Classification of cystic lung disease in adults

Classification	Description
Neoplastic	Lymphangioleiomyomatosis – sporadic as well as associated with tuberous sclerosis Pulmonary Langerhans cell histiocytosis, and non-Langerhans cell histiocytoses, including Erdheim Chester disease Other primary and metastatic neoplasms, such as sarcomas, adenocarcinomas, pleuropulmonary blastoma, etc
Genetic/developmental/congenital	Birt-Hogg-Dubé syndrome Proteus syndrome, neurofibromatosis, Ehlers-Danlos syndrome Congenital pulmonary airway malformation, bronchopulmonary dysplasia, etc
Associated with lymphoproliferative disorders	Lymphocytic interstitial pneumonia Follicular bronchiolitis Sjögren syndrome Amyloidosis Light-chain deposition disease
Infectious	Pneumocystis jiroveci Staphylococcal pneumonia Recurrent respiratory papillomatosis Endemic fungal diseases, especially coccidioidomycosis Paragonimiasis
Associated with interstitial lung diseases	Hypersensitivity pneumonitis Desquamative interstitial pneumonia
Smoking related	Pulmonary Langerhans cell histiocytosis Desquamative interstitial pneumonia Respiratory bronchiolitis
Other/miscellaneous	Post-traumatic pseudocysts Fire-eater's lung Hyper-IgE syndrome
DCLD mimics	Emphysema Alpha-1 antitrypsin deficiency Bronchiectasis Honeycombing seen in late-stage scarring interstitial lung diseases

Many DCLDs have overlapping features and can be classified in more than one category. Pulmonary Langerhans cell histiocytosis is classified as both a neoplasm and a smoking-related cystic lung disease. Similarly, desquamative interstitial pneumonia is an interstitial lung disease as well as a smoking-related cystic lung disease. Although classified as a lymphoproliferative disorder, light-chain deposition disease could also be considered under the neoplastic category. Similarly, hyper-IgE syndrome, although classified as other/miscellaneous, could also be classified under the category of infections causing cystic lung disease.

DCLD: diffuse cystic lung disease.

Reprinted with permission of the American Thoracic Society. Copyright © 2015 American Thoracic Society. Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX. Diffuse Cystic Lung Disease. Part I. Am J Respir Crit Care Med 2015; 191(12):1354-66. The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

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