RARE LUNG DISEASE: LYMPHANGIOLEIOMYOMATOSIS

Abstract

Lymphangioleiomyomatosis (LAM) is an exceptionally rare disease involvingpausal women. The etiology is unknown. Many organ systems are involved butgenerally patient present with pulmonary manifestations varying from simplecough to heamoptysis, recurrent pneumothoraces and even complicatedpleural effusion. It may be associated with tuberous sclerosis. The wide rangeof symptoms and signs make the differential diagnosis vary extensive and thephysician must be familiar with the disorder to arrive to the correct diagnosis.Treatment include estrogen ablatine therapies. Most patients eventuallyrequire lung transplant.Here we report a case of a 28 year old lady whose initial symptoms were coughand dyspnea. Later developed pneumothorax bilaterally. She had a course ofantitubercolous treatment before being diagnosed as case ofLymphangioleiomyomatosis. The relevant literature review regarding the caseis also provided.