Viskum K
Dept of Pulmonary Medicine, Bispebjerg Hospital, Copenhagen, Denmark.
Monaldi Arch Chest Dis. 1993;48(3):233-6.
Lymphangioleiomyomatosis (LAM) is an interstitial lung disease seen virtually only in women of childbearing age. The diagnosis, symptoms, paraclinical investigations including the use of CT and spirometry are reviewed. A hormonal dependency of LAM seems obvious, but the exact mechanism has never been understood. Oestrogen and Progesterone receptors may or may not be present in the myoma tissue in LAM. The receptor status seems to have no influence on the results of treatment with medroxyprogesterone or oophorectomy. In spite of the uncertain results of hormonal treatment a therapeutic trial with medroxyprogesterone 200 mg i.m. weekly is warranted. If no improvement is observed bilateral oophorectomy is recommended. Death due to respiratory insufficiency is common, but prognosis seems better than previously though, survivors for more than 20 years after diagnosis being described.
淋巴管平滑肌瘤病（LAM）是一种几乎仅见于育龄期女性的间质性肺疾病。本文回顾了其诊断、症状以及包括CT和肺活量测定在内的临床辅助检查。LAM对激素的依赖性似乎很明显，但确切机制从未被阐明。LAM的肌瘤组织中可能存在或不存在雌激素和孕激素受体。受体状态似乎对甲羟孕酮治疗或卵巢切除术的结果没有影响。尽管激素治疗的结果尚不确定，但仍有必要进行每周一次200mg甲羟孕酮肌肉注射的治疗试验。如果未观察到改善，则建议进行双侧卵巢切除术。呼吸功能不全导致的死亡很常见，但预后似乎比以前要好，已有诊断后存活超过20年的病例报道。
