Evaluating disease severity in idiopathic pulmonary fibrosis

Abstract

Accurate assessment of idiopathic pulmonary fibrosis (IPF) disease severity is integral to the care provided to patients with IPF. However, to date, there are no generally accepted or validated staging systems. There is an abundance of data on using information acquired from physiological, radiological and pathological parameters, in isolation or in combination, to assess disease severity in IPF. Recently, there has been interest in using serum biomarkers and computed tomography-derived quantitative lung fibrosis measures to stage disease severity in IPF. This review will focus on the suggested methods for staging IPF, at baseline and on serial assessment, their strengths and limitations, as well as future developments.