| Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Part 5–Other Forms of Interstitial Lung Disease |
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Hyung Koo Kang, M.D., Ph.D.1, Sun Mi Choi, M.D., Ph.D.2, Hong-Joon Shin, M.D., Ph.D.3, Hae In Jung, M.D.4, Uiri An, M.D.5, Sei Hoon Yang, M.D., Ph.D.6 |
1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Gyeonggi, Republic of Korea
2Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea
3Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Chonnam National University Hospital, Chonnam National University College of Medicine, Gwangju, Republic of Korea
4Division of Pulmonary and Allergy Medicine, Department of Internal Medicine, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Republic of Korea
5Department of Internal Medicine, The Armed Forces Capital Hospital, Seongnam, Gyeonggi, Republic of Korea
6Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Wonkwang University School of Medicine, Iksan, Republic of Korea |
Correspondence:
Sei Hoon Yang, Tel: 82-63-859-2582, Fax: 82-63-855-2025,
Email: yshpul@wku.ac.kr
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Received: 9 December 2024 • Revised: 4 February 2025 • Accepted: 8 March 2025
*Hyung Koo Kang and Sun Mi Choi contributed equally to this study as co-first authors. |
| Abstract |
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Rare forms of interstitial lung diseases (ILDs) present with unique clinical features and require different treatment strategies. Respiratory bronchiolitis-associated ILD mainly affects smokers, showing ground-glass opacities on chest computed tomography (CT) scans and pigmented macrophages in the bronchoalveolar lavage fluid. Smoking cessation is essential for treatment, with corticosteroids used for severe cases. Desquamative interstitial pneumonia, also related to smoking, is characterized by exertional dyspnea, dry cough, restrictive lung function, and ground-glass opacities on high-resolution CT. Lymphoid interstitial pneumonia involves lymphocytic proliferation and is associated with autoimmune diseases or infections, treated with corticosteroids. Acute interstitial pneumonia resembles acute respiratory distress syndrome but occurs without a clear cause and is managed with supportive care. Idiopathic pleuroparenchymal fibroelastosis results in fibrosis in the upper lobes, primarily in nonsmokers, and is diagnosed through clinical and imaging findings, with no effective treatment to improve survival. Each condition has distinct pathological features, clinical presentations, and treatment approaches, along with variable prognoses. |
| Key Words:
Interstitial lung disease, Respiratory bronchiolitis-associated interstitial lung disease, Desquamative interstitial pneumonia, Lymphoid interstitial pneumonia, Acute interstitial pneumonia, Idiopathic pleuroparenchymal fibroelastosis |
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