| Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases |
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Chul Park1, Yoomi Yeo2, A La Woo3, Jung Wan Yoo4, Goohyeon Hong5, Jong Wook Shin6, Sung Woo Park7, on behalf of the Korean Interstitial Lung Disease Study Group |
1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Ulsan University Hospital, Ulsan, South Korea
2Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, South Korea
3Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
4Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Gyeongsang National University Hospital, Gyeongsang National University, Jinju, South Korea
5Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Dongguk University Gyeongju Hospital, Dongguk University College of Medicine, Gyeongju, South Korea
6Department of Internal Medicine, Chung-Ang University Hospital, College of Medicine, Chung-Ang University, Seoul, South Korea
7Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, South Korea |
Correspondence:
Sung Woo Park,
Email: swpark@schmc.ac.kr
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Received: 14 March 2025 • Revised: 2 May 2025 • Accepted: 29 May 2025
*Chul Park and Yoomi Yeo contributed equally to this study as co-first authors. |
| Abstract |
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Interstitial lung disease (ILD) comprises a heterogeneous group of disorders characterized by interstitial compartment proliferation, inflammatory infiltration, and potential fibrosis with abnormal collagen deposition. Diagnosis requires a multidisciplinary consensus integrating clinical, radiological, and pathological findings. Idiopathic interstitial pneumonia (IIP) includes idiopathic pulmonary fibrosis (IPF), idiopathic nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), acute interstitial pneumonia (AIP), and respiratory bronchiolitis-ILD (RB-ILD), each exhibiting distinct prognostic and therapeutic implications. Some non-IPF ILDs progress despite standard treatment, classified as progressive fibrosing interstitial lung disease (PF-ILD) or progressive pulmonary fibrosis (PPF), diagnosed by worsening symptoms, physiological decline, and radiological progression. Nintedanib is conditionally recommended for refractory PPF cases. Combined pulmonary fibrosis and emphysema (CPFE) is characterized by upper-lobe predominant emphysema and lower-lobe fibrosis, frequently complicated by pulmonary hypertension and lung cancer. Interstitial lung abnormality (ILA), observed in both smokers and the general population, is associated with increased mortality and disease risk, warranting further research. Despite advancements, refinement in classification, diagnostic criteria, and therapeutic strategies remains crucial for improving patient outcomes. |
| Key Words:
Interstitial Lung Disease, Idiopathic Interstitial Pneumonia, Progressive Pulmonary Fibrosis, Idiopathic Pulmonary Fibrosis, Combined Pulmonary Fibrosis and Emphysema, Pulmonary Fibrosis, Pulmonary Emphysema, Interstitial lung abnormalities |
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