) at the time of diagnosis. The results will be presented at the American College of Chest Physicians Annual Meeting on October 26 in Montreal, Canada.

michael-durheim-news IPF-PRO Registry is an academic-industry alliance between Boehringer Ingelheim and the DCRI 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,” said the DCRI’s Michael Durheim, MD, the study’s lead author (pictured). “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. 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, 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, MD, vice president, Clinical Development and Medical Affairs, Respiratory, at Boehringer Ingelheim. “The Registry emphasizes the critical need to recognize IPF earlier and send patients to a specialist faster to determine diagnosis and care.”

IPF is characterized by inflammation and scarring of lung tissue and loss of lung function over time. Development of scarred tissue is called fibrosis. Over time, as the tissue thickens and stiffens with scarring, the lungs lose their ability to take in and transfer oxygen into the bloodstream, and vital organs do not get the oxygen they require. Individuals with IPF experience shortness of breath and often have difficulty participating in everyday physical activities.

Research indicates that IPF may affect as many as 132,000 people in the United States and the patient population may be increasing. Most patients with IPF die from the disease within three to five years of diagnosis.

The IPF-PRO Registry is a prospective, multi-center effort designed to better understand the natural progression of, and treatment approaches for, IPF. IPF-PRO is a unique collaboration between Boehringer Ingelheim and the DCRI designed to build on the knowledge of single-center studies and clinical trials.

The five-year registry is actively recruiting 300 patients with IPF diagnosed at 18 centers specialized in treating lung disease. The primary measures include: characterizing and describing the natural history of recently-diagnosed patients; understanding current practice patterns; and describing the impact of IPF on patient quality of life. The registry also will include a biomarker bank to identify potential blood or genetic markers of the disease that correlate with patient outcomes.