Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases

Abstract

Interstitial lung disease (ILD) comprises a heterogeneous group of disorders characterizedby interstitial compartment proliferation, inflammatory infiltration, and potentialfibrosis with abnormal collagen deposition. Diagnosis requires a multidisciplinary consensusintegrating clinical, radiological, and pathological findings. Idiopathic interstitialpneumonia (IIP) includes idiopathic pulmonary fibrosis (IPF), idiopathic nonspecific interstitialpneumonia, desquamative interstitial pneumonia, acute interstitial pneumonia,and respiratory bronchiolitis-ILD, each exhibiting distinct prognostic and therapeuticimplications. Some non-IPF ILDs progress despite standard treatment, classified asprogressive fibrosing-ILD or progressive pulmonary fibrosis (PPF), diagnosed by worseningsymptoms, physiological decline, and radiological progression. Nintedanib isconditionally recommended for refractory PPF cases. Combined pulmonary fibrosis andemphysema is characterized by upper-lobe predominant emphysema and lower-lobefibrosis, frequently complicated by pulmonary hypertension and lung cancer. Interstitiallung abnormality, observed in both smokers and the general population, is associatedwith increased mortality and disease risk, warranting further research. Despite advancements,refinement in classification, diagnostic criteria, and therapeutic strategiesremains crucial for improving patient outcomes.