The Air within A Case Report : Lymphangioleiomyomatosis

ABSTRACT: Lymphangiomyomatosis (LAM) is a multi-systemic, rare, progressive and life threatening cystic lung disease that is predominantly sporadic and effects women of childbearing age. It is usually characterized by proliferation of atypical smooth muscle cells around lymphatics, venules and bronchioles. Generally, patient would present with respiratory symptoms varying from simple cough, chest pain, and dyspnea to recurrent pneumothorax as well as pleural effusion. Pneumothorax is almost always present in 50% of the case. Early stages may show normal findings radiographically, but this may progress from retriculo-nodular to cystic of the lung. LAM can often be confused with other disease entities such as bronchiectasis, emphysema, pulmonary Langerhan`s cell histiocytosis (LCH), lymphocytic interstitial pneumonitis and Birt Hogg Dube syndrome (BHD). Because of the similarities of the early signs and symptoms of LAM to other cystic lung disease, diagnostics such as radiography, computed tomography and pulmonary function test are commonly requested to aid in the diagnosis of LAM. This is a case of a 46-year old female who presented with difficulty of breathing and recurrent pneumothorax. Chest radiograph was done revealing repeated collapse of lungs. Likewise chest CT scan showed findings suggestive of Lymphangioleiomyomatosis. However, histopatologic tissue characterization (lung biopsy) remains the definite examination for the diagnosis of this rare disease, which in our patient revealed emphysematous changes with bronchiolectasis. Pulmonary lymphangioleiomyomatosis is progressive and worsens over time. Currently, there has been no cure available to stop the growth and progression of the cysts in LAM. Treatment goal is to ease symptoms and prevent further complication usually by giving medications to improve air flow of lungs, oxygen therapy, remove fluids through chest tubes and aspirations and lung transplantation for severe LAM. There are clinical trials and researches being done to discover and test new treatments for LAM.

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