Opacić M, Sarac R, Mihelcić-Dabo R, Dimov-Butković D
Lijec Vjesn. 1989 Nov;111(11):387-90.
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.
肺淋巴管平滑肌瘤病（LAM）是一种发生在育龄女性的罕见疾病，其特征是肺内、纵隔和腹膜后淋巴结以及腹膜后淋巴管周围的平滑肌增生。该疾病表现为进行性呼吸困难、乳糜性胸腔积液和反复气胸。若不治疗，疾病会在首次出现症状后的十年内发展为严重的呼吸功能不全并导致死亡。我们研究了一名女性患者，根据临床症状及活检材料的病理组织学分析确诊为肺淋巴管平滑肌瘤病。有作者认为，给该患者使用孕激素可阻止疾病进一步发展。已对该患者进行了一年的孕激素治疗，并将继续进行。在上述治疗期间，与治疗前相比，多次监测的肺弥散功能和动脉血气分析基本保持不变。值得注意的是，一旦肺实质发生改变就不可逆转，因此早期诊断和及时开始治疗至关重要。
