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Published on: March 3, 2023
Spontaneous pneumothorax in diffuse cystic lung diseases
Joseph Cooley1, Yun Chor Gary Lee, Nishant Gupta
1aDepartment 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) often cause spontaneous pneumothorax, a condition with high recurrence. Early CT screening and pleurodesis after the first pneumothorax improve diagnosis and management of these rare lung disorders.
Area of Science:
- Pulmonology
- Radiology
- Genetics
Background:
- Diffuse cystic lung diseases (DCLDs) are characterized by air-filled lung cysts, increasing pneumothorax risk.
- These disorders account for about 10% of primary spontaneous pneumothoraces.
- Lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis are key DCLDs.
Purpose of the Study:
- To review the epidemiology, clinical features, and management of DCLD-associated spontaneous pneumothorax.
- To highlight the importance of early diagnosis and intervention for DCLDs.
- To discuss specific DCLDs including lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis.
Main Methods:
- Literature review focusing on DCLDs and spontaneous pneumothorax.
- Analysis of recent findings on diagnostic strategies and treatment outcomes.
- Emphasis on computed tomography (CT) screening and pleurodesis.
Main Results:
- CT screening for DCLDs post-pneumothorax is cost-effective for early diagnosis.
- Patients with DCLD-associated pneumothorax have a high recurrence rate.
- Pleurodesis after the first pneumothorax is recommended over waiting for recurrence.
Conclusions:
- Spontaneous pneumothorax is a critical diagnostic opportunity for DCLDs.
- Early diagnosis and management of DCLDs are crucial due to high pneumothorax recurrence.
- Pleurodesis does not preclude future lung transplantation.
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