Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia characterized by declining lung function, severe dyspnea, and a poor prognosis. Although antifibrotic agents have improved outcomes in patients with IPF, important challenges remain in the diagnosis, monitoring, and long-term management of the disease. Since the publication of the first Korean clinical practice guideline for interstitial lung disease (ILD) in 2018, substantial advances in research have led to important changes in the clinical management of IPF. This updated guideline incorporates evidence published over the past five years and addresses key aspects of IPF, including epidemiology, pathogenesis, risk factors, clinical manifestations, diagnosis, treatment, comorbidities, prognosis, and acute exacerbations. In addition, key clinical questions (KQs) were developed through expert consensus, and systematic reviews and meta-analyses were conducted to reassess the effects of antifibrotic agents on lung function decline, mortality, and acute exacerbations in patients with IPF. These guidelines aim to standardize the clinical management of IPF, improve diagnostic accuracy, and promote the appropriate use of therapeutic strategies. They are intended for physicians, other healthcare professionals involved in the care of patients with IPF, and healthcare policymakers in Korea.
https://e-trd.org/m/journal/view.php?doi=10.4046/trd.2026.0097
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