Lymphangioleiomyomatosis: A Challenging Case

Abstract

Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease, most prevalent in women, which affects the lung function, axial lymphatics and causes angiomyolipomas. Progressive dyspnea, pneumothorax, chylous effusions and cough are some of the clinical manifestations. It is a disease that can be difficult to manage and can have a poor prognosis, especially if not promptly addressed. We present a case report of a 43-year-old woman who was admitted in 2014 with recurrent chylothorax and diagnosed with LAM. However, since the actual best therapeutic approach, sirolimus, was not yet recommended at the time, together with the patient