Vourlekis Jason S
Lung and Upper Aerodigestive Cancer Research Group, Division of Cancer Prevention, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
Clin Chest Med. 2004 Dec;25(4):739-47, vii. doi: 10.1016/j.ccm.2004.07.001.
The idiopathic interstitial pneumonias have unknown etiology and are characterized by diffuse parenchymal lung involvement and the potential to develop pulmonary fibrosis. Most portend a reduction in life expectancy due, in part, to the absence of effective therapies. The symptoms of idiopathic interstitial pneumonia develop insidiously. In contradistinction, acute interstitial pneumonia (AIP, also known as acute interstitial pneumonitis) is unique in that it has a very rapid to fulminant onset, leading to early hospitalization and a high initial case fatality ratio but, potentially, a more favorable long-term prognosis for survivors. Despite its contemporary description nearly 20 years ago, knowledge of this disease has increased little. This review focuses on AIP and its current place among the idiopathic interstitial pneumonias.
特发性间质性肺炎病因不明,其特征为弥漫性肺实质受累并有发展为肺纤维化的可能。部分由于缺乏有效治疗方法,多数患者预期寿命缩短。特发性间质性肺炎的症状隐匿出现。相比之下,急性间质性肺炎(AIP,也称为急性间质性肺炎)具有独特性,即起病非常迅速甚至呈暴发性,导致早期住院且初始病死率高,但幸存者的长期预后可能较好。尽管近20年前就有了对该病的现代描述,但对其了解仍几乎没有增加。本综述聚焦于急性间质性肺炎及其在特发性间质性肺炎中的当前地位。