Diffuse Cystic Lung Disease. Part I

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 inc...

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Bibliographic Details
Published in:American journal of respiratory and critical care medicine 2015-06, Vol.191 (12), p.1354-1366
Main Authors: Gupta, Nishant, Vassallo, Robert, Wikenheiser-Brokamp, Kathryn A, McCormack, Francis X
Format: Article
Language:English
Subjects:
Concise Clinical Review
Histiocytosis, Langerhans-Cell - complications
Humans
Infection - complications
Lung - pathology
Lung Diseases - etiology
Lung Diseases, Interstitial - complications
Lymphangioleiomyomatosis - complications
Neoplasms - complications
Smoking - adverse effects
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Summary: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.
ISSN:1073-449X
1535-4970
DOI:10.1164/rccm.201411-2094CI