特发性肺间质纤维化合并肺癌的诊疗进展

Advances in Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis with Lung Cancer

特发性肺间质纤维化(IPF)是一种病因未明的慢性进展性致纤维化性间质性肺炎。多项临床研究证实IPF患者易合并肺癌。这两种疾病在发病机制上有一些共同通路,可以成为未来潜在的治疗靶点。由于治疗方案的选择受到限制,IPF合并肺癌(IPF-LC)患者生存期短,预后不佳。近年来出现的两种新药(吡非尼酮和尼达尼布)给IPF-LC的治疗带来了新的转机。本文主要对IPF-LC的流行病学、发病机制、临床特点及治疗的最新研究进展进行综述。

Idiopathic pulmonary fibrosis（IPF）is defined as a specific form of chronic,progressive fibrosing interstitial pneumonia of unknown cause.Several studies have documented that patients with IPF are at high risk for the development of lung cancer.These two diseases share some common pathogenic pathways,which may provide new therapeutic targets in the future.Patients with IPF and lung cancer（IPF-LC）display short survival and poor prognosis because of limited treatment options.The recent approval of two novel drugs（pirfenidone and nintedanib）has shifted the therapeutic dial for the patients with IPF-LC.This review article synthesizes the current knowledge of epidemiology,pathogenesis,clinical features and treatment of IPF-LC.