Current State of Fibrotic Interstitial Lung Disease Imaging

Abstract

Interstitial lung disease (ILD) diagnosis is complex, continuously evolving, and increasingly reliant on thin-section chest CT. Multidisciplinary discussion aided by a thorough radiologic review can achieve a high-confidence diagnosis of ILD in the majority of patients and is currently the reference standard for ILD diagnosis. CT also allows the early recognition of interstitial lung abnormalities, possibly reflective of unsuspected ILD and progressive in a substantial proportion of patients. Beyond diagnosis, CT has also become essential for ILD prognostication and follow-up, aiding the identification of fibrotic and progressive forms. The presence of fibrosis is a critical determinant of prognosis, particularly when typical features of usual interstitial pneumonia (UIP) are identified. The UIP-centric imaging approach emphasized in this review is justified by the prognostic significance of UIP, the prevalence of UIP in idiopathic pulmonary fibrosis, and its strong radiologic-pathologic correlation. In nonidiopathic pulmonary fibrosis ILD, progressive pulmonary fibrosis carries clinically significant prognostic and therapeutic implications. With growing evidence and the emergence of novel ILD-related concepts, recent updates of several imaging guidelines aim to optimize the approach to ILD. Artificial intelligence tools are promising adjuncts to the qualitative CT assessment and will likely augment the role of CT in the ILD realm.