Lymphangioleiomyomatosis

Francis X. McCormack, MD

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In This Article

Introduction

Lymphangioleiomyomatosis (LAM) is a rare, progressive, cystic lung disease that occurs almost exclusively in females, usually between menarche and menopause. The hallmarks of LAM are diffuse infiltration of the pulmonary parenchyma with atypical smooth muscle cells, airflow obstruction, pneumothorax and chylothorax, and progressive respiratory failure. LAM is often initially misdiagnosed as asthma or chronic obstructive pulmonary disease. Although patients are empirically treated with therapies that antagonize the effects of estrogen, there is no good evidence that these strategies are effective.

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