Chest
Volume 150, Issue 4, October 2016, Pages 945-965
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Special Features
Cystic Lung Diseases: Algorithmic Approach

https://doi.org/10.1016/j.chest.2016.04.026Get rights and content

Cysts are commonly seen on CT scans of the lungs, and diagnosis can be challenging. Clinical and radiographic features combined with a multidisciplinary approach may help differentiate among various disease entities, allowing correct diagnosis. It is important to distinguish cysts from cavities because they each have distinct etiologies and associated clinical disorders. Conditions such as emphysema, and cystic bronchiectasis may also mimic cystic disease. A simplified classification of cysts is proposed. Cysts can occur in greater profusion in the subpleural areas, when they typically represent paraseptal emphysema, bullae, or honeycombing. Cysts that are present in the lung parenchyma but away from subpleural areas may be present without any other abnormalities on high-resolution CT scans. These are further categorized into solitary or multifocal/diffuse cysts. Solitary cysts may be incidentally discovered and may be an age related phenomenon or may be a remnant of prior trauma or infection. Multifocal/diffuse cysts can occur with lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, tracheobronchial papillomatosis, or primary and metastatic cancers. Multifocal/diffuse cysts may be associated with nodules (lymphoid interstitial pneumonia, light-chain deposition disease, amyloidosis, and Langerhans cell histiocytosis) or with ground-glass opacities (Pneumocystis jirovecii pneumonia and desquamative interstitial pneumonia). Using the results of the high-resolution CT scans as a starting point, and incorporating the patient’s clinical history, physical examination, and laboratory findings, is likely to narrow the differential diagnosis of cystic lesions considerably.

Section snippets

Systematic Approach to Cystic Lung Disease

A summary of the systematic approach to cystic lung disease is given in Figure 6.

Additional Testing (Other than HRCT Scan)

While HRCT scan is extremely useful in diagnosing cystic lung disease, further testing and confirmation may be required to establish a definite diagnosis and to initiate specific treatment. Diseases where confirmation of diagnosis (in the presence of compatible cystic change on HRCT) is recommended prior to initiation of treatment include:

  • 1)

    LAM: Confirmatory features may include the presence of angiomyolipomas, chylous effusions, lymphangioleiomyoma, elevated serum vascular endothelial growth

Conclusions

In this review, we provide a systemic approach to aid clinicians in the diagnosis of cystic lung disease. Rather than classify each diagnosis in a single category, we have taken the most common presentations of each entity and placed them in one or more categories. Characteristics of cysts on HRCT imaging, considered together with clinical features, are helpful in guiding diagnostic and management strategies. First, we differentiate cysts from cavities, panlobular and centrilobular emphysema,

Acknowledgments

Financial/nonfinancial disclosures: The authors have reported to CHEST the following: D. L. is a consultant to Parexel, Inc, Boehringer Ingelheim, Inc, Genentech, Inc, Gilead, Inc, and Veracyte, Inc. He has received support from Parexel, Inc, Siemens, Inc, and the National Heart, Lung, and Blood Institute. D. N. is a consultant to Siemens Medical, Inc for their development of a computer-assisted detection device. None declared: (Suhail Raoof, P. B., R. V., J. R., N. G., Sabiha Raoof, J. G., M.

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