LYMPHANGIOLEIOMYOMATOSIS

Course overview

LYMPHANGIOLEIOMYOMATOSIS (LAM) IS A PRIMARY DISEASE OF THE LUNG PARENCHYMA THAT IS 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, MAINLY DYSPNEA ON EXERTION. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY, PRESENTATION AND DIAGNOSIS OF LYMPHANGIOLEIOMYOMATOSIS AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.