Baseline Characteristics Of Patients In The Idiopathic Pulmonary Fibrosis Prospective Outcomes (Ipf-Pro) Registry

SESSION TITLE: Updates in Diffuse and Orphan Lung Diseases SESSION TYPE: Original Investigations PRESENTED ON: October 18-21, 2020 PURPOSE: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease associated with decline in lung function, impaired quality of life and high mortality. The IPF-PRO Registry is an observational US registry of patients with IPF. Patients with IPF that was diagnosed or confirmed at the enrolling center in the past 6 months were enrolled into the registry between June 2014 and October 2018. Patients listed for lung transplant were excluded. We examined the demographic and clinical characteristics of the cohort at enrollment. METHODS: Retrospective data collected from patients’ medical records included medical history, pulmonary function tests and medication use. Patient-reported outcome questionnaires were administered at enrollment. RESULTS: A total of 1002 patients were recruited at 47 sites across the US. Median (Q1, Q3) age was 70 (65, 75) years. Most patients were male (74.7%), white (94.9%), and current or former smokers (66.9%). The majority of patients (65.4%) had a diagnosis of definite IPF according to the ATS/ERS/JRS/ALAT 2011 criteria. Median (Q1, Q3) forced vital capacity was 69.8 (59.2, 80.2) % predicted and diffusing capacity was 41.9 (32.2, 50.6) % predicted. Median (Q1, Q3) total score on the St George’s Respiratory Questionnaire was 39.6 (25.5, 53.7). The most frequently reported comorbidities were gastroesophageal reflux disease (56.2%), coronary artery disease (29.8%), obstructive sleep apnea (27.8%) and diabetes (20.5%). Based on the opinion of the investigator, 12.6% of patients had clinically significant emphysema on HRCT. Approximately half (53.4%) of patients were receiving an approved antifibrotic therapy at enrollment. Oxygen with activity was used by 35.2% of patients and oxygen at rest by 20.0% of patients. The majority of patients (62.5%) had private insurance. CONCLUSIONS: The cohort of patients with IPF enrolled into the IPF-PRO Registry is mostly male, white, and elderly, with impaired lung function and health-related quality of life and a high prevalence of comorbidities. CLINICAL IMPLICATIONS: Longitudinal follow-up data from the IPF-PRO Registry will provide insights into the clinical course, impact and treatment of IPF, mechanisms of disease, and factors associated with better outcomes. DISCLOSURES: Employee relationship with Boehringer Ingelheim Please note: >$100000 Added 03/23/2020 by Shaun Bender, source=Web Response, value=Salary Employee relationship with Boehringer Ingelheim Pharmaceuticals Please note: >$100000 Added 03/25/2020 by Craig Conoscenti, 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 Daniel Dilling, source=Web Response No relevant relationships by Megan Neely, source=Web Response Speaker/Speaker's Bureau relationship with Genentech Please note: $20001 - $100000 Added 03/20/2020 by Justin Oldham, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Boehringer Ingelheim Please note: $20001 - $100000 Added 03/20/2020 by Justin Oldham, source=Web Response, value=Honoraria PI for IPF PRO/ ILD PRO study at DCRI relationship with Boehringer Ingelheim Please note: >$100000 Added 03/24/2020 by Scott Palmer, source=Web Response, value=Grant/Research Support Statistical support for IPF-PRO Registry relationship with Boehringer Ingelheim Please note: $5001 - $20000 Added 03/24/2020 by Eric Yow, source=Web Response, value=Grant/Research Support Removed 03/24/2020 by Eric Yow, source=Web Response Statistical support for IPF-PRO Registry relationship with Boehringer Ingelheim Please note: $5001 - $20000 Added 03/24/2020 by Eric Yow, source=Web Response, value=Salary