Marie I, Cailleux N, Henry J, Janvresse A, Muir J F, Lévesque H, Courtois H
Département de médecine interne, CHU, Rouen, France.
Rev Med Interne. 1999 Sep;20(9):806-9. doi: 10.1016/s0248-8663(00)88689-0.
Lymphangioleiomyomatosis is an uncommon disorder of unknown origin, which exclusively occurs in women of reproductive age. The condition is characterized by proliferation of immature smooth muscle cells throughout the lungs, i.e., in the peribronchial, perilymphatic, and perivascular areas. This results in obliteration of the respiratory tract and in the development of cysts. Lymphangioleiomyomatosis has a poor prognosis due to both numerous lung complications and progression of the disease to respiratory failure.
We report the case of a patient in whom lymphangioleiomyomatosis was fortuitously diagnosed from chest CT scan, itself performed for the diagnosis of pulmonary embolism. This case is therefore of particular interest.
Our results suggest that the prevalence of lymphangioleiomyomatosis is probably underestimated due to its clinical latency and the absence of specific laboratory tests. Therefore, the development of non-invasive radiological methods should permit early diagnosis of the disease.
淋巴管平滑肌瘤病是一种病因不明的罕见疾病，仅发生于育龄女性。该病的特征是未成熟平滑肌细胞在整个肺部增殖，即在支气管周围、淋巴管周围和血管周围区域。这导致呼吸道闭塞并形成囊肿。由于众多肺部并发症以及疾病进展为呼吸衰竭，淋巴管平滑肌瘤病预后不良。
我们报告了一例患者，其淋巴管平滑肌瘤病是在因诊断肺栓塞而进行的胸部CT扫描中偶然发现的。因此，该病例特别值得关注。
我们的结果表明，由于淋巴管平滑肌瘤病的临床隐匿性以及缺乏特异性实验室检查，其患病率可能被低估。因此，开发非侵入性放射学方法应能实现该病的早期诊断。
