Interstitial lung diseases (ILDs) encompass a complex group of hundreds of lung disorders that affect lung tissue with variable morphologies and clinical presentations. The most extensively studied type of ILD is idiopathic pulmonary fibrosis (IPF), which is characterised by progressive pulmonary fibrosis, a decline in lung function, and high mortality with a histological pattern of usual interstitial pneumonia (UIP). A proportion of patients with other types of ILD also develop a progressive fibrosing phenotype, including idiopathic nonspecific interstitial pneumonia (NSIP), as well as restrictive allograft syndrome (RAS) and idiopathic pleuroparenchymal fibroelastosis (iPPFE) with a histological pattern of alveolar fibroelastosis (AFE). RAS is a novel form of chronic lung allograft dysfunction first described in 2011 [1]. Focusing on remodelling-associated angiogenesis, both sprouting and intussusceptive, Ackermann and co-workers present histopathology, microvascular anatomy and gene expression in three main subtypes of interstitial lung disease: UIP, NSIP and AFE http://bit.ly/2NtmV6D

中文翻译：

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揭开间质性肺疾病中脉管系统的形态分子改变的神秘面纱

间质性肺病 (ILD) 包括数百种肺部疾病，这些疾病影响具有不同形态和临床表现的肺组织。研究最广泛的 ILD 类型是特发性肺纤维化 (IPF)，其特征是进行性肺纤维化、肺功能下降和高死亡率，组织学模式为普通间质性肺炎 (UIP)。一部分患有其他类型 ILD 的患者也会出现进行性纤维化表型，包括特发性非特异性间质性肺炎 (NSIP)，以及限制性同种异体移植综合征 (RAS) 和特发性胸膜实质弹性纤维变性 (iPPFE)，其组织学模式为肺泡弹性纤维变性 (AFE) ）。RAS 是一种新型的慢性肺移植物功能障碍，于 2011 年首次描述 [1]。