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Updated: Aug 25, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
High-Resolution Computed Tomography of Cystic Lung Disease
Joanna E Kusmirek1, Cristopher A Meyer1
1Department of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.
Cystic lung diseases (CLDs) involve air-filled spaces in the lungs, often diagnosed with chest CT. Recognizing these conditions and their mimics is crucial for accurate diagnosis and treatment.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Cystic lung diseases (CLDs) are defined by multiple, thin-walled, air-filled spaces in lung tissue.
- Cyst formation can stem from various causes including congenital, autoimmune, inflammatory, infectious, or neoplastic processes.
- Distinguishing CLDs from mimics like emphysema and bronchiectasis is vital to avoid misdiagnosis.
Purpose of the Study:
- To provide an overview of cystic lung diseases (CLDs).
- To highlight the role of chest CT in diagnosing and evaluating CLDs.
- To discuss common diffuse CLDs (DCLDs).
Main Methods:
- Review of literature on cystic lung diseases.
- Emphasis on chest CT findings for diagnosis and workup.
- Classification based on cyst characteristics and distribution.
Main Results:
- Chest CT is instrumental in diagnosing CLDs, guiding further evaluation based on cyst morphology, number, and distribution.
- Diffuse CLDs (DCLDs) are characterized by 10 or more cysts.
- Common DCLDs include lymphangioleiomyomatosis, pulmonary Langerhans' cell histiocytosis, lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, and amyloidosis/light chain deposition disease.
Conclusions:
- Accurate identification of CLDs and their mimics through imaging is essential.
- Chest CT plays a key role in the diagnostic pathway for patients with suspected cystic lung abnormalities.
- Understanding the spectrum of DCLDs aids in appropriate patient management.
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