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Abstract：
Idiopathic interstitial pneumonias (IIP) are a heterogeneous group characterized by unknown aetiology. Establishment of a correct diagnosis of a distinct IIP requires a multidisciplinary approach integrating clinical presentation, physiological data, radiological appearance and histological findings. The 2013 update of the American Thoracic Society/European Respiratory Society classification summarises progress in the field of IIP and outlines potential areas for future research. The main entities defined by the 2002 statement on IIP are preserved, but major IIP are now distinguished from rare IIP and the new category of unclassifiable IIP is introduced. In addition, the existence of idiopathic non-specific interstitial pneumonia as a separate chronic fibrosing IIP and idiopathic pleuroparenchymal fibroelastosis as a specific rare entity are acknowledged. Moreover, the major IIP are categorized according to the features chronic fibrosing, smoking-related and acute/subacute clinical course. Furthermore, a clinical classification of IIP according to disease behaviour with suggestions for treatment goals and monitoring strategies is provided. The goal of this review is to discuss the areas of uncertainty in the updated multidisciplinary classification of IIP and point out potential consequences for clinical management.
摘要：
特发性间质性肺炎（IIP）是一个病因不明的异质性群体。建立不同的IIP的正确诊断需要整合临床表现的多学科方法，生理数据,放射学外观和组织学发现。2013年美国胸科学会/欧洲呼吸学会分类更新总结了IIP领域的进展，并概述了未来研究的潜在领域。2002年关于IIP的声明所定义的主要实体被保留，但是主要的IIP现在与稀有的IIP区分开，并引入了不可分类的IIP的新类别。此外,特发性非特异性间质性肺炎作为一种独立的慢性纤维化IIP和特发性胸膜实质纤维弹性增生症作为一种特殊的罕见实体的存在是公认的.此外，主要的IIP根据慢性纤维化的特征进行分类，吸烟相关和急性/亚急性临床病程。此外，根据疾病行为对IIP进行临床分类,并提供治疗目标和监测策略建议.这篇综述的目的是讨论IIP更新的多学科分类中的不确定性领域，并指出临床管理的潜在后果。
