Idiopathic pulmonary fibrosis (IPF) is a progressive pulmonary disease that is ultimately fatal. Although the diagnosis of IPF has been revolutionized by high-resolution computed tomography, this imaging modality still exhibits significant limitations, particularly in assessing disease progression and therapy response. The need for noninvasive regional assessment has become more acute in light of recently introduced novel therapies and numerous others in the pipeline. Thus, it will likely be valuable to complement 3-dimensional imaging of lung structure with 3-dimensional regional assessment of function. This challenge is well addressed by hyperpolarized (HP) 129Xe magnetic resonance imaging (MRI), exploiting the unique properties of this inert gas to image its distribution, not only in the airspaces, but also in the interstitial barrier tissues and red blood cells. This single-breath imaging exam could ultimately become the ideal, noninvasive tool to assess pulmonary gas-exchange impairment in IPF. This review article will detail the evolution of HP 129Xe MRI from its early development to its current state as a clinical research platform. It will detail the key imaging biomarkers that can be generated from the 129Xe MRI examination, as well as their potential in IPF for diagnosis, prognosis, and assessment of therapeutic response. We conclude by discussing the types of studies that must be performed for HP 129Xe MRI to be incorporated into the IPF clinical algorithm and begin to positively impact IPF disease diagnosis and management.
*Department of Radiology, Duke University Medical Center, Durham, NC
†Department of Infection, Immunity & Cardiovascular Disease, Academic Radiology, University of Sheffield, Western Bank, UK
Supported by NIH/NHLBI R01 HL105643 and NIH/NHLBI R01HL126771.
The authors declare no conflicts of interest.
Correspondence to: Joseph G. Mammarappallil, MD, PhD, Department of Radiology, Duke University Medical Center, 2301 Erwin Road, P.O. Box 3808, Durham, NC 27710 (e-mail: firstname.lastname@example.org).