Korean Clinical Practice Guidelines for Interstitial Lung Disease: Part 2. Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia marked by declining lung function, severe dyspnea, and a poor prognosis. Antifibrotic agents have improved outcomes in patients with IPF, but diagnosing the disease, monitoring its course, and managing it over the long term remain challenging. Since the publication of the first Korean clinical practice guideline for interstitial lung disease in 2018, research has advanced substantially and has reshaped the clinical management of IPF. This updated guideline draws on evidence published over the past 5 years and covers the epidemiology, pathogenesis, risk factors, clinical manifestations, diagnosis, treatment, comorbidities, prognosis, and acute exacerbations of IPF. Expert consensus was also used to formulate key clinical questions, and systematic reviews and meta-analyses were then performed to reassess how antifibrotic agents affect lung function decline, mortality, and acute exacerbations in patients with IPF. This guideline aims to standardize the clinical management of IPF, improve diagnostic accuracy, and encourage the appropriate use of available treatments. It is intended for physicians, other healthcare professionals who care for patients with IPF, and healthcare policymakers in Korea.