Recent advances in idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) remains the most common of the idiopathic interstitial pneumonias and portends a poor prognosis. Significant strides have been made in the approach to diagnosis and in the ability to predict outcome in the last few years. Advances in high-resolution CT (HRCT) scanning have allowed an accurate diagnosis obviating the need for surgical biopsy in many patients. Furthermore, HRCT scanning may aid in determining prognosis and identifying disease progression. The appropriate use of the HRCT scan requires a multidisciplinary iterative approach incorporating all available data to reach a final diagnosis. However, there remains great heterogeneity in disease progression. Pulmonary hypertension and acute exacerbations of IPF negatively influence prognosis and are increasingly a target of therapy. There has been an increase in the number of well-designed clinical trials of IPF that have focused on more specific targets. While no cure has yet been found, each trial expands our understanding regarding the natural course of the disease and the impact of targeted therapy. In the interim, lung transplantation, which appears to improve survival in a subset of IPF patients, remains the only intervention. The objective of this article is to review advances in the understanding of IPF and the evidence for the findings outlined above.