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Am J Respir Crit Care Med. 2015 Jun 15;191(12):1354-66. doi: 10.1164/rccm.201411-2094CI.

Diffuse Cystic Lung Disease. Part I.

Author information

1
1 Division of Pulmonary, Critical Care, and Sleep Medicine and.
2
2 Veterans Affairs Medical Center, Department of Veterans Affairs, Cincinnati, Ohio.
3
3 Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota; and.
4
4 Department of Pathology and Laboratory Medicine, University of Cincinnati, Cincinnati, Ohio.
5
5 Division of Pathology and Laboratory Medicine and.
6
6 Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Abstract

The diffuse cystic lung diseases (DCLDs) are a group of pathophysiologically heterogenous processes that are characterized by the presence of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the pulmonary parenchyma. Although the mechanisms of cyst formation remain incompletely defined for all DCLDs, in most cases lung remodeling associated with inflammatory or infiltrative processes results in displacement, destruction, or replacement of alveolar septa, distal airways, and small vessels within the secondary lobules of the lung. The DCLDs can be broadly classified according to underlying etiology as those caused by low-grade or high-grade metastasizing neoplasms, polyclonal or monoclonal lymphoproliferative disorders, infections, interstitial lung diseases, smoking, and congenital or developmental defects. In the first of a two-part series, we present an overview of the cystic lung diseases caused by neoplasms, infections, smoking-related diseases, and interstitial lung diseases, with a focus on lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.

KEYWORDS:

high-resolution computed tomography; lung cysts; lymphangioleiomyomatosis; pulmonary Langerhans cell histiocytosis; tuberous sclerosis

PMID:
25906089
PMCID:
PMC5442966
DOI:
10.1164/rccm.201411-2094CI
[Indexed for MEDLINE]
Free PMC Article

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