Immature Smooth Muscle Infiltration in Sporadic Lymphangioleiomyomatosis: A Case Study.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that causes progressive pulmonary damage. It typically affects young reproductive-age females with tuberous sclerosis complex (TSC). The clinical manifestations of LAM result from the progressive invasion of abnormal smooth muscle cells into lung parenchyma, lymphatics, or pulmonary vasculature. A 43-year-old female presented to the emergency department with a 4-week history of radiating chest pain and shortness of breath. The workup revealed multiple bilateral, small, parenchymal cysts and a left spontaneous pneumothorax. A wedge biopsy confirmed the diagnosis of lymphangioleiomyomatosis. Although LAM is often associated with TSC, it can also be sporadic. Treatment often relies on the degree of lung function and extent of lung damage.