Associations between resources and practices of ILD centers and outcomes in patients with idiopathic pulmonary fibrosis: data from the IPF-PRO Registry
Authors: de Andrade Joao A. , Kulkarni Tejaswini , Neely Megan L. , Hellkamp Anne S. , Case Amy Hajari , Culver Daniel A. , Guntupalli Kalpalatha , Bender Shaun , Conoscenti Craig S. , Snyder Laurie D. ,   the IPF-PRO Registry Investigators, Baker Albert , Beegle Scott , Belperio John A. , Condos Rany , Cordova Francis , Culver Daniel A. , Dilling Daniel , Fitzgerald John , Silhan Leann , Flaherty Kevin R. , Gibson Kevin , Gulati Mridu , Guntupalli Kalpalatha , Gupta Nishant , Case Amy Hajari , Hotchkin David , Huie Tristan J. , Kaner Robert J. , Kim Hyun J. , Lancaster Lisa H. , Steele Mark , Lasky Joseph A. , Lee Doug , Liesching Timothy , Lipchik Randolph , Lobo Jason , Luckhardt Tracy R. , de Andrade Joao A. , Mageto Yolanda , Huang Howard , Menon Prema , Mageto Yolanda , Namen Andrew , Oldham Justin M. , Paul Tessy , Zhang David , Podolanczuk Anna , Lederer David , Patel Nina M. , Porteous Mary , Kreider Maryl , Raj Rishi , Mohabir Paul , Ramaswamy Murali , Russell Tonya , Sachs Paul , Safdar Zeenat , Shafazand Shirin , Glassberg Marilyn , Siddiqi Ather , Asi Wael , Sigal Barry , Strek Mary E. , Suliman Sally , Roman Jesse , Tabak Jeremy , Walia Rajat , Whelan Timothy P. M. 
Published: January 2022

Background

Performance benchmarks for the management of idiopathic pulmonary fibrosis (IPF) have not been established. We used data from the IPF-PRO Registry, an observational registry of patients with IPF managed at sites across the US, to examine associations between the characteristics of the enrolling sites and patient outcomes.

Methods

An online survey was used to collect information on the resources, operations, and self-assessment practices of IPF-PRO Registry sites that enrolled ≥ 10 patients. Site variability in 1-year event rates of clinically relevant outcomes, including death, death or lung transplant, and hospitalization, was assessed. Models were adjusted for differences in patient case mix by adjusting for known predictors of each outcome. We assessed whether site-level heterogeneity existed for each patient-level outcome, and if so, we investigated potential drivers of the heterogeneity.

Results

All 27 sites that enrolled ≥ 10 patients returned the questionnaire. Most sites were actively following > 100 patients with IPF (70.4%), had a lung transplant program (66.7%), and had a dedicated ILD nurse leader (77.8%). Substantial heterogeneity was observed in the event rates of clinically relevant outcomes across the sites. After controlling for patient case mix, there were no outcomes for which the site variance component was significantly different from 0, but the p-value for hospitalization was 0.052. Starting/completing an ILD-related quality improvement project in the previous 2 years was associated with a lower risk of hospitalization (HR 0.60 [95% CI 0.44, 0.82]; p = 0.001).

Conclusions

Analyses of data from patients with IPF managed at sites across the US found no site-specific characteristics or practices that were significantly associated with clinically relevant outcomes after adjusting for patient case mix.

Trial registration ClinicalTrials.gov, NCT01915511. Registered 5 August 2013, https://clinicaltrials.gov/ct2/show/NCT01915511