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Diagnostic approach to the adult with cystic lung disease

Anupam Kumar, MD
Robert M Kotloff, MD
Section Editor
Talmadge E King, Jr, MD
Deputy Editors
Helen Hollingsworth, MD
Geraldine Finlay, MD


Cystic lung diseases represent a heterogenous group of disorders that share in common the radiographic feature of multiple air-filled lucencies surrounded by discrete walls.

Conditions that are commonly referred to as diffuse cystic lung diseases include lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), Birt-Hogg-Dubé syndrome (BHD), and lymphoid interstitial pneumonia (LIP), although the differential diagnosis can at times expand to encompass other extremely rare etiologies [1,2].

An approach to the diagnosis of diffuse cystic lung disease in adults will be reviewed here. The clinical manifestations, evaluation, and management of the individual causes of diffuse cystic lung disease are discussed in greater detail separately. (See "Sporadic lymphangioleiomyomatosis: Clinical presentation and diagnostic evaluation" and "Pulmonary Langerhans cell histiocytosis" and "Birt-Hogg-Dubé syndrome" and "Lymphoid interstitial pneumonia in adults".)


Cysts and parenchymal lucencies that mimic cystic disease are typically defined by their appearance on high resolution computed tomography (table 1).

Cysts — A pulmonary cyst is defined as a "round parenchymal lucency or low-attenuating area with a well-defined interface with normal lung" [3]. (See 'Parenchymal lucencies that may mimic cysts' below.)

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Literature review current through: Nov 2017. | This topic last updated: Jun 23, 2017.
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