Idiopathic pulmonary fibrosis: current and future directions

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and is increasingly recognised. Prior to the advent of effective therapies, achieving an early diagnosis was arguably of little prognostic consequence given IPF was considered an untreatable and uniformly fatal disease. The advent of new drug treatments has given hope for the future and raised the profile of IPF. International management guidelines highlight the critical role of radiology as part of an interstitial lung disease multidisciplinary team approach in reaching an accurate and early diagnosis of IPF. The diagnostic criteria and levels of diagnostic confidence for the radio-pathological pattern associated with the clinical syndrome of IPF, usual interstitial pneumonia (UIP), appear seemingly straightforward; however, with increasing research and recognition of radiopathological interobserver variability, limitations of this classification model are becoming increasingly apparent. This review describes ancillary radiological features, comorbidities, and emerging new entities that potentially co-exist with IPF. Beyond diagnosis radiology is developing as a key prognostic tool to inform longitudinal patient evaluation. These diagnostic and prognostic clinical challenges and the future role of radiology in IPF are discussed.