Report two case of lymphangioleiomyomatosis (LAM) with presentation of recurrent pneumothorax

Abstract

Pulmonary Lymphangioleiomyomatosis (LAM) is a rare and female-dominant disease and associated with smooth muscle cell proliferation, (LAM) which presents with diffuse progressive destruction of the pulmonary parenchyma. The incidence is less than 1 per million, which results in cystic lung disease and presente commonly with dyspnea and pneumothorax. Work up for diagnosis and exclusion other disease as: Sjögren’s syndrome type A, B and mazement of angiotensin-converting enzyme (ACE) levels, alpha-1-antitrypsin levels, and vascular endothelial growth factor (VEGF) anti bodies. Definitive diagnosis limited only to tissue sampling. No effective treatments are currently proposed for this disease. Two 49 and 39year-old woman who was referred with recurrent pneumothorax, after thoracotomy and resection, diagnosed was made with pulmonary Lymphangioleiomyomatosis. After pulmonary Lymphangioleiomyomatosis was performed, they treated with anti-estrogen therapy and symptomatic supportive care. In follow up pneumothorax was not occurred again. Pulmonary Lymphangioleiomyomatosis is a very rare disease that cannot be effectively cured.