特发性肺纤维化发病机制及血管新生作用的研究进展

Advance of the pathogenesis of idiopathic pulmonary fibrosis and angiogenesis

特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)是一种原因不明的进行性疾病,局限于肺部的弥漫性肺泡炎和肺泡结构紊乱,最终导致以肺纤维化伴蜂窝状改变为特征。因其进展快、死亡率高,被世界卫生组织称为肺系疑难疾病。近年来,IPF发病机制及治疗靶点研究已成为国际研究的热点,但尚无有效治疗措施。因此,加深对IPF发病机制的阐明对了解疾病的发生、发展及防治具有重要意义。目前,血管新生与IPF关系已逐渐成为国际研究热点。文中主要就IPF发病机制最新研究进展及血管新生在IPF中的作用进行综述。

Idiopathic pulmonary fibrosis （IPF） is a diffuse alveolitis and alveolar structural disorder which is unexplained, progressive and confined to the lungs. Uhimately, it leads to typical pulmonary fibrosis with honeycomb change. For its rapid progres- sion and high mortality, IPF is called refractory disease in the pulmonary system by the World Health Organization. In recent years, the study of IPF pathogenesis and therapeutic targets has become the hotspot of international research, however, there haven＇t been effec-tive treatment measures till now. Consequently, it is significant for knowing the occurrence, development and prevention of the disease to deepen the understanding of idiopathic pulmonary fibrosis mechanism. Presently, the relations between angiogenesis and idiopathic pulmonary fibrosis have gradually become the hot points of international research. It will summarize the effects of major progress from the latest studies on the pathogenesis of IPF and the role of angiogenesis in IPF in this paper.