Elucidating the causes and examining the latest clinical findings in pulmonary fibrosis: recent progress and future objectives

Abstract

In the last decade, we have witnessed many important advances in the current understanding of the clinical disease process that is now defined as idiopathic pulmonary fibrosis (IPF) and how it differs from the other “members” of the idiopathic interstitial pneumonias (IIPs). We have gained insight into the genetic associations with IPF and new insights into pathogenesis. Importantly, we have begun to distinguish IPF from the other IIPs with much more precision and confidence. Recent investigations have been published that identify clues to potential future therapeutic strategies. Each of these developments has been reflected within the articles in the present issue of European Respiratory Review , providing a comprehensive update across the field of IPF but, most importantly, highlighting areas of more rapid evolution and future potential. The articles have summarised what are perceived to be the areas of growth in this group of fibrosing lung diseases, and, more specifically, in IPF. The histopathological patterns of fibrosis observed in each of the IIPs are different, and this is also reflected by the clinical behaviour of the individual diseases. Idiopathic usual interstitial pneumonia (UIP) generally progresses in a more aggressive fashion than the other IIPs and each has some distinctive clinical features that distinguish them. Despite these differences, obtaining a consistent and uniform diagnosis of each of the IIPs can be difficult, and further investigation is needed to reduce the inconsistencies in diagnosis between expert and community clinicians. Issues of overlapping patterns of disease and combinations of patterns will be challenges for the future, particularly with regard to concepts of pathogenesis, outcome and treatment. Despite these confounders, the imaging of patients using high-resolution computed tomography (HRCT) has, in general, enabled a growing number of physicians to make a confident clinical diagnosis without recourse to surgical lung biopsy (SLB). In this regard, …