Birt-Hogg-Dube syndrome

At the time of diagnosis of Birt-Hogg-Dube (BHD) syndrome, a computed tomography (CT) scan, or high resolution CT scan if available, should be done to determine the number, location, and size of any cysts in the lungs.^[1]  There is no recommended management of the lung cysts.  Lung cysts related to BHD have not been associated with long-term disability or fatality.^[2]  The main concern is that the cysts may increase the chance of developing a collapsed lung (pneumothorax).

If an individual with BHD experiences any symptoms of a collapsed lung - such as chest pain, discomfort, or shortness of breath - they should immediately go to a physician for a chest x-ray or CT scan.^[1]  Therapy of a collapsed lung depends on the symptoms, how long it has been present, and the extent of any underlying lung conditions.^[2]  It is thought that collapsed lung can be prevented by avoiding scuba diving, piloting airplanes, and cigarette smoking.^[2][3]

Individuals with BHD who have a history of multiple instances of collapsed lung or signs of lung disease are encouraged to see a lung specialist (pulmonologist).^[3]