Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.

Abstract

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.