Prognosis and prognostic factors for chronic fibrosing idiopathic interstitial pneumonias

Abstract

Progressive lung fibrosis is frequently observed in patients with idiopathic interstitial pneumonias (IIPs), especially in those with idiopathic pulmonary fibrosis (IPF) being a representative form of IIPs characterized by a poor prognosis, even in the era of antifibrotic therapy. Predicting prognosis is essential for informing patients and determining future treatment strategies. Multiple prognostic factors have been reported for these diseases. The international clinical practice guidelines for IPF were repeatedly revised in 2010, 2018, and 2022, and the multidisciplinary classification of IIPs was updated in 2013. These updates have led to changes in how patients are classified under various IIP subtypes. Recent advances in the areas of genetic polymorphisms, radiological image analysis, and deep learning technology have helped identify multiple prognosis predictive factors for IPF and other forms of IIPs. This review provides an updated summary of the prognosis and prognostic predictors of IPF and other chronic fibrotic IIPs, incorporating recent reports published since antifibrotic therapy became the standard treatment for IPF and other forms of progressive pulmonary fibrosis, and covers other latest advancements and technologies as well.