Department of Radiology, Duke University Medical Center, Durham, NC.
Department of Infection, Immunity & Cardiovascular Disease, Academic Radiology, University of Sheffield, Western Bank, UK.
J Thorac Imaging. 2019 Mar;34(2):136-150. doi: 10.1097/RTI.0000000000000392.
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) Xe 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 Xe 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 Xe 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 Xe MRI to be incorporated into the IPF clinical algorithm and begin to positively impact IPF disease diagnosis and management.
特发性肺纤维化（IPF）是一种进行性肺部疾病，最终导致死亡。尽管高分辨率计算机断层扫描（HRCT）的出现彻底改变了 IPF 的诊断方式，但这种成像方式仍然存在明显的局限性，尤其是在评估疾病进展和治疗反应方面。鉴于最近推出的新型疗法以及众多正在研发中的疗法，对非侵入性区域性评估的需求变得更加迫切。因此，用三维区域性功能评估来补充肺结构的三维成像可能会很有价值。这一挑战很好地被超极化（HP）氙磁共振成像（MRI）解决了，该技术利用这种惰性气体的独特特性来对其分布进行成像，不仅可以在肺泡中，还可以在间质屏障组织和红细胞中成像。这种单次呼吸成像检查最终可能成为评估 IPF 中肺换气功能障碍的理想非侵入性工具。本文综述了 HP Xe MRI 从早期发展到目前作为临床研究平台的历程。本文详细介绍了可以从 Xe MRI 检查中生成的关键成像生物标志物，以及它们在 IPF 中的诊断、预后和治疗反应评估中的潜在用途。最后，我们讨论了为将 HP Xe MRI 纳入 IPF 临床算法并开始对 IPF 疾病诊断和管理产生积极影响，必须进行的研究类型。
