Park Sanghoon, Lee Eun Joo
Paju SOK Internal Medical Clinic, Paju, Korea.
Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.
Korean J Intern Med. 2017 Mar;32(2):229-238. doi: 10.3904/kjim.2016.242. Epub 2017 Feb 28.
Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. A number of assessment methods that can be used to confirm CLDs are discussed, including high-resolution computed tomography, pathologic approaches, and genetic/ serologic markers, together with treatment modalities, including new therapeutic drugs currently being evaluated. The CLDs covered by this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dube syndrome, lymphocytic interstitial pneumonia/follicular bronchiolitis, and amyloidosis.
囊性肺疾病（CLD）是一组以存在多个囊肿为特征的肺部疾病，囊肿定义为充满空气的透亮区或低衰减区，边界为薄壁（通常<2mm）。随着计算机断层扫描的广泛应用，对CLD的认识有所增加。本文探讨了囊肿形成的机制以及CLD的诊断方法。讨论了一些可用于确诊CLD的评估方法，包括高分辨率计算机断层扫描、病理方法和基因/血清学标志物，以及治疗方式，包括目前正在评估的新型治疗药物。本综述涵盖的CLD包括淋巴管平滑肌瘤病、肺朗格汉斯细胞组织细胞增多症、Birt-Hogg-Dube综合征、淋巴细胞间质性肺炎/滤泡性细支气管炎和淀粉样变性。
