The Burden of Idiopathic Pulmonary Fibrosis

In a recent presentation at AMCP, Dr Steven D Nathan, MD, FCCP, Inova Fairfax Hospital, discussed the effects of forced vital capacity (FVC) decline and suspected early acute idiopathic pulmonary fibrosis (IPF) exacerbation on utilization and outcomes. Dr Nathan highlighted two recent studies examining FVC and IPF.

The first study, published in Respiratory Medicine [Respiratory Medicine. 2015;109:1582-1588], found that patients who have suspected acute exacerbations of IPF within 6 months of IPF diagnosis are more likely to experience worse subsequent clinical outcomes and require more health care services than patients who do not have early acute exacerbations.

Specifically, the study links suspected acute exacerbations after IPF diagnosis with higher rates of mortality, subsequent acute exacerbations, and acute care services related to the pulmonary disease.

Early acute exacerbations of IPF increased the risk of death 2.9 times and the rate of subsequent acute exacerbations 3.9 times, researchers reported. Additionally, early acute exacerbations were linked with a 4.4-fold increase in the rate of emergency department visits and an 8-fold increase in the rate of hospitalizations.

Researchers came to their findings through a retrospective chart review of 490 patients with IPF. Patient data was submitted by 168 pulmonologists from across the United States via an online case report form. To be eligible for inclusion, patients had to be age 40 years or older with a confirmed date of first IPF diagnosis and a high-resolution computed tomography or lung biopsy taken between January 1, 2011, and June 30, 2013. Pulmonary function tests using FVC must have been recorded near the time of initial IPF diagnosis and, again, 6 months later.

The second study, published in BMC Pulmonary Medicine [BMC Pulmonary Medicine. 2015;15:167], found that a greater decline in lung function in the 6 months following a diagnosis of IPF was linked to a higher risk of further disease progression, suspected acute exacerbation, and mortality in a chart review study of 490 patients.

The study used chart data extracted by 168 treating pulmonologists from a variety of practice settings and regions across the United States. Patients age 40 and older were eligible for chart abstraction if they had a confirmed date of IPF diagnosis and a high-resolution computed tomography or lung biopsy between January 1, 2011, and June 30, 2013. In addition, FVC tests measuring pulmonary function had to occur around the time of initial IPF diagnosis and 6 months afterward.

Researchers looked at the relative change in FVC percent predicted (FVC%) during the first 6 months after IPF diagnosis and categorized patients into 3 separate groups: stable (a decline of less than 5%), marginal decline (a decline between 5 and 9%), and significant decline (a decline of 10% or more). Among the study population, 50% were stable, 20% experienced marginal decline, and 29% experienced significant decline during the first 6 months.

Researchers then looked at outcomes and health care resource utilization for each group from 6 months following diagnosis to the last point of contact with the physician. That observation time averaged 583 days.

After adjustment for demographic and clinical characteristics, the findings suggested greater FVC% decline is linked with higher risk of suspected acute exacerbation, hospitalization, and death. 

This presentation was sponsored by Boehringer Ingelheim Pharmaceuticals.