Pulmonary lymphangioleiomyomatosis

A diagnostic challenge in a developing country

Abstract

Lymphangioleiomyomatosis is a rare neoplastic disease that almost exclusively affects young women, with an incidence of 3 to 8 cases per 1 million women, caused by an abnormal proliferation of smooth muscle–like cells. It´s characterized by cystic lung destruction, chylous pleural effusions, lymphangioleiomyomas, and angiomyolipomas that eventually lead to respiratory failure. LAM present clinically as one of two forms, either sporadic or as a tuberous sclerosis complex. We present the case of a 39-year-old, previously healthy female, with a history of chronic progressive dyspnea, dry cough whose initial radiologic studies showed a right pleural effusion and an interstitial lung pattern. We eventually arrived on the diagnosis of pulmonary lymphangioleiomyomatosis, but despite this, the patient evolved poorly. In conclusion, lymphangioleiomyomatosis is an extremely rare and complex disease that requires effective investigative capacities.