Important pulmonary fibrosis research results from five studies examining access to care and outcomes for patients living with PF and interstitial lung disease (ILD) are being presented at ATS 2022, according to the Pulmonary Fibrosis Foundation (PFF).
The research investigated urban and rural differences among patients, hospitalization rates based on ILD type, access to palliative care services and patient outcomes across the PFF Care Center Network. Four of the five studies used data from the PFF Patient Registry, an observational database established in 2016 that follows more than 2,000 patients with ILD at PFF Care Center Network (CCN) sites.
“Research is a top priority for the PFF, and we are pleased that investigators and providers are using the Registry and other PFF resources to shed light on the many complex and debilitating presentations of PF,” said William T. Schmidt, President, and CEO of the PFF.
Highlights of the pulmonary fibrosis research follow:
Differences in Patient Outcomes Across the PFF Care Center Network (Poster 11457)
- Using PFF Patient Registry data, this study suggests that site-level variation exists with respect to key clinical outcomes for patients with ILD managed at PFF CCN sites. This research supports further investigation to identify practice patterns and resources that are associated with improved patient outcomes.
- Authors: J.S. Lee, R. Boente, E. White, C. Baxter, J. E. Shore, H. R. Collard
Hospitalization Rates in Various Interstitial Lung Diseases: An Analysis of the Pulmonary Fibrosis Foundation (PFF) Patient Registry (Poster 8147)
- Researchers analyzed rates of hospitalization in patients the PFF Patient Registry and discovered that rates were similar between ILD subtypes. The need for inpatient hospitalization was associated with an increased risk of death or transplant in the overall cohort. Risk of death or transplant was highest following hospitalization in idiopathic pulmonary fibrosis (IPF) and lowest in non-IPF idiopathic interstitial pneumonia.
- Authors: C.S. King, R. V. Ignacio, E. Freiheit, S. Arya, V. Khangoora, A. L. Singhal, A. Nyquist, A. Brown, O. A. Shlobin, K. R. Flaherty, S. D. Nathan
Visual and Quantitative CT Derived Parameters Predict Transplant-Free Survival in Patients with Interstitial Lung Disease: Results from the Pulmonary Fibrosis Foundation Registry (Poster 8487)
- Using published diagnostic criteria for usual interstitial pneumonia (UIP) in a population of individuals with clinically diagnosed ILD in the PFF Registry, researchers explored the relationship between CT pattern, fibrosis extent, and transplant-free survival. A definite UIP pattern and extent of fibrosis scored by data-driven textural analysis (DTA) are predictors of transplant-free survival in a heterogeneous group of patients diagnosed with ILD.
- Authors: A.S. Oh, D. A. Lynch, K. R. Flaherty, S. M. Humphries, Pulmonary Fibrosis Foundation
Comparison of Interstitial Lung Disease Diagnoses in Urban and Rural Areas Among Patients in the Pulmonary Fibrosis Foundation Patient Registry (Poster 11428)
- Using the PFF Patient Registry, the study team found that hypersensitivity pneumonitis and exposure-related ILDs were more prevalent in ILD patients who live in rural areas and were associated with certain types of fuel use. The investigators concluded that coal and wood fuel use may explain the different types of ILDs found in patients who live in rural areas.
- Authors: A.E. Dimmock, E. White, Y. Furuya, E. Freiheit, H. Kim
Discordance in Actual Versus Perceived Access to Palliative Care Services for Pulmonary Fibrosis Foundation Care Centers (Poster 5851)
- Providers at PFF Care Center Network sites view palliative care as an important resource for their patients with pulmonary fibrosis. However, significant discordance exists between provider-reported and actual access to local outpatient and inpatient palliative care services, which may be an important barrier to palliative care referral for patients with pulmonary fibrosis. Researchers used the PFF Care Center Network, a group of 68 medical centers nationwide that specialize in the multidisciplinary care of individuals living with PF.
- Authors: R.A. Gersten, B. Seth, K. O. Lindell, S. K. Danoff