Lymphangioleiomyomatosis and Other Cystic Lung Diseases

Immunol Allergy Clin North Am. 2023 May;43(2):359-377. doi: 10.1016/j.iac.2023.01.003.

Abstract

Cysts and cavities in the lung are commonly encountered on chest imaging. It is necessary to distinguish thin-walled lung cysts (≤2 mm) from cavities and characterize their distribution as focal or multifocal versus diffuse. Focal cavitary lesions are often caused by inflammatory, infectious, or neoplastic processes in contrast to diffuse cystic lung diseases. An algorithmic approach to diffuse cystic lung disease can help narrow the differential diagnosis, and additional testing such as skin biopsy, serum biomarkers, and genetic testing can be confirmatory. An accurate diagnosis is essential for the management and disease surveillance of extrapulmonary complications.

Keywords: Amyloidosis; Birt-Hogg-Dubé syndrome (BHD); Cystic lung disease; Light chain deposition disease; Lymphangioleiomyomatosis (LAM); Lymphoid interstitial pneumonia; Pulmonary Langerhans cell histiocytosis (LCH).

Publication types

  • Review

MeSH terms

  • Birt-Hogg-Dube Syndrome* / complications
  • Birt-Hogg-Dube Syndrome* / diagnosis
  • Birt-Hogg-Dube Syndrome* / pathology
  • Cysts* / complications
  • Cysts* / diagnosis
  • Cysts* / pathology
  • Diagnosis, Differential
  • Histiocytosis, Langerhans-Cell* / complications
  • Histiocytosis, Langerhans-Cell* / diagnosis
  • Histiocytosis, Langerhans-Cell* / pathology
  • Humans
  • Lung / pathology
  • Lung Diseases* / diagnosis
  • Lung Diseases* / etiology
  • Lymphangioleiomyomatosis* / diagnosis
  • Lymphangioleiomyomatosis* / etiology
  • Lymphangioleiomyomatosis* / therapy
  • Tomography, X-Ray Computed / methods

Supplementary concepts

  • Cystic Disease Of Lung