PFF Registry Drives Strides in Pulmonary Fibrosis Research

A host of new research studies have increased understanding of pulmonary fibrosis (PF) and interstitial lung disease (ILD) thanks to information gathered by the PFF Registry, a program comprised of clinical and community databases. Launched in 2016, the PFF Patient Registry is a database of physician-reported medical information from more than 2,000 patients who obtain care at participating PFF Care Centers across the United States. Researchers have used these data to advance the understanding of PF and ILD with the goal of accelerating the development of treatment options and improving outcomes for all of those affected by these diseases.

Data collected from the Patient Registry have served as a vital source of information for over 40 developing or completed research studies, many of which have resulted in abstracts, posters, and published manuscripts on topics such as lung transplantation, disparities, and impacts of patient clinical characteristics.

To continue expanding the understanding of PF, the PFF recently introduced another database, the PFF Community Registry, which relies on self-reported information from patients and lung transplant recipients who have been diagnosed with PF or ILD, as well as their caregivers and biological family members. There are more than 1,100 participants enrolled in the Community Registry, which continues to grow.

“There is no limit to the number of participants in the PFF Community Registry, and data will be used to advance research on topics including patient perceptions about their care and diagnosis,” said Junelle Speller, MBA, Vice President of the PFF Registry. “The PFF Registry databases are gathering a wealth of longitudinal data on all forms of PF and ILD, providing unparalleled insight into the experience of patients, lung transplant recipients, caregivers, and family members.”

More research on PF and ILD is critical to help expand treatment options and answer key questions about these devastating diseases. Dr. Kevin Flaherty, Steering Committee Chair for the PFF Registry said, “We encourage researchers and providers alike to use PFF Registry data and other PFF resources to examine the many complex presentations of PF and ILD and their effects on different populations.”

PF Health Disparities Impact Black and Hispanic Patients

A study published this month in JAMA Network Open examined differences in age at onset of pulmonary fibrosis outcomes among Black, Hispanic, and White adults in the U.S. Ayodeji Adegunsoye, MD, a 2018 PFF Scholar, and his colleagues used a cohort of 1,904 patients from the PFF Patient Registry and validated that cohort with data from four distinct medical centers across the U.S. In their manuscript, “Evaluation of Pulmonary Fibrosis Outcomes by Race and Ethnicity in U.S. Adults,” found that Black patients face earlier diagnosis, earlier hospitalization, earlier lung transplant, and earlier death than Hispanic and White patients. The study also found that White patients predominantly had idiopathic pulmonary fibrosis, most Hispanic patients had hypersensitivity pneumonitis, and Black patients had more autoimmune related pulmonary fibrosis.

PRECISIONS Study Investigating Whether NAC May Provide Clinical Benefit

In partnership with the PFF, the PRECISIONS study is supported by a $22 million grant from the National Institutes of Health (NIH) and the Three Lakes Foundation, a philanthropic family organization. Flaherty and PFF Chief Medical Officer Joseph Lasky, MD, in collaboration with the PRECISIONS team, presented insights on a clinical trial centered upon “Design and Rationale for the Prospective Treatment Efficacy in IPF Using Genotype for NAC Selection.” The paper focused on use of N-acetylcysteine (NAC), an inexpensive and well-tolerated agent for treatment of lung and other organ diseases as treatment for IPF. The study is investigating whether NAC provides clinical benefit in the quarter of patients with IPF that carry a specific genetic variant, the TOLLIP rs3750920 TT genotype.

Study Links Air Pollution to Increased Mortality and Worse Baseline Lung Function

A study published in JAMA Internal Medicine online in October cross-referenced satellite-based pollution data with medical data from 6,500 patients with ILD, including 1,800 in the PFF Patient Registry. The paper, “Association of Particulate Matter Exposure with Lung Function and Mortality Among Patients with Fibrotic Interstitial Lung Disease,” found that exposure to particulate matter 2.5 μm or less in diameter (PM_2.5) is associated with adverse outcomes among patients with ILD regarding baseline severity, disease progression, and mortality. Sulfate, ammonium, and nitrate constituents were also associated with the most harm, highlighting the need for reductions in human-derived sources of pollution. The study was led by Gillian Goobie, MD, a 2020 PFF Scholar while at the University of Pittsburgh, who is now an Assistant Professor at the University of British Columbia.

Rural Patients Seen at PFF Clinical Sites Not at Greater Risk than Those in Urban Areas

Hyun Kim, MD, of the University of Minnesota, presented findings from her abstract, “Outcomes in Interstitial Lung Disease Patients Living in Economically Disadvantaged and Rural Areas: An Analysis of the Pulmonary Fibrosis Foundation Registry,” at the October 2022 CHEST conference.  Kim and her colleagues analyzed PFF Registry data and found that patients with ILD in rural areas do not appear to have greater risk for death, lung transplant, acute exacerbation, or disease progression compared with those in urban areas. 

To learn more about the PFF Registry, visit pffregistry.org. Researchers seeking data on PF and ILD can find the latest research updates by visiting pulmonaryfibrosis.org/pff-registry/for-researchers.

For more information about PF symptoms, risk factors, and treatment options, visit AboutPF.org.  

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About the Pulmonary Fibrosis Foundation

The mission of the Pulmonary Fibrosis Foundation is to accelerate the development of new treatments and ultimately a cure for pulmonary fibrosis. Until this goal is achieved, the PFF is committed to advancing improved care of patients with PF and providing unequaled support and education resources for patients, caregivers, family members, and health care providers. The PFF has a three-star rating from Charity Navigator and is an accredited charity by the Better Business Bureau (BBB) Wise Giving Alliance. The Foundation has met all of the requirements of the National Health Council Standards of Excellence Certification Program® and has earned the Guidestar Platinum Seal of Transparency. For more information, visit pulmonaryfibrosis.org or call 844.TalkPFF (844.825.5733).