Idiopathic pulmonary fibrosis (IPF) is a chronic debilitating fibrotic lung disease leading to respiratory failure and ultimately to death. Noninvasive biomarkers, for the early diagnosis, differential diagnosis, prognosis, and prediction of therapeutic response, are needed. Previous studies support a role for periostin in lung fibrosis. The aim of our study was to analyze periostin levels in the airways of patients with IPF and to investigate its role as a useful predictive biomarker of the disease. We enrolled 30 IPF patients and 5 control subjects. All subjects underwent all standard radiological, functional, and biological examinations for IPF diagnosis and staging and exhaled breath condensate (EBC) collection. Periostin was assessed by an enzyme-linked immunosorbent assay kit on EBC. Periostin was dosable in the EBC of all subjects enrolled. We found higher exhaled periostin levels in IPF patients than healthy controls (65.5 ± 23.5 pg/mL vs. 33 ± 21.4 pg/mL, p < 0.05). Moreover, in receiver operating characteristic analysis, the clinical reference value of periostin was 37.88 pg/mL to discriminate patients with IPF from healthy subjects, with the area under the curve of 0.8815. There was no significant correlation between periostin levels and gender or pulmonary function tests. These preliminary results support our working hypothesis that periostin is dosable in the airways of patients with IPF. As the circulating periostin, also airways periostin may be a potential biomarker to support IPF diagnosis and to monitor disease progression during follow-up.

中文翻译：

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气道骨膜素在特发性肺纤维化患者临床实践中的潜在作用

特发性肺纤维化 (IPF) 是一种慢性使人衰弱的纤维化肺病，可导致呼吸衰竭并最终导致死亡。需要用于早期诊断、鉴别诊断、预后和治疗反应预测的无创生物标志物。以前的研究支持骨膜蛋白在肺纤维化中的作用。我们研究的目的是分析 IPF 患者气道中骨膜蛋白的水平，并研究其作为一种有用的疾病预测生物标志物的作用。我们招募了 30 名 IPF 患者和 5 名对照受试者。所有受试者都接受了所有标准的放射学、功能和生物学检查，以进行 IPF 诊断和分期以及呼出气冷凝物 (EBC) 收集。骨膜蛋白通过酶联免疫吸附测定试剂盒在 EBC 上进行评估。Periostin 在所有入组受试者的 EBC 中均可给药。p  < 0.05)。此外，在受试者操作特征分析中，periostin的临床参考值为37.88 pg/mL，用于区分IPF患者与健康受试者，曲线下面积为0.8815。骨膜蛋白水平与性别或肺功能测试之间没有显着相关性。这些初步结果支持我们的工作假设，即骨膜素可在 IPF 患者的气道中给药。作为循环骨膜蛋白，气道骨膜蛋白也可能是支持 IPF 诊断和监测随访期间疾病进展的潜在生物标志物。