摘要特发性肺纤维化( IPF)是一种原因未明的纤维化型的间质性肺炎,患者中位生存期只有2~3年,因此,及时的诊断和治疗尤为重要。目前Ⅱ型肺泡细胞表面抗原( KL-6)、肺表面活性蛋白A( SP-A)和肺表面活性蛋白D( SP-D)在国外被应用于临床,有较好的敏感度,并能预示疾病的进展和预后,但是在诊断的特异性上存在不足。尚有一些处于研究早期的潜在生物学标志物,以期研究能发现高度特异性和敏感度的生物学标志物。本文主要从与IPF的病理生理机制有关的肺泡内皮细胞功能紊乱、肺纤维化和免疫系统功能紊乱3个方面阐释近年来生物学标志物的研究。(中华检验医学杂志,2016,39:68-70)
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abstractsIdiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown etiology , a median survival time of which is 2 to 3 years.The diagnosis and treatment are important for IPF in time.Krebs von den lungen-6(KL-6), Surfactant protein-A(SP-A) and Surfactant protein-D(SP-D) are acceptable biomarkers in clinical for idiopathic pulmonary fibrosis in Japan,which have shown good sensitivity at diagnosis IPF and predict the prognoses for patients with IPF . However , the differential diagnosis of IPF from other interstitial lung diseases is still challenging .Other biomarkers are being developed , one of which would have the best specificity and sensitivity at diagnosis IPF.Those biomarkers about pathogenesis of IPF includes alveolar epithelial cell dysfunction , fibrogenesis and immune dysregulation are shown .They are potential to account for underlying disease mechanisms , accelerated drug development and advance clinical management.
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