Predicting and treating pulmonary fibrosis with proteomic biomarker investigations.

Idiopathic pulmonary fibrosis (IPF) is a chronic, rare and fatal disease that is the conse-quence of aberrant remodeling and defective repair mechanisms within the lung cul-minating in the loss of alveolar integrity. Although significant progress has been made in understanding the pathogenesis, it would be crucial to identify biomarkers for diagnosis, prognosis and prediction of therapy response to improve management of this challenging and debilitating disease. Omics technologies have profoundly advanced the understanding of disease mechanisms, presenting considerable potential for the identification of clinically relevant biomarkers. To date, specific molecular pathways have been implicated in the onset and progression of idiopathic pulmonary fibrosis, including abnormal wounding, fibroblast proliferation, inflammation, deposition of extracellular matrix, oxidative stress, endoplasmic reticulum stress and coagulation system. This review highlights the role of proteomics in identifying key biomarkers for IPF, focusing on their clinical relevance, including diagnosis, prognosis, disease progression and the identification of new therapeutic options, in light of the most recent technological advancements in mass spectrometry.