LAM is a rare, proliferative disease affecting the lungs, kidneys and axial lymphatic of abnormal smooth muscles, and exhibits features of tumor. The characteristics of this disease are progressive pulmonary dysfunction, cystic destruction of lungs and abdominal tumors. It usually occurs in young woman. The progressive growth of smooth muscle cells in LAM can obstruct the bronchioles, leading to obstruction of airways and pneumothorax. LAM usually shows symptoms like chest pain, hemoptysis, dyspnea and cough. It causes death within 10 years of diagnosis because of respiratory failure.
HOW IS LYMPHANGIOLEIOMYOMATOSIS DIAGNOSED?
LAM may occur in approximately 30-40% of patient with tuberous sclerosis.