Tomashefski J F, Dahms B, Bruce M
Arch Pathol Lab Med. 1985 Oct;109(10):910-6.
We studied pleura from patients with cystic fibrosis (CF) in order to define abnormalities that predispose to pneumothorax or are unique to CF. We compared the histology of CF pleura with that of young non-CF adults with "idiopathic" pneumothorax. Both CF and non-CF patients with pneumothorax showed distorted elastic fibers in areas of pleural fibrosis, adhesions, or air "cysts." Following pneumothorax, chronic inflammation, granulation tissue, fibrosis, mesothelial hyperplasia, and reactive eosinophilic pleuritis were also common. Although CF pleura appeared more intensely inflamed, only myxoid connective tissue and vascular proliferation were significantly more frequent in CF. Columnar, vacuolated mesothelial cells (Dunnill lesion) were focally observed only in patients with CF. We conclude that extensive degenerative pleural changes may predispose to pneumothorax in CF and represent a nonspecific response to chronic inflammation.
我们研究了囊性纤维化（CF）患者的胸膜，以确定易导致气胸的异常情况或CF特有的异常情况。我们将CF胸膜的组织学与患有“特发性”气胸的年轻非CF成年人的胸膜组织学进行了比较。患有气胸的CF患者和非CF患者在胸膜纤维化、粘连或空气“囊肿”区域均显示弹性纤维扭曲。气胸后，慢性炎症、肉芽组织、纤维化、间皮增生和反应性嗜酸性胸膜炎也很常见。尽管CF胸膜的炎症似乎更严重，但仅黏液样结缔组织和血管增生在CF中明显更常见。仅在CF患者中局灶性观察到柱状、空泡状间皮细胞（邓尼病变）。我们得出结论，广泛的退行性胸膜改变可能使CF患者易患气胸，并代表对慢性炎症的非特异性反应。
