Diagnostic Accuracy of Computed Tomography in Lymphangioleiomyomatosis

Dear Editor,
Lymphangioleiomyomatosis (LAM) is a rare disease classified by the World Health Organization (WHO) as a low-grade perivascular epithelioid tumor. It involves the development of multiple cysts in the epithelial layers due to the proliferation of abnormal smooth muscle cells, known as LAM cells. This disease primarily affects women, typically around the age of 35, and while it is mostly idiopathic, it is associated with tuberous sclerosis in approximately 15% of cases. Early symptoms are often mild, but the disease progresses and is characterized by dyspnea, with a strong association with spontaneous pneumothorax.3 Additionally, about 1% of patients with scleroderma exhibit LAM-like changes in their lungs.

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