PFF Registry News


Pulmonary Fibrosis Patient Registry Data Reveal PF, ILD Outcome Variations

Recent studies based on data from the Pulmonary Fibrosis Foundation Patient Registry,1 presented at the American Thoracic Society (ATS) 2022 Conference, “provide important information on potential disparities and differences in the diagnosis, prognosis, and management of patients with pulmonary fibrosis or interstitial lung disease,” said Joyce S. Lee, MD, MS, lead author of 1 of those studies and the senior medical advisor for research and health care quality at the Pulmonary Fibrosis Foundation (PFF).

Established in 2016, the PFF registry includes over 2000 participants in various care setting and regions across the US.1 Such registries “lay the foundation for developing evidence-based care for patients living with PF,” explained Dr Lee, who is also the associate professor of medicine in the division of pulmonary sciences and critical care and director of the Interstitial Lung Disease (ILD) Program at the University of Colorado Anschutz Medical Campus in Aurora.

The research presented at ATS, which is summarized here, explored outcome differences between sites of care, variations in disease in urban and rural areas, predictors of transplant free survival, access to palliative care, and more. The studies analyzed data from the PFF Patient Registry as well as provider surveys from the PFF Care Center Network (CCN), a database that includes a vetted list of more than 65 medical centers across the US with expertise in treating patients with fibrotic lung disease via a multidisciplinary approach.1

Varying Site-Level Outcomes Suggest Potential Variations in Practice

Dr Lee and colleagues examined site-level variation in clinical outcomes among 1474 patients with idiopathic pulmonary fibrosis (IPF; 60.7%), collagen vascular disease-related ILD (16.8%), non-IPF idiopathic interstitial pneumonia (IIP; 10.7%) and hypersensitivity pneumonitis (8.5%) across 38 PFF CCN sites.2 The results demonstrated site-level variation for time to death (P =.0227), time to first death or transplant (P =.0002), time to first hospitalization (P <.0001), and time to first death, transplant, or hospitalization (P =.0029) but no variation for 1-year change in percent predicted forced vital capacity (FVC; P =.29) or DLCO (P =.12).2

“Additional studies are needed to determine if there are practice pattern differences and/or resources that can account for site-level variation,” the study authors concluded.2

The Effect of Environment: ILD Diagnoses in Urban vs Rural Areas

In a retrospective study of 1840 patients from 42 PFF Registry sites, Dimmock and colleagues investigated differences in ILD diagnosis in urban vs rural areas.3 Analyses revealed similar rates of IPF, non-IPF IIP, and connective tissue-associated ILD diagnoses between patients in urban and rural residents, while those in rural areas showed higher rates of fibrotic hypersensitivity pneumonitis and exposure-related ILD. Additionally, patients residing in counties with coal fuel use and county wood fuel use were more likely to have exposure-related ILD (P <.0001) and fibrotic hypersensitivity pneumonitis (P =.0001), respectively.3

Predictors of Transplant-Free Survival

Noting the importance of classifying computed tomography (CT) pattern and quantifying disease extent in patients with fibrosing ILD, Oh and colleagues explored associations between CT pattern, fibrosis extent, and transplant-free survival based on 1133 baseline CT scans from ILD patients in the PFF Registry.4

Multivariate analysis showed that a definite UIP pattern (hazard ratio [HR], 1.63; 95% CI, 1.19-2.23; P =.002) and data-driven textural analysis (DTA) score (HR, 1.05; 95% CI, 1.04-1.05; P <.0001) were significant predictors of transplant-free survival after adjusting for age, sex, body mass index, and smoking history.4

Reasons for Hospitalization

King and colleagues investigated variations in hospitalization rates and related outcomes based on ILD type among 1989 patients in the PFF Registry.5 The results demonstrated similar hospitalization rates across ILD types, ranging from 40.6% to 53.3%. The need for inpatient hospitalization was linked to an increased risk of death or transplant (HR, 1.57; 95% CI, 1.34-1.84), with 1 of these outcomes occurring in 40.9% of hospitalized patients.5

Compared to patients with IPF, an unadjusted analysis showed the following risk of death or transplant following hospitalization among patients with other ILDs: collagen vascular/autoimmune (HR 0.55; 95% CI, 0.40-0.77), hypersensitivity pneumonitis (HR, 0.71; 95% CI, 0.48-1.06), non-IPF IIP (HR, 0.45; 95% CI, 0.30- 0.69), and “other” forms of ILD (HR, 0.56; 95% CI, 0.29-1.08).5

Patients Report Lack of Access to Palliative Care

In addition to the registry-based studies, Gersten and colleagues examined national data on palliative care availability from the Center to Advance Palliative Care (CAPC), comparing it provider reports on the local accessibility of palliative care for ILD patients. Provider data came from a survey of 94 physicians, advanced practice providers, and nurses at 68 PFF Care Centers.6

Although 96% of respondents’ institutions offer inpatient palliative care according to CAPC data, 15% of respondents indicated a lack of such access. For outpatient palliative care, national data showed availability at 97% of institutions, while 38% of respondents reported no such access. Among centers with more than 1 participant in the survey, dissonant responses between providers were noted in 52% of cases.6

These findings “suggest that providers may be unaware of local access to palliative care, which may be an important barrier to palliative care referral in the ILD population,” the authors wrote. Future research should explore the utility of efforts to increase provider awareness of palliative care availability with the aim of reducing this barrier.

Takeaways and Next Steps

Although patient registries, in themselves, cannot solve patient care problems, the information gleaned from registry data can open the door to evidence-based solutions for patients with PF, as well as to conversations around improving access to vital resources for our patients,” said Dr. Lee.

“Looking ahead, we are expanding the PFF Registry with the launch of the new PFF Community Registry, which will rely on data provided by patients living with PF or ILD, lung transplant recipients who have had PF or ILD, and their caregivers or family members,” Dr Lee said. These data “will allow us to determine if the findings from these studies are generalizable to the broader patient population.”


  1. Pulmonary Fibrosis Foundation. Accessed August 4, 2022.
  2. Lee, JS, Boente R, White E, Baxter C, Shore JE, Collard HR. Differences in patient outcomes across the PFF Care Center Network. Am J Respir Crit Care Med. 2022; 205:A5573.
  3. Dimmock AE, White E, Furuya Y, Freiheit E, Kim H. Comparison of interstitial lung disease diagnoses in urban and rural areas among patients in the Pulmonary Fibrosis Foundation Patient Registry. Am J Respir Crit Care Med. 2022; 205:A5738.
  4. Oh, AS, Lynch DA, Flaherty KR, Humphries SM. Visual and quantitative CT derived parameters predict transplant-free survival in patients with interstitial lung disease: Results from the Pulmonary Fibrosis Foundation Registry. Am J Respir Crit Care Med. 2022; 205:A3424.
  5. King CS, Ignacio RV, Freiheit E, et al. Hospitalization rates in various interstitial lung diseases: An analysis of the Pulmonary Fibrosis Foundation (PFF) Patient Registry. Am J Respir Crit Care Med. 2022; 205:A3422.
  6. Gersten RA, Seth B, Lindell KO, Danoff SK.Discordance in actual versus perceived access to palliative care services for Pulmonary Fibrosis Foundation Care Centers. Am J Respir Crit Care Med. 2022; 205:A2712.

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