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隐源性纤维性肺泡炎中的腱生蛋白免疫反应性

Tenascin immunoreactivity in cryptogenic fibrosing alveolitis.

作者信息

Wallace W A, Howie S E, Lamb D, Salter D M

机构信息

Department of Pathology, Edinburgh University Medical School, U.K.

出版信息

J Pathol. 1995 Apr;175(4):415-20. doi: 10.1002/path.1711750409.

Abstract

Tenascin is a hexameric extracellular matrix (ECM) glycoprotein which has been demonstrated to have a temporal relationship with active scar formation in adult tissues. We hypothesized that this ECM protein might therefore serve to identify areas of active scarring in lung biopsies from patients with cryptogenic fibrosing alveolitis (CFA). The distribution of tenascin was examined in open lung biopsies from ten patients with CFA, six patients with sarcoidosis, and six pulmonary resection specimens from patients with no evidence of interstitial lung disease, using an immunohistochemical technique. Immunoreactive tenascin was not identified in histologically normal control lung parenchyma and was only focally found around large aggregates of granulomas in sarcoidosis. In the CFA, tenascin production was demonstrated in minimally damaged alveolar walls and areas of active disease but not in end-stage scarred lung. There was considerable local heterogeneity of staining within cases, which did not appear to relate to the density of the local inflammatory infiltrate. Large plaques of tenascin were noted to be particularly associated with hyperplastic type II alveolar epithelial lining cells, which are recognized to produce fibrogenic cytokines. The examination of tenascin expression in open lung biopsies from patients with CFA may be useful in assessing fibrogenic activity and may thus provide additional prognostic information.

摘要

腱生蛋白是一种六聚体细胞外基质(ECM)糖蛋白,已被证明在成体组织中与活跃的瘢痕形成存在时间上的关联。我们据此推测,这种ECM蛋白可能有助于识别隐源性纤维化肺泡炎(CFA)患者肺活检中活跃瘢痕形成的区域。采用免疫组织化学技术,对10例CFA患者、6例结节病患者的开胸肺活检标本以及6例无间质性肺疾病证据患者的肺切除标本中腱生蛋白的分布进行了检测。在组织学正常的对照肺实质中未发现免疫反应性腱生蛋白,在结节病中仅在大的肉芽肿聚集周围局灶性发现。在CFA中,腱生蛋白在轻度受损的肺泡壁和活跃病变区域有表达,但在终末期瘢痕化肺中无表达。病例内染色存在相当大的局部异质性,这似乎与局部炎症浸润的密度无关。注意到腱生蛋白的大片状表达特别与增生性II型肺泡上皮衬里细胞相关,已知这些细胞可产生促纤维化细胞因子。检测CFA患者开胸肺活检标本中腱生蛋白的表达可能有助于评估纤维化活性,从而可能提供额外的预后信息。

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