Comparing effectiveness of prognostic tests in idiopathic pulmonary fibrosis

Introduction: Idiopathic pulmonary fibrosis (IPF) is a debilitating and progressive fibrotic interstitial lung disease often resulting in death over several years. Prediction of disease course or survival remains of keen interest for clinicians and patients though a commonly used test or tool remain elusive. Areas covered: We undertook a comprehensive review of the published literature highlighting prognostic indicators and predictors of survival in IPF. Baseline and longitudinal clinical, functional, histopathologic, and radiologic findings have been extensively studied as prognostic predictors, both individually and in composite models. Recent approaches include automated quantifiable radiologic scoring, circulating biomarkers, and genetic polymorphisms or abnormalities. This review highlights individual and composite predictors and their relative utility in clinical practice and research studies. Expert opinion: There is a growing body of knowledge highlighting readily available individual and composite predictors of outcome, though none have come to the forefront for common clinical use. Recent advances include quantitative imaging analysis, circulating serologic markers, and genetic testing, which may be more standardized and less prone to lead-time bias or related complications and comorbidities.