[Lymphangiomyomatosis of the lungs]

Pulmonary lymphangiomyomatosis (LAM) is a rare disease at the women's child bearing age, characterized by the proliferation of smooth muscles along the lymphatics in the lung, in mediastinal and retroperitoneal lymph nodes as well as in retroperitoneal lymph vessels. The disease manifests itself as a progressive dyspnea, chylous pleural effusions and reccurent pneumothorax. Uncured the disease progresses to a serious respiratory insufficiency and death within a period of ten years since the manifestation of the first symptoms. We studied a female patient in whom the diagnosis of pulmonary lymphangiomyomatosis was established on clinical findings and pathohistologic analysis of biopsy material. The patient was offered progesterone which, according to some authors, stops further progression of the disease. The progesterone treatment was carried out for a year and it is going to be continued. During the period mentioned, repeatedly controlled lung diffusing capacity and arterial blood gas analysis remained basically unchanged compared to these before treatment. It is noteworthy, that once formed changes in the parenchyma are irreversible, so early diagnosis and timely started treatment are of basic importance.