Molecular classification of idiopathic pulmonary fibrosis: personalized medicine, genetics and biomarkers

Respirology. 2015 Oct;20(7):1010-22. doi: 10.1111/resp.12569. Epub 2015 Jun 24.


Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic lung disease associated with high morbidity and poor survival. Characterized by substantial disease heterogeneity, the diagnostic considerations, clinical course and treatment response in individual patients can be variable. In the past decade, with the advent of high-throughput proteomic and genomic technologies, our understanding of the pathogenesis of IPF has greatly improved and has led to the recognition of novel treatment targets and numerous putative biomarkers. Molecular biomarkers with mechanistic plausibility are highly desired in IPF, where they have the potential to accelerate drug development, facilitate early detection in susceptible individuals, improve prognostic accuracy and inform treatment recommendations. Although the search for candidate biomarkers remains in its infancy, attractive targets such as MUC5B and MPP7 have already been validated in large cohorts and have demonstrated their potential to improve clinical predictors beyond that of routine clinical practices. The discovery and implementation of future biomarkers will face many challenges, but with strong collaborative efforts among scientists, clinicians and the industry the ultimate goal of personalized medicine may be realized.

Keywords: biomarker; genetics; idiopathic pulmonary fibrosis.

Publication types

  • Review

MeSH terms

  • Biomarkers / metabolism*
  • Chemokine CXCL13 / genetics
  • Early Diagnosis
  • Forecasting
  • Genomics
  • Humans
  • Idiopathic Pulmonary Fibrosis* / diagnosis
  • Idiopathic Pulmonary Fibrosis* / genetics
  • Idiopathic Pulmonary Fibrosis* / metabolism
  • Idiopathic Pulmonary Fibrosis* / physiopathology
  • Idiopathic Pulmonary Fibrosis* / therapy
  • Matrix Metalloproteinase 3 / genetics
  • Precision Medicine / trends*
  • Prognosis
  • Proteomics


  • Biomarkers
  • CXCL13 protein, human
  • Chemokine CXCL13
  • MMP3 protein, human
  • Matrix Metalloproteinase 3