Early progression of familial pulmonary fibrosis

Doctor inspects and examines CT scan of chest, lungs.

Familial pulmonary fibrosis (FPF) is a genetic, severe form of idiopathic pulmonary fibrosis (IPF) that progresses quickly in many individuals. Researchers from Vanderbilt University Medical Center (VUMC) recently evaluated asymptomatic relatives of patients with FPF to determine early risk factors that indicate disease progression.

The study, “Progressive Early Interstitial Lung Abnormalities in Persons At-Risk for Familial Pulmonary Fibrosis,” was published in the American Journal of Respiratory and Critical Care Medicine

Margaret Salisbury, MDMargaret Salisbury, MDVUMCUsing high resolution chest CT scans, the research team detected minor early changes in participants’ lungs that could signal initial progression of FPF. This is a critical advancement for individuals who have not yet developed symptoms.

Typically, people are not diagnosed with the condition until they have known side effects, such as cough and shortness of breath. By the time a symptomatic patient has been diagnosed, their survival time is often three to five years. 

The study identified individuals for the study using a VUMC registry of families with FPF. The relatives were between the ages of 40 and 75 and were available to periodically come to VUMC for screenings and scans, pulmonary function tests and blood tests. 

“The main goal of the study is to evaluate characteristics, particularly CT findings, that are associated with subclinical disease — abnormal changes on CT that are getting worse over time or the development of symptomatic pulmonary fibrosis in these at-risk individuals,” said Margaret Salisbury, MD, first author of the study and assistant professor of medicine at VUMC, in a university news article.

This is an ongoing study that is still enrolling people who are genetically at-risk of FPF. National Jewish Health in Denver also contributed to initial research.

“Our goal, and the goal of a lot of other people in the field, is to eventually do clinical trials to see if IPF, meaning symptomatic disease, can be prevented,” Dr. Salisbury said. “If we want to prevent something, we have to know what it looks like in its very earliest stages. This study provides very useful information about people that we could target for enrollment in future clinical trials trying to prevent the development of the progression of symptomatic pulmonary fibrosis.”

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