Lymphangioleiomyomatosis

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.

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