Gabbrielli S, Stanflin N, Melli G
Pneumology Department, Villa d'Ognissanti, Florence.
Pathologica. 1990 Mar-Apr;82(1078):119-23.
A new framework for pulmonary fibroses may be obtained by distinguishing the "true" forms (solely collagen hyperplasia) from the "false" forms or mesenchymal fibril lung diseases (collagen, reticular and elastic hyperplasia with neo-angiogenesis): the "true fibroses" are then divisible into those not causing architectural subversion of the lung and those which bring it about. Pulmonary architectural subversion is common to true granulomatous fibroses and mesenchymal fibril lung diseases: it is, in our opinion, the product of a combination of factors, including the hyperactivity of a fibroblast sub-population normally in the minority, stimulated by T lymphocytes which are activated by an autoimmune response to type I collagen produced in excess.
通过区分“真性”形式(仅为胶原增生)与“假性”形式或间质性纤维肺病(伴有新生血管形成的胶原、网状和弹性增生),可能会得到一个关于肺纤维化的新框架:“真性纤维化”可再分为不会导致肺结构破坏的和会导致肺结构破坏的两类。肺结构破坏在真性肉芽肿性纤维化和间质性纤维肺病中很常见:我们认为,它是多种因素共同作用的结果,这些因素包括通常占少数的成纤维细胞亚群的过度活跃,该亚群受到T淋巴细胞的刺激,而T淋巴细胞是由对过量产生的I型胶原的自身免疫反应激活的。