Abstract
Idiopathic 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.(Chin J Lab Med, 2016, 39: 68-70) Key words: Idiopathic pulmonary fibrosis; Biological markers; Pulmonary alveoli
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