- Author:
Sanghoon PARK
1
;
Eun Joo LEE
Author Information
- Publication Type:Review
- Keywords: Cystic lung disease; Lymphangioleiomyomatosis; Histiocytosis, Langerhans-cell; Birt-Hogg-Dube syndrome
- MeSH: Amyloidosis; Birt-Hogg-Dube Syndrome; Bronchiolitis; Diagnosis*; Histiocytosis, Langerhans-Cell; Lung Diseases*; Lung*; Lymphangioleiomyomatosis
- From:The Korean Journal of Internal Medicine 2017;32(2):229-238
- CountryRepublic of Korea
- Language:English
- Abstract: 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.