特发性肺纤维化治疗进展

Treatment progress of idiopathic pulmonary fibrosis

特发性肺纤维化（idiopathic pulmonary fibrosis, IPF）是指原因不明并以普通型间质性肺炎（UIP）为特征性病理改变的一种慢性炎症性间质性肺疾病，主要表现为弥漫性肺泡炎、肺泡单位结构紊乱和肺纤维化。近来对IPF的治疗研究焦点开始从传统的抗炎治疗向以肺泡上皮和成纤维细胞为主要治疗靶点的抗纤维化治疗转变，一些抗纤维化的药物，如吡啡尼酮、干扰素γ-1b、秋水仙碱等已显示出较理想的治疗前景，在国外已进入Ⅱ期或Ⅲ期临床试验，其确切疗效亦尚待证实。而细胞因子拮抗剂和基因疗法则是目前治疗研究的热点。本文将对当前常用的治疗方法及现代治疗的新进展作一概述。

Idiopathic pulmonary fibrosis （IPF） is a chronic inflammatory interstitial lung disease whose etiopathogenisis is still unclear,with the pathological pattern of usual interstitial pneumonia （UIP）. The disease mainly manifests as diffuse alveolitis,alveolus unit disorganization and lung fibrosis. Recently the loci of IPF treatment research have changed from the traditional anti-inflammation to anti-fibrosis which mainly targets alveolar epithelium and fibroblast, some anti-fibrotic medicines, like pirfenidone, interferon gamma-lb,colchicine etc have demonstrated an ideal treatment prospect,and entered Ⅱ stage or Ⅲ stage of clinical test in overseas,its certain curative effect still needs to be confirmed. Anticytokin and gene therapy are the hot spots of investigation at present. This review makes an outline to the current treatment progress of IPF.