LYMPHANGIOLEIOMYOMATOSIS

Course overview

LYMPHANGIOLEIOMYOMATOSIS (LAM) IS A PRIMARY DISEASE OF THE LUNG PARENCHYMA CAUSED BY ABNORMAL GROWTH OF SMOOTH MUSCLE CELLS IN THE LUNG VASCULATURE, LYMPHATICS, AND ALVEOLI THAT LEADS TO THE FORMATION OF MULTIPLE CYSTS IN THE LUNGS BILATERALLY AND RESPIRATORY SYMPTOMS, SUCH AS DYSPNEA ON EXERTION. THE MAIN INVOLVED ORGANS ARE THE LUNGS, BUT LAM CAN HAVE EXTRAPULMONARY MANIFESTATIONS IN THE KIDNEYS, CAUSING BENIGN RENAL ANGIOMYOLIPOMAS, OR CAN SOMETIMES CAUSE PERIVASCULAR EPITHELIOID CELL TUMORS WITH VISCERAL ORGAN INVOLVEMENT. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY, PRESENTATION, AND DIAGNOSIS OF LYMPHANGIOLEIOMYOMATOSIS AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.