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.