Abstract

SESSION TITLE: Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis SESSION TYPE: Original Investigations PRESENTED ON: 10/08/2018 07:30 am - 08:30 am PURPOSE: Idiopathic pulmonary fibrosis (IPF) is one of the leading indications for lung transplant. The timing of lung transplant in IPF varies due to the general nature of the guidelines from the ISHLT and center-specific implementation practices. More data are needed to describe the time from diagnosis of IPF to transplant in clinical practice and the characteristics of patients who undergo transplant. METHODS: Patients with IPF enrolled in the ongoing Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry in the US from its inception on 5 June 2014 to 7 June 2017 comprised the analysis cohort. Individuals enrolled in the registry had newly diagnosed IPF or were newly confirmed with IPF by the enrolling center, and were not listed for lung transplant at the time of enrollment. Time from confirmed diagnosis of IPF at the enrolling center to lung transplant, and clinical characteristics at the time of confirmed diagnosis of IPF, were evaluated descriptively in patients who subsequently received a transplant, who died and who did not receive a transplant and had not died. RESULTS: Of 563 patients in this analysis cohort, 15 (2.7%) had received a lung transplant and 482 (85.6%) did not receive a lung transplant and were alive. Median (Q1, Q3) time from confirmed diagnosis of IPF to lung transplant was 11.0 (5.7, 15.0) months, with Kaplan-Meier estimated transplant rates (95% CI) of 2.5% (1.3%, 4.7%) at 1 year and 6.0% (3.3%, 10.8%) at 2 years post-diagnosis. At the time of confirmed diagnosis of IPF, patients who went on to receive a lung transplant had lower median FVC % predicted (53.8% vs 70.6%), lower median DLco % predicted (36.0% vs 42.6%) and lower median 6-minute walk test distance (350 m vs 383 m) than patients who did not receive a lung transplant and were alive. A greater proportion of patients who went on to receive a lung transplant were using supplemental oxygen with activity than patients who did not receive a lung transplant and were alive (66.7% vs 30.1%). CONCLUSIONS: Data from the IPF-PRO Registry suggest that in patients not listed for lung transplant at enrollment, a low proportion of patients had a transplant within 2 years. Those that underwent transplant had more severe disease. CLINICAL IMPLICATIONS: Further analyses of data from the ongoing IPF-PRO Registry will provide valuable insights into clinical practices around lung transplant for IPF in the US. DISCLOSURES: Employee relationship with Boehringer Ingelheim Please note: >$100000 Added 03/01/2018 by Shaun Bender, source=Web Response, value=Salary Employee relationship with Boehringer Ingelheim Pharmaceuticals Please note: >$100000 Added 03/01/2018 by Craig Conoscenti, source=Web Response, value=Salary Consultant relationship with Boehringer Ingelheim Please note: $20001 - $100000 Added 03/15/2018 by Kevin Flaherty, source=Web Response, value=Consulting fee Consultant relationship with Roche/Genentech Please note: $1001 - $5000 Added 03/15/2018 by Kevin Flaherty, source=Web Response, value=Consulting fee Consultant relationship with Sanofi Genzyme Please note: $1001 - $5000 Added 03/15/2018 by Kevin Flaherty, source=Web Response, value=Consulting fee Consultant relationship with Veracyte Please note: $5001 - $20000 Added 03/15/2018 by Kevin Flaherty, source=Web Response, value=Consulting fee Employee relationship with Boehringer-Ingelheim Pharmaceuticals Inc Please note: >$100000 Added 03/01/2018 by Thomas Leonard, source=Web Response, value=Salary No relevant relationships by Megan Neely, source=Web Response PI relationship with BI Please note: $1001 - $5000 Added 03/15/2018 by Scott Palmer, source=Web Response, value=Consulting fee PI relationship with BI Please note: >$100000 Added 03/15/2018 by Scott Palmer, source=Web Response, value=Grant/Research Support No relevant relationships Added 02/28/2018 by Laurie Snyder, source=Web Response, value=Consulting feeRemoved 02/28/2018 by Laurie Snyder, source=Web Response No relevant relationships Added 02/28/2018 by Laurie Snyder, source=Web Response, value=Consulting feeRemoved 02/28/2018 by Laurie Snyder, source=Web Response No relevant relationships by Eric Yow, source=Web Response

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