[Lung lymphangioleiomyomatosis (LAM)]

Lymphangioleiomyomatosis (LAM) is extreme rare diffuse lung disease of unknown cause seen almost exclusively in women of child-bearing age and rarely postmenopausal which indicates the involvement of hormones-estrogens. It results from proliferation of the cells having a smooth muscle cell phenotype (LAM cells) in the lung, and very often in the kidney and axial lymphatics and lymph nodes or any combination. It may occur sporadically or in association with the neurocutaneous syndrome--tuberous sclerosis. LAM cell proliferation may obstruct bronchioles, lymph vessels and venules that lead to airflow obstruction, formation of lung cysts, bullas and pneumothoraces, chylothorax, chylous ascites, hemosiderosis and hemoptysis. Approxymately 400 cases of LAM have been reported so far, most of them in USA. The average survival is about 8.5 years. There is no specific therapy. There are attempts with progesterone, lung transplantation, Doxycicline besides the symptomatic therapy. A new drug Rapamycin is tested. We are reporting 43-year old woman admitted in the Clinic for pulmonary diseases and TB "Podhrastovi" because of progressive dyspnea and suspect lung diffuse fibrosis, after the surgical treatment of spontaneous pneumothorax. The diagnosis of lung lymphangiomyomatosis was established by chest X ray, computerized chest tomography (CT), pathohistological findings of open lung biopsy. The treatment with progesterone is underway with other symptomatic therapy.