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特发性肺纤维化急性加重的新认识

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Author:: No author available

Journal Title:: Chinese Journal of Tuberculosis and Respiratory Diseases

Issue:: 5

DOI:: 10.3760/cma.j.issn.1001-0939.2017.05.011

Key Word:: No keyword available

Abstract：特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)是一种病因不清,局限在肺脏的慢性、进行性、纤维化性间质性肺疾病,其组织学和(或)胸部高分辨率CT(high-resolution computed tomography,HRCT)特征为普通型间质性肺炎(usual interstitial pneumonia,UIP) [1-2].IPF临床过程表现为多样性,其中相当比例的患者可在相对稳定期间突然出现不可预测的急性加重,导致患者的急性呼吸衰竭,甚至死亡[3-4].2007年美国心肺与血液研究所(the National Heart,Lung,and Blood Institute,NHLBI)发起的IPF临床研究网(the Idiopathic Pulmonary Fibrosis Clinical Research Network,IPFnet)提出了IPF急性加重的定义与诊断标准[5].此后,该定义与诊断标准广泛应用于临床实践与试验,加深了人们对于IPF急性加重的认识,推动了IPF急性加重临床和治疗的研究.但是,多年来的临床实践与研究也发现,IPF急性加重的定义和诊断标准等在基础和临床相关研究中存在着一些问题[6].2016年由Collard等[7]众多专家们组成的国际专题工作组,再次对近10年来发表的IPF急性加重相关文献进行了复习,依据现有的研究证据,对IPF急性加重定义及诊断标准等相关问题提出了重要的修改建议.

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