New Guidelines for Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common and fatal idiopathic interstitial pneumonia and is characterized by chronic progressive pulmonary fibrosis of indeterminate etiology. In 2018, the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society published joint clinical practice guidelines for IPF. The guidelines require exclusion of known causes of interstitial lung disease and identification of a radiological and/or pathologic pattern of usual interstitial pneumonia (UIP). The diagnosis of IPF is a multidisciplinary process, involving pulmonologists, radiologists, pathologists and, if necessary, experts in other medical fields. The 2022 guidelines for IPF revisit and clarify the radiological and pathological features of UIP. In addition, recommendations regarding transbronchial lung cryobiopsy, antacid medication, and antireflux surgery are revised or established based on up-to-date evidence in the 2022 guidelines. The new guidelines also encompass the definition and treatment of progressive pulmonary fibrosis (PPF). PPF comprises diverse fibrotic interstitial lung diseases other than IPF, which progress despite standard treatment. The diagnosis of PPF is based on symptoms, physiologic evidence, and radiologic evidence of progression. The use of nintedanib was suggested for patients with PPF other than IPF who are unresponsive to standard treatment. This review introduces and discusses the recommendations for the diagnosis and treatment of IPF and PPF in the new international guidelines.