Korean J Intern Med.  2017 Mar;32(2):229-238. 10.3904/kjim.2016.242.

Diagnosis and treatment of cystic lung disease

• Park S
• Lee EJ

• KMID: 2371644
• DOI: http://doi.org/10.3904/kjim.2016.242

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.

Keyword

Cystic lung disease; Lymphangioleiomyomatosis; Histiocytosis, Langerhans-cell; Birt-Hogg-Dube syndrome

MeSH Terms

Amyloidosis
Birt-Hogg-Dube Syndrome
Bronchiolitis
Diagnosis*
Histiocytosis, Langerhans-Cell
Lung Diseases*
Lung*
Lymphangioleiomyomatosis