Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis
复制标题
DOI：
10.1055/s-0034-1393036
复制
发表时间：
2015-10-01
期刊：
影响因子：
1.2
通讯作者：
Prasse, A.
中科院分区：
文献类型：
--
作者：
Prasse, A.
特发性肺纤维化（IPF）是最常见的特发性间质性肺炎，也是老年人的一种疾病。吸烟和长期暴露于损害肺泡上皮细胞的物质是IPF发生的风险因素。也有遗传易感性的证据。IPF定义为特发性间质性肺纤维化（UIP）。IPF的诊断是复杂的，并且基于排除与UIP模式相关的其他疾病。唯一的治疗方法是肺移植。近年来，IPF的治疗取得了突破性进展。吡非尼酮和尼达尼布目前有两种化合物获批用于治疗IPF。
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF.