NEWS RELEASE

For Immediate Release

Contact: Dorothy Coyle, 773-332-6201

(CHICAGO) May 19, 2023 – The Pulmonary Fibrosis Foundation (PFF), the nation’s leading pulmonary fibrosis research, education, and advocacy organization, announced research from its Care Center Network (CCN) and PFF Patient Registry at the American Thoracic Society  (ATS) International Conference 2023, May 19-24, in Washington D.C.

“The PFF is committed to advancing research and accelerating treatments to improve the lives of people living with pulmonary fibrosis and interstitial lung disease,” said Dr. Joseph Lasky, Chief Medical Officer of the PFF. “Data from our Care Center Network and Patient Registry have been vitally important to researchers exploring the many challenging questions about these diseases.”

PFF research presented at ATS 2023 explores cigarette smoking and the risk of ILD; rural and urban differences in access to care and treatment; factors associated with persistence with antifibrotic therapy; and leveraging imaging and biosamples to improve diagnosis and risk of progression in ILD and interstitial lung abnormalities (ILA).

PFF Scholars Dr. Allison DeDent, Dr. Cathryn Lee and Dr. Bhavika Kaul are among the presenters at ATS 2023.  PFF Scholars are early-stage researchers who receive funding and mentoring support for their cutting-edge PF research. Dr. DeDent will present Barriers and Facilitators to Care for Rural Populations with Interstitial Lung Disease: Perspectives From Pulmonary Fibrosis Foundation Care Center Network Providers at a Mini-Symposium and Perceptions of Pulmonary Fibrosis Foundation Care Center Network Providers Caring for Rural Patients With Interstitial Lung Disease at a Thematic Poster Session. Dr. Lee will discuss her work during two Thematic Poster Sessions, Non-Tobacco Exposures in Combined Pulmonary Fibrosis and Emphysema Across ILDs: A Case-Control Analysis and Cigarette Smoking and Risk of Interstitial Lung Disease Diagnosis: A Systematic Review and Meta-Analysis. Dr. Kaul will present at a Meet The Expert Session, Leveraging Big Data To Bridge The Knowledge-Outcome Gaps in Rare Lung Disease.

Access to care and disparities based on geography were the focus of three studies developed by the PFF’s Care Center Network Rural Health Group. Data from the PFF Patient Registry, an observational database established in 2016 that followed more than 2,000 patients with ILD at PFF Care Center Network (CCN) sites was also used in several studies.

Poster #14023: Rural Versus Urban Disparities in Access to Care and Treatment of Interstitial Lung Disease: An Analysis of the Pulmonary Fibrosis Foundation Patient Registry

R. Boente, A.M. DeDent, Z. Li, J.E. Shore, S. Schaht, H. Kim

Thematic Poster Session C39 – Care and Evaluation of Patients with Interstitial Lung Disease

Tuesday, May 23, 11:30 a.m.-1:15 p.m.

Summary: Data from the PFF Registry suggests that disparities in access to care exist for ILD patients in rural areas, however, they did not lead to more frequent hospitalizations or emergency department visits. Larger studies with more rural-inclusive databases are needed to better understand disparities and how to improve access to care for rural patients living with ILD.

Poster #11244: Barriers and Facilitators to Care for Rural Populations With Interstitial Lung Disease: Perspectives From Pulmonary Fibrosis Foundation Care Center Network Providers

A.M. DeDent, J. Shore, R. Bascom, J. Reichuber, M. Scholand, R. Boente, T. Kulkarni, H. Kim, the Pulmonary Fibrosis Foundation Rural Health Outreach Committee

Mini Symposium A94 – Behavioral Science and Health Services Research

Sunday, May 21, 2:51-3:03 p.m.

Summary: Providers in the PFF Care Center Network were surveyed and though most providers within the CCN reported similar ILD visit frequency for all patients, regardless of where they lived (rural or urban), many identified important barriers affecting the diagnosis and treatment of rural patients with ILD.

Poster #9763: Perceptions of Pulmonary Fibrosis Foundation Care Center Network Providers Caring for Rural Patients With Interstitial Lung Disease

A.M. DeDent, J.E. Shore, R. Bascom, J. Reichuber, M. Scholand, R. Boente, A.M. Nambiar, T. Kulkarni, H. Kim, the Pulmonary Fibrosis Foundation Rural Health Outreach Committee

Thematic Poster Session C39 – Care and Evaluation of Patients with Interstitial Lung Disease

Tuesday, May 23, 11:30 a.m.-1:15 p.m.

Summary: The PFF CCN Rural Health Outreach Committee examined the perceptions of ILD providers practicing in ILD subspecialty centers across the United States to understand their experience caring for rural populations patients with ILD. Providers identified barriers and facilitators to caring for rural populations with ILD at the patient, provider, and healthcare system-level. Additional studies examining the perceptions of patients and community providers living and practicing in rural areas are needed to better understand these findings as well as the impact of these barriers and facilitators on patient outcomes.

Poster #815: Factors Associated with Persistence with Antifibrotic Therapy in Patients with IPF Enrolled in the PFF Patient Registry

T. Kulkarni, K. Flaherty, S. Gupta, Y. Tu, A. Hajari Case

Poster Discussion Session A102

Sunday, May 21, 2:15-4:15 p.m.

Summary: An analysis employing PFF Registry data identified several differences between patients who remained on antifibrotic therapy (AFT) and those who discontinued treatment. Understanding the characteristics of patients living with IPF who continue therapy in a real-world setting may help inform physicians in their shared decision making and support for patients on AFT.

D91: Looking for the Crystal Ball: Biomarkers to Predict Disease Progression and Mortality in ILD

J. Oldham, Y. Huang, S. Ma, C. T. Lee, J. Kim, J. V. Pugashetti, K. Mou, A. Schwab, M. E. Strek, A. O. Adegunsoye, A. Linderholm, F. J. Martinez, I. Noth

Mini Symposium

Wednesday, May 24, 12-2 p.m.

Presentation: Proteomic Determinants of Idiopathic Pulmonary Fibrosis Survival

12:24-12:36 p.m.

Summary: PFF Registry data was used as a discovery cohort to assist with identifying serum protein biomarkers associated with IPF survival. Over 200 biomarkers were identified, many that were novel and may mechanistically contribute to IPF progression. Additional research is needed to characterize the role each of these proteins play in IPF progression, and whether they may serve as new targets for treatment.

Poster #824: Leveraging Imaging and Biosamples to Improve Diagnosis and Risk Prediction in ILA and ILD

Poster Discussion Series Session A23 – A Proteomic Signature to Predict Rapidly Progressive Idiopathic Pulmonary Fibrosis

Sunday, May 21, 9-11 a.m.

J. Oldham, J. V. Pugashetti, J. Kim, C. T. Lee, Y. Huang, S. Ma, K. Mou, A. Schwab, A. O. Adegunsoye, A. Linderholm, M. E. Strek, F. J. Martinez, I. Noth

Summary: PFF Registry data was utilized as a discovery cohort and further validated with other sources to identify a proteomic signature to identify patients who may have rapidly progressing IPF. These findings suggest a blood test could help to identify patients that may benefit from early transplant referral.

Poster #10552: Non-Tobacco Exposures in Combined Pulmonary Fibrosis and Emphysema Across ILDs: A Case-Control Analysis

C. Lee, R. Strykowski, K.C. Selvan, R. Jablonski, K. Mak, L. Chelala, J.H.Chung, O. Adegunsoye, M.E. Strek

Thematic Poster Session A38 – Advancing Our Understanding and Treatment of ILD

Sunday, May 21, 11:30 a.m.-1:15 p.m.

Summary: Male smokers with idiopathic pulmonary fibrosis (IPF) are often characterized as having combined pulmonary fibrosis and emphysema (CPFE) syndrome. Yet, less is known about non-tobacco inhalational exposures and the prevalence of non-IPF diagnoses in these patients. This single center study observed CPFE in a wide variety of ILD subtypes and found that inhalational co-exposures are common in these patients. The appearance of emphysema on HRCT could potentially be utilized as a radiologic marker for inhalational exposures in ILD. Additional data regarding sex-exposure interactions and relationship to disease progression is needed.

Poster #10739: Cigarette Smoking and Risk of Interstitial Lung Disease Diagnosis: A Systematic Review and Meta-Analysis

C. Lee, S. Elmrayed, S. Gandhi, J. Feary, C. Barber, H. Barners, C. Peters, M.L. Salisbury, K. A. Johannson

Thematic Poster Session A38 – Advancing Our Understanding and Treatment of ILD 

Sunday, May 21, 11:30 a.m.-1:15 p.m.

Summary: In this systematic review and meta-analysis, cigarette smoking was associated with a nearly three-fold odds of ILD diagnosis compared to those who do not smoke. This study adds to the prior findings that smoking is a risk factor for ILD.

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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 to 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).