Corticosteroids for Acute Exacerbations of Idiopathic Pulmonary Fibrosis: Patterns and Outcomes

Boston University Medical Campus, Boston MA

Investigators

Linked publications, trials & patents

Abstract

PROJECT SUMMARY In the proposal entitled, “Corticosteroids in Acute Exacerbations of Idiopathic Pulmonary Fibrosis: Practices and Outcomes” I will determine current practice patterns and outcomes associated with corticosteroid therapy for acute exacerbations of idiopathic pulmonary fibrosis in critically ill patients. Every year, 10-20% of patients with idiopathic pulmonary fibrosis (IPF) – a progressive, fibrosing lung disease – experience acute exacerbations (AE-IPF), often leading to hospital admission and critical illness. These acute respiratory deteriorations result in high morbidity and mortality for IPF patients; patients who develop critical illness have in-hospital mortality rates of 20-35% and a median survival post-exacerbation of 3-4 months. Corticosteroids are a mainstay of therapy for patients with AE-IPF based on weak evidence, and guidelines do not make recommendations as to dosing, route, timing, and duration of corticosteroids. Given lack of clear guidelines and evidence, use patterns are likely highly variable. Expert consensus has called for new evidence to guide AE-IPF management. In this proposal, I will fill these large knowledge gaps by 1) characterizing practice patterns of corticosteroid dosing, route, initiation timing, and duration for AE-IPF in a large, national cohort and 2) determining outcomes associated with dosing strategies for corticosteroids using comparative effectiveness methodology. Completion of this proposal will help inform future studies for AE-IPF management and build an evidence base for best practices. In addition to the direct experiential learning provided by completion of this proposal, I will partake in didactics, scientific seminars, and research conferences throughout this award period, in order to advance my competency in clinical research design and methodology. Further, I will obtain the skills necessary for data collection and statistical analysis using large databases. With the direct mentorship of my sponsors, the supportive environment of The Pulmonary Center at Boston University Medical Center, and professional development courses, I will successfully incorporate the results of this proposal into a Career Development Award where I will focus on implementing best practices for corticosteroid use in AE-IPF. This proposal is directly aligned with the goals of the National Heart, Lung, and Blood Institute objectives which include optimizing clinical research to improve health and reduce disease and leveraging new opportunities in data science to open new frontiers in heart, lung, blood, and sleep research. This research grant will be performed under the direct mentorship of two health services investigators with expertise in critical care epidemiology and outcomes research and under the advice of a comparative effectiveness methodological expert and two experts in idiopathic pulmonary fibrosis.