OUTCOMES OF PATIENTS WITH COMBINED IDIOPATHIC PULMONARY FIBROSIS AND EMPHYSEMA IN THE IPF-PRO REGISTRY

Abstract

SESSION TITLE: Late-breaking Abstract Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: Emphysema is a common comorbidity in patients with IPF. The presence of emphysema in patients with idiopathic pulmonary fibrosis (IPF) has been associated with higher mortality in some studies. We used data from the IPF-PRO Registry, a multicenter US registry of patients with IPF, to examine the characteristics of patients with and without emphysema at the time of enrollment and associations between the presence of emphysema and clinical outcomes. METHODS: Enrollment spanned June 2014 to October 2018. Patients without an HRCT scan, FEV1 value and FVC value at enrollment were excluded from these analyses. We defined emphysema on the basis of investigators’ evaluation of HRCT scans, by prospectively recording the presence of “clinically significant emphysema” on an HRCT scan. Associations between emphysema at enrollment and times to death or lung transplant; hospitalization; and death, lung transplant or hospitalization over follow-up were analyzed using Cox regression models. RESULTS: Of 934 patients in the analysis cohort, 119 (12.7%) had emphysema. Compared with patients without emphysema, patients with emphysema were older (median 72 vs 70 years) and higher proportions were current/former smokers (88.2% vs 63.7%), used oxygen with activity (49.6% vs 31.9%) or at rest (30.8% vs 18.4%), had congestive heart failure (13.6% vs 4.8%) and had a prior respiratory hospitalization (25.0% vs 16.7%). Patients with emphysema had a higher median FVC (71.8 vs 69.4 % predicted) and lower median DLco (35.3 vs 43.6 % predicted) than patients without emphysema. Kaplan–Meier rates of death or lung transplant at 1 year were 17.5% (95% CI: 11.7, 25.8) and 11.2% (95% CI: 9.2, 13.6) in patients with and without emphysema, respectively. Rates of hospitalization at 1 year were 21.6% (95% CI: 14.6, 29.6) and 20.6% (95% CI: 17.9, 23.5) in patients with and without emphysema, respectively. Rates of death, lung transplant or hospitalization at 1 year were 30.4% (95% CI: 22.9, 39.7) and 26.3% (95% CI: 23.4, 29.5) in patients with and without emphysema, respectively. The presence of emphysema at enrollment was not significantly associated with death or lung transplant (HR 1.07 [95% CI: 0.74, 1.54]); hospitalization (HR 1.18 [95% CI: 0.87, 1.60]); or death, lung transplant, or hospitalization (HR 1.02 [95% CI: 0.75, 1.37]), after adjusting for clinical risk factors at enrollment. CONCLUSIONS: Approximately 13% of patients in the IPF-PRO Registry had clinically significant emphysema on HRCT at enrollment. There was no significant difference in the risk of death, lung transplant, or hospitalization between patients who did and did not have emphysema. CLINICAL IMPLICATIONS: In this cohort, the physiologic characteristics and comorbidities of patients with IPF and emphysema differed from those of patients with IPF alone, but the presence of emphysema did not independently drive outcomes. DISCLOSURES: Employee relationship with Boehringer Ingelheim Please note: >$100000 Added 03/23/2020 by Shaun Bender, source=Web Response, value=Salary Advisory Committee Member relationship with Boehringer Ingelheim Please note: $5001 - $20000 Added 03/16/2020 by Daniel Culver, source=Web Response, value=Honararia and Research support Speaker/Speaker's Bureau relationship with Genentech Please note: $1001 - $5000 Added 03/16/2020 by Daniel Culver, source=Web Response, value=Honoraria Consultant relationship with aTyr Please note: $1001 - $5000 Added 03/18/2020 by Daniel Culver, source=Web Response, value=Honoraria and Travel Consultant relationship with Forest Pharmaceuticals Please note: $1-$1000 Added 03/16/2020 by Daniel Culver, source=Web Response, value=No fees received yet Removed 03/20/2020 by Daniel Culver, source=Web Response Consultant relationship with Mallinckrodt Pharmaceuticals Please note: $1001 - $5000 Added 03/20/2020 by Daniel Culver, source=Web Response, value=Consulting fee No relevant relationships by Anne Hellkamp, source=Web Response Speaker/Speaker's Bureau relationship with Boehringer Please note: $1-$1000 Added 07/14/2020 by David Hotchkin, source=Web Response, value=Honoraria No relevant relationships by Hyun Kim, source=Web Response Employee relationship with Boehringer-Ingelheim Inc Please note: >$100000 Added 03/30/2020 by Thomas Leonard, source=Web Response, value=Salary Speaker/Speaker's Bureau relationship with Boehringer Ingelheim Please note: $20001 - $100000 Added 04/02/2020 by Lake Morrison, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Roche Genentech Please note: $20001 - $100000 Added 07/14/2020 by Lake Morrison, source=Web Response, value=Honoraria No relevant relationships by Megan Neely, source=Web Response No relevant relationships by Laurie Snyder, source=Web Response