Presentation
Chronic progressive dyspnea
Patient Data
Both lungs show multiple wide-spread thin-walled cysts of variable sizes. Some of them have coalesced into larger cysts. These cysts are seen replacing most of the lung parenchyma.
Case Discussion
The clinical presentation along with the radiographic features are suggestive of lymphangioleiomyomatosis (LAM). It is a rare multisystem disorder that almost exclusively affects women of childbearing age. Patients usually present with exertional dyspnea. As a cystic lung disease, it can lead to recurrent pneumothorax (occurs in up to 80% of cases). The hallmark of the disease is the presence of uniformly distributed multiple thin-walled cysts throughout the lungs, some of them may coalesce in late disease.
As a multisystem disorder, it can affect other organs; please refer to the original article lymphangioleiomyomatosis for more information about other manifestations and associations of the disease.
Unable to process the form. Check for errors and try again.