博莱霉素
特发性肺纤维化
发病机制
肺纤维化
医学
病理
肺
纤维化
炎症
呼吸道疾病
免疫学
内科学
化疗
作者
James D Williamson,Laura R. Sadofsky,Simon P. Hart
标识
DOI:10.3109/01902148.2014.979516
摘要
Idiopathic pulmonary fibrosis (IPF) is a devastating disease of unknown etiology, for which there is no curative pharmacological therapy. Bleomycin, an anti-neoplastic agent that causes lung fibrosis in human patients has been used extensively in rodent models to mimic IPF. In this review, we compare the pathogenesis and histological features of human IPF and bleomycin-induced pulmonary fibrosis (BPF) induced in rodents by intratracheal delivery. We discuss the current understanding of IPF and BPF disease development, from the contribution of alveolar epithelial cells and inflammation to the role of fibroblasts and cytokines, and draw conclusions about what we have learned from the intratracheal bleomycin model of lung fibrosis.
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