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Am J Respir Crit Care Med. 2015 Jul 1;192(1):17-29. doi: 10.1164/rccm.201411-2096CI.

Diffuse Cystic Lung Disease. Part II.

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 have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management.

KEYWORDS:

Birt-Hogg-Dubé syndrome; Sjögren syndrome; follicular bronchiolitis; high-resolution computed tomography; lymphoid interstitial pneumonia

PMID:
25906201
PMCID:
PMC5447298
DOI:
10.1164/rccm.201411-2096CI
[Indexed for MEDLINE]
Free PMC Article

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