Progressive pulmonary fibrosis (PPF)

Abstract

Progressive pulmonary fibrosis (PPF) is defined as the presence of at least two of the three criteria, which are worsening respiratory symptoms, functional decline, and radiological progression in patients with interstitial lung disease with radiological pulmonary fibrosis for known or unknown reasons other than IPF, within the previous year (1). A conditional recommendation has been made for nintedanib in the treatment of PPF, and further studies are needed for pirfenidone (1). In this review, the diagnostic and therapeutic approach to progressive pulmonary fibrosis with its new name, previously known as progressive fibrotic interstitial lung diseases, will be discussed, accompanied by updates.