Karampitsakos Theodoros, Tzilas Vasilios, Tringidou Rodoula, Steiropoulos Paschalis, Aidinis Vasilis, Papiris Spyros A, Bouros Demosthenes, Tzouvelekis Argyris
First Academic Department of Pneumonology, Hospital for Diseases of the Chest, "Sotiria", Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Pathology Department, Hospital for Diseases of the Chest,"Sotiria", Messogion Avenue 152, Athens 11527, Greece.
Pulm Pharmacol Ther. 2017 Aug;45:1-10. doi: 10.1016/j.pupt.2017.03.016. Epub 2017 Apr 1.
Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease of unknown etiology. With a gradually increasing worldwide prevalence and a mortality rate exceeding that of many cancers, IPF diagnosis and management are critically important and require a comprehensive multidisciplinary approach. This approach also involves assessment of comorbid conditions, such as lung cancer, that exerts a dramatic impact on disease survival. Emerging evidence suggests that progressive lung scarring in the context of IPF represents a risk factor for lung carcinogenesis. Both disease entities present with major similarities in terms of pathogenetic pathways, as well as potential causative factors, such as smoking and viral infections. Besides disease pathogenesis, anti-cancer agents, including nintedanib, have been successfully applied in the treatment of patients with IPF while an oncologic approach with a cocktail of several pleiotropic anti-fibrotic agents is currently in the therapeutic pipeline of IPF. Nevertheless, epidemiologic association between IPF and lung cancer does not prove causality. Currently there is significant lack of knowledge supporting a direct association between lung fibrosis and cancer reflecting to disappointing therapeutic algorithms. An optimal therapeutic strategy for patients with both IPF and lung cancer represents an amenable need. This review article synthesizes the current state of knowledge regarding pathogenetic commonalities between IPF and lung cancer and focuses on clinical and therapeutic data that involve both disease entities.
特发性肺纤维化(IPF)是一种病因不明的慢性纤维化肺病。随着全球患病率逐渐上升且死亡率超过许多癌症,IPF的诊断和管理至关重要,需要综合多学科方法。这种方法还包括评估合并症,如肺癌,其对疾病生存率有显著影响。新出现的证据表明,IPF背景下的进行性肺瘢痕形成是肺癌发生的危险因素。这两种疾病实体在发病机制途径以及潜在病因因素(如吸烟和病毒感染)方面存在主要相似之处。除了疾病发病机制外,包括尼达尼布在内的抗癌药物已成功应用于IPF患者的治疗,而一种使用多种多效抗纤维化药物的肿瘤学治疗方法目前正处于IPF的治疗研发中。然而,IPF与肺癌之间的流行病学关联并不能证明因果关系。目前,严重缺乏支持肺纤维化与癌症直接关联的知识,这反映在令人失望的治疗方案中。针对同时患有IPF和肺癌的患者的最佳治疗策略是一种迫切需求。这篇综述文章综合了关于IPF和肺癌发病机制共性的当前知识状态,并重点关注涉及这两种疾病实体的临床和治疗数据。