Lymphangioleiomyomatosis (LAM) is a rare idiopathic disorder of unclear origin, which almost exclusively occurs to women before menopause. It is characterized by nonneoplastic diffuse proliferation of atypical immature smooth muscle cells around small airways and vessels. We report the case of a 64-year-old woman kept for chronic progressive dyspnea related to a diffuse LAM.The discussion includes a literature review that describes the physiopathology, clinical features, chest computed tomography aspects, and differential diagnosis regarding this rare disease.Through this case report, pulmonary LAM should be included in the list of differential diagnoses for cases of pulmonary cystic lesions, even in postmenopausal women.
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