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Curr Opin Pulm Med. 2017 Jul;23(4):323-333. doi: 10.1097/MCP.0000000000000391.

Spontaneous pneumothorax in diffuse cystic lung diseases.

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aDepartment of Internal Medicine, University of Cincinnati, Cincinnati, Ohio, USAbPleural Medicine Unit, Institute for Respiratory HealthcDepartment of Respiratory Medicine, Sir Charles Gairdner HospitaldSchool of Medicine and Pharmacology, University of Western Australia, Perth, Western Australia, AustraliaeDivision of Pulmonary, Critical Care and Sleep Medicine, University of CincinnatifMedical Service, Veterans Affairs Medical Center, Cincinnati, Ohio, USA.



Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis.


DCLDs are responsible for approximately 10% of apparent primary spontaneous pneumothoraces. Computed tomography screening for DCLDs (Birt-Hogg-Dubé syndrome, lymphangioleiomyomatosis, and pulmonary Langerhans cell histiocytosis) following the first spontaneous pneumothorax has recently been shown to be cost-effective and can help facilitate early diagnosis of the underlying disorders. Patients with DCLD-associated spontaneous pneumothorax have a very high rate of recurrence, and thus pleurodesis should be considered following the first episode of spontaneous pneumothorax in these patients, rather than waiting for a recurrent episode. Prior pleurodesis is not a contraindication to future lung transplant.


Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.

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