Progress in the management of IPF-related acute exacerbations: a goal for patients, respirologists and intensivists
- Ganesh Raghu⇑
- Ganesh Raghu, Center for Interstitial Lung Diseases, University of Washington, Seattle, WA 98195, USA. E-mail: graghu{at}uw.edu
Abstract
Until clinical trial results are available, treatment decisions for acute exacerbations of IPF must be made on an individual basis http://ow.ly/FFxi30hY7uz
From the author:
I appreciate the expressed views of F. Vincent and colleagues, regarding the need to address treatment consideration of acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF). While the mortality of patients manifesting AE of IPF is unfortunately high, F. Vincent and colleagues argue that supportive care in the intensive care unit (ICU) is an appropriate consideration, including noninvasive and/or mechanical ventilation for patients. Indeed, well-informed patients may opt for such aggressive supportive measures in the ICU, and for some patients listed for lung transplantation, such aggressive supportive measures may serve as a “bridge” for a suitable lung to become available during a set time. Such decisions will need to be made on a case by case basis and tailored to patients’ preferred choices, advance directives that patients may have in place and other individual needs. In addition, other confounding factors, such as comorbid conditions if present, which may have a poor outcome otherwise, should be taken into consideration when providing aggressive supportive care in the ICU for patients manifesting AE of IPF.
The need for clinical trials in this subgroup of patients manifesting AE of IPF is evident. Although Azuma et al. [1] reported ∼15% of patients in the placebo arm to have manifested AE of IPF in a phase II clinical trial, all other clinical trials to date have reported much lower percentages of patients manifesting AE of IPF [2]. The difficulty in obtaining all the necessary clinical data with a high-resolution computed tomography chest scan to meet the criteria for AE of IPF, leading to missing data from centres unable to perform all the required evaluation to ascertain the diagnosis of AE of IPF, results in additional difficulties in designing the much needed well-designed clinical trials [3]. The need for adjudication of the events as true AE of IPF is imperative. Given the relatively small percentage of patients manifesting AE of IPF in clinical trials to date [2], the feasibility of enrolling the required sample size to determine the efficacy of a treatment regimen will be challenging and will require large numbers of patients recruited from several specialised centres worldwide.
Acknowledging these factors, indeed, well-designed clinical trials to determine a safe and efficacious pharmacotherapeutic intervention for the AE of IPF are much needed. In this regard, it is with great enthusiasm that we look forward to successful outcomes for patients with IPF manifesting AE of IPF in the recently approved placebo-controlled randomised phase III trial evaluating the efficacy of cyclophosphamide in conjunction with corticosteroids in IPF-related AEs in France (EXAFIP; ClinicalTrials.gov identifier NCT02460588). The results of this clinical trial are eagerly waited.
Footnotes
Conflict of interest: G. Raghu reports personal fees from Boehringer-Ingelheim, Sanofi and Veracyte; and other fees as a consultant for IPF studies from Boehringer-Ingelheim, BMS, Fibrogen, Gilead, Promedior, Roche-Genentech, Sanofi, United Therapeutics, UCB and Veracyte; all outside the submitted work.
- Received January 18, 2018.
- Accepted January 18, 2018.
- Copyright ©ERS 2018