Extract
Lymphangioleiomyomatosis (LAM) is a rare multi-system lung disease that may involve kidneys (e.g., angiomyolipomas [AML]) and lymphatics (e.g., lymphangioleiomyomas, chylous effusions). LAM occurs with increased frequency in patients with Tuberous Sclerosis Complex (TSC), an autosomal-dominant neurocutaneous disorder, associated with mutations in the TSC1 and TSC2 genes [1, 2]. LAM predominantly affects women of childbearing age although it is found as well in post-menopausal women. Patients with LAM develop cysts throughout their lungs, leading to a reduction of pulmonary function, e.g. forced expiratory volume in 1 s (FEV1) or diffusion capacity (DLco) [2]. Although LAM has been described as a diffuse cystic lung disease with a homogeneous distribution [2, 3], we report here that LAM patients have more cystic disease in the middle regions of the lung with effects predominately on FEV1 [2].
Footnotes
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Conflict of interest: Mr. Hasani has nothing to disclose.
Conflict of interest: Dr. Matthew has nothing to disclose.
Conflict of interest: Ms. Goljamali has nothing to disclose.
Conflict of interest: Dr. Chen has nothing to disclose.
Conflict of interest: Dr. Stylianou has nothing to disclose.
Conflict of interest: Dr. Pirooznia has nothing to disclose.
Conflict of interest: Dr. DaSilva has nothing to disclose.
Conflict of interest: Dr. Chen has nothing to disclose.
Conflict of interest: Dr. Wen has nothing to disclose.
Conflict of interest: Dr. Moss has nothing to disclosure.
- Received October 7, 2020.
- Accepted November 1, 2020.
- Copyright ©ERS 2021