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摘要: 特发性间质性肺炎(idiopathic interstitial pneumonia, ⅡP)是一组原因不明的弥漫性实质性肺疾病, 主要累及肺间质。随着对ⅡP研究的不断深入, ⅡP相关分类和治疗方案也经历了不断演变的过程。本文系统回顾ⅡP分类变迁、特发性肺纤维化(idiopathic pulmonary fibrosis, IPF)急性加重概念演变以及不同时期IPF治疗方案出台的背景和最新进展, 并对今后ⅡP的分类、诊断和治疗前景进行展望。
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关键词:
- 特发性间质性肺炎 /
- 分类 /
- 特发性肺纤维化, 急性加重 /
- 治疗
Abstract: Idiopathic interstitial pneumonias (ⅡPs) are a group of heterogeneous diffuse parenchymal lung diseases of unknown etiology, which mainly involve the interstitium of the lung. With the deepening of research, the classification and treatment of ⅡPs have gone through a process of continuous evolution。This article reviewed the changes in the classification of ⅡPs, the evolution of the concept and definition of acute exacerbation of idiopathic pulmonary fibrosis, and the background and latest progress in the treatment plan at different periods of IPF.We also prospected the classification, diagnosis, and treatment of ⅡPs in the future. -
表 1 2002年美国胸科学会/欧洲呼吸学会特发性间质性肺炎病理分型及对应临床诊断
病理类型 对应临床诊断名称 寻常型(普通型间质性肺炎) 特发性肺纤维化 非特异性间质性肺炎 非特异性间质性肺炎 机化性肺炎 隐源性机化性肺炎 弥漫性肺泡损伤 急性间质性肺炎 呼吸性细支气管炎 呼吸性细支气管炎间质性肺病 脱屑性间质性肺炎 脱屑性间质性肺炎 淋巴细胞性间质性肺炎 淋巴细胞性间质性肺炎 
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表 2 2013年美国胸科学会/欧洲呼吸学会特发性间质性肺炎病理分型
病理类型 主要的特发性间质性肺炎 特发性肺纤维化 非特异性间质性肺炎 隐源性机化性肺炎 急性间质性肺炎 呼吸性细支气管炎间质性肺病 脱屑性间质性肺炎 罕见的特发性间质性肺炎 特发性胸膜肺弹力纤维增生症 特发性淋巴性间质性肺炎 不能分类的特发性间质性肺炎
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表 3 2013年美国胸科学会/欧洲呼吸学会特发性间质性肺炎临床分类国际多学科共识
临床行为 治疗目的 监测策略 可逆性或自限性疾病(如呼吸性细支气管炎间质性肺病) 去除可能的原因 短期(3~6个月)观察, 以判断疾病进展 伴有进展危险因素的可逆性疾病(如非特异性间质性肺炎、脱屑性间质性肺炎、隐源性机化性肺炎) 短期观察证实治疗有效, 长期合理治疗 长期观察保证治疗效果稳定 伴有部分残留的稳定病变(如某些纤维化型非特异性间质性肺炎) 维持目前状态 长期观察评估疾病进程 具有潜在稳定, 但可能进展的不可逆疾病(如某些纤维化型非特异性间质性肺炎) 预防进展 长期观察评估疾病进程 即使积极治疗, 仍呈不可逆进行性进展的疾病(如特发性肺纤维化、某些纤维化型非特异性间质性肺炎) 延缓疾病进展 长期观察评估疾病进程, 判断是否需要肺移植或有效的辅助治疗方法
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