Stovin P G, Lum L C, Flower C D, Darke C S, Beeley M
Thorax. 1975 Oct;30(5):497-509. doi: 10.1136/thx.30.5.497.
Two cases of pulmonary lymphangiomyomatosis (PL) are described and 33 other cases from the literature are reviewed. These are compared with one case of tuberous sclerosis with pulmonary involvement (PTS) and 32 other cases from the literature. There are no differences in lung function between these two conditions, both of which show airways obstruction associated with diffuse radiological lung changes. There are, however, both clinical and radiological differences and also differences in the distribution of the lesions and the histological location of the excessive smooth muscle; these indicate that PL and PTS are probably different entities and not polar forms of one condition. Finally, the strictly female incidence of PL suggests a sex-linked disorder, and it is postulated that this may be related to congenital pulmonary lymphangiectases.
本文描述了两例肺淋巴管肌瘤病（PL），并复习了文献中另外33例病例。将这些病例与1例合并肺部受累的结节性硬化症（PTS）及文献中另外32例病例进行了比较。这两种疾病的肺功能无差异，均表现为与弥漫性肺部影像学改变相关的气道阻塞。然而，在临床和影像学方面存在差异，病变分布及过度平滑肌的组织学位置也存在差异；这些表明PL和PTS可能是不同的实体，而非一种疾病的两极形式。最后，PL严格的女性发病率提示存在性连锁疾病，据推测这可能与先天性肺淋巴管扩张有关。
