Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology that traditionally affects young women of child bearing or premenopausal age. It is characterized by proliferation of atypical smooth muscle cells, preferentially along bronchovascular structures that causes progressive respiratory failure. Due to its unusual and nonspecific presenting symptoms, patients often receive missed or delayed diagnosis. This disease occurs sporadically or in association with the genetic disease tuberous sclerosis complex (TSC). We present 34-year-old young premenopausal woman with LAM.

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