Precision medicine advances in idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a highly heterogeneous, unpredictable and ultimately lethal chronic lung disease. Over the last decade, two anti-fibrotic agents have been shown to slow disease progression, however, both drugs are administered uniformly with minimal consideration of disease severity and inter-individual molecular, genetic, and genomic differences. Advances in biological understanding of disease endotyping and the emergence of precision medicine have shown that "a one-size-fits-all approach" to the management of chronic lung diseases is no longer appropriate. While precision medicine approaches have revolutionized the management of other diseases such as lung cancer and asthma, the implementation of precision medicine in IPF clinical practice remains an unmet need despite several reports demonstrating a large number of diagnostic, prognostic and theragnostic biomarker candidates in IPF. This review article aims to summarize our current knowledge of precision medicine in IPF and highlight barriers to translate these research findings into clinical practice.

Keywords: Idiopathic pulmonary fibrosis; Personalized medicine; Precision medicine; Theragnostic biomarkers.

Fig. 1

Schematic representation of personalized medicine approaches that could be implemented in future clinical practice for patients with IPF. Early screening could lead to timely diagnosis, alter the nature course of the disease (red demarcation) and improve outcomes (blue demarcation). Implementation of the 52-gene signature could considerably improve the prognostic performance of GAP index. A plethora of other biomarkers could have prognostic and/or theragnostic role.

Fig. 2

52-gene signature trends in high-risk patients with IPF shift after anti-fibrotic therapy initiation. Panel A and B show up and down scores derived from SAMS respectively. Scores shift their trends over time in high (continuous red line) vs low (continuous black line) risk groups after antifibrotic initiation. Panel C shows FVC trends of treated patients. A simultaneous reduction in up score and increase in down score is shown with black line, while other score changes are shown with red lines. (Modified from the article of Herazo-Maya et al, Lancet Respiratory Medicine 2017 with permission.)