RARE LUNG DISEASE: LYMPHANGIOLEIOMYOMATOSIS

Saeed Khan, Mazhar Ali Khan, Arshad Javaid

Abstract


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.


Keywords


Lung disease; Lymphangioleiomyomatosis; Peshawar; Pakistan.

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