title: PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery.
abstract: Despite major advances in the understanding of the pathogenesis of idiopathic pulmonary fibrosis (IPF), diagnosis and management of the condition continue to pose significant challenges. Clinical management of IPF remains unsatisfactory due to limited availability of effective drug therapies, a lack of accurate indicators of disease progression, and an absence of simple short-term measures of therapeutic response. The identification of more accurate predictors of prognosis and survival in IPF would facilitate counseling of patients and their families, aid communication among clinicians, and would guide optimal timing of referral for transplantation. Improvements in molecular techniques have led to the identification of new disease pathways and a more targeted approach to the development of novel anti-fibrotic agents. However, despite an increased interest in biomarkers of IPF disease progression there are a lack of measures that can be used in early phase clinical trials. Careful longitudinal phenotyping of individuals with IPF together with the application of novel omics-based technology should provide important insights into disease pathogenesis and should address some of the major issues holding back drug development in IPF. The PROFILE (Prospective Observation of Fibrosis in the Lung Clinical Endpoints) study is a currently enrolling, prospective cohort study designed to tackle these issues.
authors.affiliation: Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK. t.maher@rbht.nhs.uk
authors.forename: Toby M
authors.initials: TM
authors.lastname: Maher
mesh_terms: D015415:Biomarkers / Q000378:metabolism; D018450:Disease Progression; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D010641:Phenotype; D011237:Predictive Value of Tests; D011506:Proteins / Q000378:metabolism*; D040901:Proteomics / Q000379:methods; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D011506:Proteins
keywords: Biomarkers; PROFILE study; clinical management; idiopathic pulmonary fibrosis; pharmacological treatment; prognosis