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Diffuse Lung Disease |

Newly Diagnosed IPF at Tertiary Care Pulmonary Centers: Initial Results From the Idiopathic Pulmonary Fibrosis — PROspective Outcomes (IPF-PRO) Registry

Michael Durheim, MD; Emily O'Brien, MD; Victoria Gamerman, MS; Jamie Todd, MD; Laurie Snyder, MD; Kevin Anstrom, PhD; Scott Palmer, MD; Craig Conoscenti, MD
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Duke Clinical Research Institute and Duke University Medical Center, Durham, NC


Chest. 2015;148(4_MeetingAbstracts):362A. doi:10.1378/chest.2268509
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Abstract

SESSION TITLE: Diagnosis and Treatment of IPF

SESSION TYPE: Original Investigation Slide

PRESENTED ON: Monday, October 26, 2015 at 01:30 PM - 02:30 PM

PURPOSE: Idiopathic pulmonary fibrosis (IPF) is often diagnosed after multidisciplinary evaluation at tertiary care pulmonary centers. However, across such centers in the United States, little is known about the characteristics of patients with IPF at the time of diagnosis. We examined the clinical course leading to a diagnosis of IPF, the characteristics of the diagnostic evaluation, and baseline disease characteristics among patients enrolled in the national, multi-center IPF-PRO registry.

METHODS: We analyzed data from the first 49 patients enrolled in the IPF-PRO registry over 10 US centers between June 2014 and March 2015. Study inclusion required a new diagnosis of IPF at the enrolling center, consistent with ATS/ERS/JRS/ALAT diagnostic criteria. Diagnostic evaluations in the 12 months preceding study enrollment, and demographic and physiologic measures at the time of IPF diagnosis were abstracted from medical records. We report descriptive statistics as n (%) for categorical variables and median (IQR) for continuous variables.

RESULTS: Patients were 73% male, with a median age of 72 (67-75) years. Median time from onset of first respiratory symptoms to IPF diagnosis at the subspecialty clinic was 16 months. Forty-eight of 49 patients (98%) had undergone high-resolution chest CT (HRCT) as part of their diagnostic evaluations, while 10 (20%) had undergone surgical biopsy. By the time of enrollment, pulmonary function tests demonstrated considerable impairment, with median FVC of 72% (61-81%) predicted and DLCO of 39% (34-48%) predicted. Fourteen (29%) and 22 (45%) required supplemental oxygen at rest and with activity, at up to 6 and 8 L/min, respectively. The most commonly reported comorbidities were gastroesophageal reflux disease (69%), coronary artery disease (31%), and sleep apnea (29%).

CONCLUSIONS: The majority of new IPF diagnoses made at tertiary care pulmonary centers were based on HRCT and clinical information, with only a minority undergoing surgical biopsy. Most patients had respiratory symptoms for greater than one year prior to diagnosis. At the time of center diagnosis, the IPF-PRO registry population had substantial physiologic impairment, supplemental oxygen needs, and comorbidity burden.

CLINICAL IMPLICATIONS: Longitudinal follow-up among patients enrolled in the IPF-PRO registry will create opportunities to better understand outcomes and disease progression across a broad range of patients with IPF.

DISCLOSURE: Michael Durheim: Grant monies (from industry related sources): The IPF-PRO Registry is funded by Boehringer Ingelheim Pharmaceuticals, Inc. and coordinated by the Duke Clinical Research Institute. Emily O'Brien: Grant monies (from industry related sources): The IPF-PRO Registry is funded by Boehringer Ingelheim Pharmaceuticals, Inc. and coordinated by the Duke Clinical Research Institute. Victoria Gamerman: Employee: Victoria Gamerman is an employee of Boehringer Ingelheim Jamie Todd: Grant monies (from industry related sources): Monies paid to institution by Boehringer Ingelheim to perform specific tasks relevant to this abstract. No monies paid directly to Jamie Todd. Laurie Snyder: Grant monies (from industry related sources): The IPF-PRO Registry is funded by Boehringer Ingelheim Pharmaceuticals, Inc. Kevin Anstrom: Grant monies (from industry related sources): The IPF-PRO Registry is funded by Boehringer Ingelheim Pharmaceuticals, Inc. and coordinated by the Duke Clinical Research Institute. Scott Palmer: Grant monies (from industry related sources): The IPF-PRO Registry is funded by Boehringer Ingelheim Pharmaceuticals, Inc. and coordinated by the Duke Clinical Research Institute. Craig Conoscenti: Employee: Craig Conoscenti is an employee of Boehringer Ingelheim

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