Corrin B, Butcher D, McAnulty B J, Dubois R M, Black C M, Laurent G J, Harrison N K
Department of Pathology, National Heart and Lung Institute, Royal Brompton Hospital, London, UK.
Histopathology. 1994 Feb;24(2):145-50. doi: 10.1111/j.1365-2559.1994.tb01293.x.
To study the role of transforming growth factor-beta 1 (TGF-beta 1) in the pathogenesis of pulmonary fibrosis we have examined lung biopsies from nine patients with systemic sclerosis and interstitial lung disease, eight with 'lone' cryptogenic fibrosing alveolitis, two with cystic fibrosis, two with extrinsic allergic alveolitis, two with Langerhans' cell histiocytosis, one with lymphangioleiomyomatosis, one with giant cell interstitial pneumonia, and one adenocarcinoma of the lung. In cryptogenic fibrosing alveolitis, both 'lone' and associated with systemic sclerosis alveolar macrophages, bronchial epithelium and hyperplastic type II pneumonocytes expressed intracellular TGF-beta 1. Extracellular TGF-beta 1 was found in the fibrous tissue immediately beneath the bronchial and hyperplastic alveolar epithelium. In normal lung, however, the alveolar epithelium and alveolar interstitium were negative for both forms of TGF-beta 1. There was strong expression of TGF-beta 1 in hyperplastic mesothelium and its underlying connective tissue and in Langerhans' cells in the two cases of histiocytosis. In the organizing pneumonia in cystic fibrosis, the intraalveolar buds of granulation tissue reacted strongly for the extracellular form of TGF-beta 1 and the overlying hyperplastic epithelium expressed the intracellular form. In lymphangioleiomyomatosis, the aberrant smooth muscle cells strongly expressed intracellular TGF-beta 1 and the extracellular form was expressed in the adjacent connective tissue. In giant cell interstitial pneumonia, the numerous alveolar macrophage including the multinucleate forms, expressed intracellular TGF-beta 1, as did the hyperplastic alveolar epithelium.(ABSTRACT TRUNCATED AT 250 WORDS)
为研究转化生长因子β1（TGF-β1）在肺纤维化发病机制中的作用，我们检测了9例系统性硬化症合并间质性肺病患者、8例“特发性”隐源性纤维性肺泡炎患者、2例囊性纤维化患者、2例外源性过敏性肺泡炎患者、2例朗格汉斯细胞组织细胞增多症患者、1例淋巴管平滑肌瘤病患者、1例巨细胞间质性肺炎患者及1例肺腺癌患者的肺活检标本。在隐源性纤维性肺泡炎中，无论是“特发性”还是合并系统性硬化症的病例，肺泡巨噬细胞、支气管上皮及增生的II型肺上皮细胞均表达细胞内TGF-β1。在支气管及增生的肺泡上皮下方的纤维组织中发现细胞外TGF-β1。然而，在正常肺组织中，肺泡上皮及肺泡间质的两种形式的TGF-β1均为阴性。在两例组织细胞增多症中，增生的间皮及其下方的结缔组织以及朗格汉斯细胞中TGF-β1表达强烈。在囊性纤维化的机化性肺炎中，肺泡内肉芽组织芽对细胞外形式的TGF-β1反应强烈，其上方增生的上皮表达细胞内形式。在淋巴管平滑肌瘤病中，异常的平滑肌细胞强烈表达细胞内TGF-β1，细胞外形式在相邻结缔组织中表达。在巨细胞间质性肺炎中，众多肺泡巨噬细胞包括多核巨噬细胞表达细胞内TGF-β1，增生的肺泡上皮也如此。（摘要截于250词）
