特发性肺纤维化发病机制的研究进展

特发性肺纤维化是指以肺泡上皮细胞损伤、成纤维细胞大量增生和细胞外基质聚集增多为病理特征且病因不明的一类慢性间质性肺疾病。由于病因不清,目前发病率约为16.3/100,000,且缺乏有效诊疗手段和治疗药物。肺纤维化对人体健康危害极大,愈后困难,存活率较低。因此加深对纤维化机制的阐明对于了解疾病的发生、发展和防治就显得十分必要,更是人类对健康的迫切要求。本文就近几年关于特发性肺纤维化发病机制的研究进展作一简要综述。

Advances in Research of Pathogenesis of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is one of a group of chronic interstitial lung diseases of unknown causes,and is characterized by alveolar epithelial cell injury,abnormal proliferation of fibroblasts and excessive deposition of extracellular matrix.There are limited therapeutic options,poor prognosis,low long-term survival rate and is associated with increasing incidence since the pathogenic mechanisms are elusive.This demands a better understanding of the molecular mechanisms underlying the pathogenesis.It is most essential to know the onset and progression of this disease,especially in relevance to human health.In this paper,we reviewed the advances in research of idiopathic pulmonary fibrosis.