Diagnostic approach to the adult with cystic lung disease
- Anupam Kumar, MD
Anupam Kumar, MD
- Clinical Assistant Professor, Division of Pulmonary & Critical Care
- Spectrum Health-Michigan State University College of Human Medicine
- Robert M Kotloff, MD
Robert M Kotloff, MD
- Chairman, Department of Pulmonary Medicine
- Respiratory Institute Cleveland Clinic
- Section Editor
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- Deputy Editors
- Helen Hollingsworth, MD
Helen Hollingsworth, MD
- Deputy Editor — Pulmonary, Critical Care, and Sleep Medicine
- Associate Professor of Medicine
- Boston University School of Medicine
- Geraldine Finlay, MD
Geraldine Finlay, MD
- Senior Deputy Editor — UpToDate
- Deputy Editor — Pulmonary, Critical Care, and Sleep Medicine
- Associate Professor
- Tufts University School of Medicine
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" . (See 'Parenchymal lucencies that may mimic cysts' below.)
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- Parenchymal lucencies that may mimic cysts
- CAUSES OF CYSTIC LUNG DISEASE
- SUSPECTING AN ETIOLOGY FOR CYSTIC LUNG DISEASE
- Clinical history
- - Age, sex, and ethnicity
- - Smoking
- - Family history
- Pulmonary manifestations
- - Pneumothorax
- - Pleural effusion
- Extrapulmonary manifestations
- RADIOGRAPHIC FEATURES
- Appearance and distribution of cysts
- Ground glass opacities
- Pleural effusion
- Intra-abdominal features
- DIAGNOSTIC APPROACH
- Laboratory testing
- - Serologic studies
- - Genetic testing
- Surgical lung biopsy
- Skin biopsy
- SUMMARY AND RECOMMENDATIONS