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2015 ; 191
(12
): 1354-66
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Diffuse Cystic Lung Disease Part I
#MMPMID25906089
Gupta N
; Vassallo R
; Wikenheiser-Brokamp KA
; McCormack FX
Am J Respir Crit Care Med
2015[Jun]; 191
(12
): 1354-66
PMID25906089
show ga
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.
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