Acute Exacerbation in Interstitial Lung Disease.

Acute Exacerbation in Interstitial Lung Disease.
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DOI:
10.3389/fmed.2017.00176
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发表时间:
2017
影响因子:
3.9
通讯作者:
Behr J
Behr J
中科院分区:
医学3区
文献类型:
--
作者:
Leuschner G;Behr J

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特发性肺纤维化急性加重(AE-IPF)定义为在不到1个月内发生的急性、临床显著恶化,无明显临床原因,如液体超负荷、左心衰竭或肺栓塞。在病理生理学上,肺泡损伤是AE-IPF的主要特征,在组织病理学上表现为弥漫性肺泡损伤,在放射学上表现为高分辨率计算机断层扫描上的弥漫性双侧毛玻璃样混浊。越来越多的文献关注特发性肺纤维化以外的间质性肺病急性加重(AE-ILD)。基于共同的病理生理学,普遍认为AE-ILD可影响所有间质性肺疾病(ILD)患者,但在具有基础常见间质性肺炎模式的患者中发生率明显更高。AE-ILD的病因尚不完全清楚,但有不同的风险因素和触发因素,如感染、机械应力和微抽吸。一般而言,AE-ILD的预后较差,并且与6-12个月内的高死亡率相关。尽管缺乏循证数据,但在临床实践中,AE-ILD通常采用高剂量皮质类固醇治疗和抗生素治疗。本文旨在总结AE-ILD的临床特征、诊断、管理和预后,并更新该领域的最新进展。
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has been defined as an acute, clinically significant deterioration that develops within less than 1 month without obvious clinical cause like fluid overload, left heart failure, or pulmonary embolism. Pathophysiologically, damage of the alveoli is the predominant feature of AE-IPF which manifests histopathologically as diffuse alveolar damage and radiologically as diffuse, bilateral ground-glass opacification on high-resolution computed tomography. A growing body of literature now focuses on acute exacerbations of interstitial lung disease (AE-ILD) other than idiopathic pulmonary fibrosis. Based on a shared pathophysiology it is generally accepted that AE-ILD can affect all patients with interstitial lung disease (ILD) but apparently occurs more frequently in patients with an underlying usual interstitial pneumonia pattern. The etiology of AE-ILD is not fully understood, but there are distinct risk factors and triggers like infection, mechanical stress, and microaspiration. In general, AE-ILD has a poor prognosis and is associated with a high mortality within 6–12 months. Although there is a lack of evidence based data, in clinical practice, AE-ILD is often treated with a high dose corticosteroid therapy and antibiotics. This article aims to provide a summary of the clinical features, diagnosis, management, and prognosis of AE-ILD as well as an update on the current developments in the field.
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