Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis
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DOI：
10.1111/j.1398-9995.2005.00719.x
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发表时间：
2005-04
期刊：
影响因子：
12.4
通讯作者：
Sergio Harari;A. Caminati
中科院分区：
文献类型：
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作者：
Sergio Harari;A. Caminati
特发性肺纤维化（IPF）是最常见的病因不明的间质性肺炎，也是最具侵袭性的间质性肺疾病。 IPF 通过外科肺活检发现普通间质性肺炎 (UIP) 来确诊 (1-5)。特发性间质性肺炎 (IIP) 是一组弥漫性实质肺疾病 (DPLD)，也称为间质性肺疾病。 IIP 是一组异质性非肿瘤性疾病，由不同类型的炎症和纤维化对肺实质造成损害所致 (1)。 IIP包括IPF、非特异性间质性肺炎(NSIP)、隐源性机化性肺炎(COP)、急性间质性肺炎(AIP)、呼吸性细支气管炎相关间质性肺疾病(RB-ILD)、脱屑性间质性肺炎(DIP)和淋巴细胞性间质性肺炎(LIP)。需要强调的是，IPF 是一种进行性且不可逆转的疾病，迄今为止，还没有任何药物能够改变 IPF 的进行性自然病程及其通常的最终结果 (1,2,6)。其特点是放射学上明显的间质浸润主要影响肺基底，并伴有进行性呼吸困难和肺功能恶化，病理上表现为成纤维细胞增殖和细胞外基质积聚，导致肺结构不可逆的扭曲 (1)。目前 IPF 的严格定义为基础和临床研究提供了新的焦点，这将提高对该疾病发病机制的了解并刺激新疗法的开发 (7)。定义
Idiopathic pulmonary fibrosis (IPF) is the most common of the interstitial pneumonias of unknown etiology and the most aggressive interstitial lung disease. IPF is confirmed by the identification of usual interstitial pneumonia (UIP) on surgical lung biopsy (1–5). The idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases (DPLDs) also described as interstitial lung diseases. The IIPs are a heterogeneous group of nonneoplastic disorders resulting from damage to the lung parenchyma by varying patterns of inflammation and fibrosis (1). The IIPs include the entities of IPF, nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), and lymphocytic interstitial pneumonia (LIP). It is important to emphasize that IPF is a progressive and irreversible illness and, until now, there has been no available drug that has been able to modify the progressive natural course of IPF and its usual terminal outcome (1, 2, 6). It is characterized by radiographically evident interstitial infiltrates predominantly affecting the lung bases and by progressive dyspnea and worsening of pulmonary function, pathologically by fibroblast proliferation and extracellular matrix accumulation resulting in irreversible distortion of the architecture of the lung (1). The current strict definition of IPF provides a new focus for basic and clinical research that will improve insight into the pathogenesis of this disorder and stimulate the development of novel therapies (7). Definition