Diagnosis and treatment of cystic lung disease

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.

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

Publication types

  • Review

MeSH terms

  • Amyloidosis / diagnosis
  • Birt-Hogg-Dube Syndrome / diagnosis
  • Cysts / diagnosis
  • Cysts / etiology
  • Cysts / therapy
  • Histiocytosis, Langerhans-Cell / diagnosis
  • Humans
  • Lung Diseases / diagnosis*
  • Lung Diseases / etiology
  • Lung Diseases / therapy*
  • Lung Diseases, Interstitial / diagnosis
  • Lung Neoplasms / diagnosis
  • Lymphangioleiomyomatosis / diagnosis

Supplementary concepts

  • Cystic Disease Of Lung
  • Lymphoid Interstitial Pneumonia