[Pulmonary lymphangioleiomyomatosis as a very rare interstitial lung disease]

Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting premenopausal women characterized by an abnormal proliferation of smooth muscle cells that leads to the obstruction of airways, lymph and blood vessels. We present a case of a 46-year-old woman who was admitted to our department with dyspnoea and dry cough. The patient had a history of spontaneous pneumothorax 2 years prior to admission. Physical examination revealed dull percussion note on the lower right side of the chest. The chest X-ray film showed diffuse interstitial parenchymal infiltration and flattened costodiaphragmatic angle on the right side. The high resolution computerized tomography (HRCT) scan showed the numerous air filled cysts, about 25 mm in diameter with thin regular walls and liquid in the right pleural cavity. The effusion in a pleural cavity was chylous. Airway obstruction (FEV1/FVC 57% of predicted), markedly elevated residual volume (140%), and decreased DLCO were observed in functional pulmonary tests, and she underwent diagnostic videothoracoscopy. Pulmonary biopsy specimens confirmed diagnosis of LAM. The patient has been under careful observation, no treatment was instituted. The patient remains clinically stabile. During the last six months of observation she has normal sex hormone levels, therefore there exists a possibility of postmenopausal remission of symptoms.