Saeed Khan, Mazhar Ali Khan, Arshad Javaid.
Rare lung disease: lymphangioleiomyomatosis.
Pak J Chest Med Jan ;21(2):76-9.

Lymphangioleiomyomatosis (LAM) is an exceptionally rare disease involving pausal women. The etiology is unknown. Many organ systems are involved but generally patient present with pulmonary manifestations varying from simple cough to heamoptysis, recurrent pneumothoraces and even complicated pleural effusion. It may be associated with tuberous sclerosis. The wide range of symptoms and signs make the differential diagnosis vary extensive and the physician must be familiar with the disorder to arrive to the correct diagnosis. Treatment include estrogen ablatine therapies. Most patients eventually require lung transplant. Here we report a case of a 28 year old lady whose initial symptoms were cough and dyspnea. Later developed pneumothorax bilaterally. She had a course of antitubercolous treatment before being diagnosed as case of Lymphangioleiomyomatosis. The relevant literature review regarding the case is also provided.

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