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First Results Released from Lung Fibrosis Registry

CT scan of lungs with IPF

High-resolution CT scan of lungs with idiopathic pulmonary fibrosis (IPF Editor, Wikimedia Commons)

22 October 2015. The first results from a registry of individuals with idiopathic pulmonary fibrosis show most people in the group have limited lung function and show symptoms for about a year before the disease is diagnosed. These findings and other characteristics of people taking part in the Idiopathic Pulmonary Fibrosis – PRospective Outcomes (IPF-PRO) Registry will be presented next week at the annual meeting of American College of Chest Physicians in Montreal.

Idiopathic pulmonary fibrosis is a chronic, progressive lung disease, usually affecting people between the ages of 50 and 70. The disorder results in fibrosis or scar tissue building up in the lungs, limiting the ability of lungs to transfer oxygen to the blood stream. The most common symptoms are shortness of breath and a dry hacking cough, as well as a loss of appetite and weight loss in some cases. The scarring of lung tissue increases over time, often leading to other serious lung conditions, including lung cancer and blood clots in the lungs.

From 13 to 20 per 100,000 people worldwide experience idiopathic pulmonary fibrosis. Some 100,000 people in the U.S. have the condition, with 30,000 to 40,000 new cases diagnosed each year. Most patients die within five years following diagnosis.

IPF-PRO is a project of the pharmaceutical company Boehringer Ingelheim and Duke Clinical Research Institute, a division of Duke University’s medical school that conducts clinical studies and manages patient registries. The registry began in April 2014 and is expected to run for five years, enrolling up to 300 individuals with idiopathic pulmonary fibrosis. Participants in the registry agree to allow the progression of their disease be tracked over this period, as well as provide blood samples for testing, including DNA analysis, every six months.

The registry so far enrolled 49 individuals. Those first participants indicate, at the time they enrolled, their disease already progressed to the point their capacity to exhale air, after a maximum inhalation, was reduced by an average of 28 percent. In addition, their  lungs’ ability to transfer oxygen to red blood cells was, on average, only 39 percent of normal.

IPF-PRO participants say they displayed symptoms of the disease for about a year before their diagnosis. Among nearly all participants (98%) that diagnosis consisted of a high-resolution computerized tomography or CT scan, while 10 of the 49 participants (20%) also had a surgical biopsy.

Moreover, participants in the registry report a marked degradation in the quality of their lives. Nearly half (45%) of the individuals say they need supplemental oxygen when engaging in activities, while 19 percent indicate a need for more oxygen even when resting. Large percentages also report other disorders, including gastroesophageal reflux disease (69%), coronary artery disease (31%), and sleep apnea (29%).

Michael Durheim, an instructor at Duke medical school and lead author of the report, says in a joint statement that the registry plans to continue collecting data on diagnosis and treatment patterns, but relate those data to blood and genetic markers, as well as patient outcomes.

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