Protein C anticoagulant system in patients with interstitial lung disease
Protein C anticoagulant system in patients with interstitial lung disease
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DOI：
10.1164/ajrccm.157.6.9709078
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发表时间：
1998-06-01
影响因子：
24.7
通讯作者：
Adachi, Y
中科院分区：
文献类型：
--
作者：
Kobayashi, H;Gabazza, EC;Adachi, Y
肺泡腔内的促凝血活性过高可能在肺纤维化的发病机制中起相关作用。高凝状态是由于促凝血因子和抗凝因子之间的平衡被破坏而引起的。本研究的目的是评估11例特发性肺纤维化（IPF）、14例结节病和16例胶原血管病（CVD）相关间质性肺病（CVD-ILD）患者支气管肺泡灌洗液（BALF）和血浆中抗凝蛋白C（PC）通路的分子标志物水平。6名健康非吸烟志愿者作为对照组。结节病和CVD-ILD患者的凝血激活标志物凝血酶-抗凝血酶III复合物（达特）的PALE浓度显著高于对照受试者。IPF（610 +/- 150 ng/ml）、结节病（680 +/-170 ng/ml）和CVD-ILD（1，580 +/- 600 ng/ml）患者PALE中的PC水平显著高于对照受试者（230 +/- 140 ng/ml）。与对照受试者（1.08 +/- 0.23 ng/ml）相比，IPF（0.46 +/- 0.16 ng/ml）、结节病（0.43 +/- 0.11 ng/ml）和CVD-ILD（0.50 +/- 0.15 ng/ml）患者的活化PC-PC抑制剂（APC-PCI）复合物的PALE浓度显著降低。IPF（2.70 +/- 1.74 ng/mug）、结节病（1.94 +/- 0.82 ng/mug）和CVD-ILD（1.89 +/- 0.68 ng/mug）患者的APC-PCI/PC比值显著低于对照受试者（15.91 +/- 8.45 ng/mug）。与对照受试者相比，CVD-ILD患者的APC-PCI血浆水平和APC-PCI/PC比值也显著降低。总体而言，这些结果表明，在ILD患者中PC活化降低伴促凝血活性增加。
Excessive procoagulant activity in the alveolar space may play a relevant role in the pathogenesis of pulmonary fibrosis. Hypercoagulability results from the disruption of the balance between the procoagulant and anticoagulant factors. The aim of this study was to assess the levels of molecular markers of the anticoagulant protein C (PC) pathway in the bronchoalveolar lavage fluid (BALF) and plasma of 11 patients with idiopathic pulmonary fibrosis (IPF), 14 with sarcoidosis and 16 with collagen vascular disease (CVD)-associated interstitial lung disease (CVD-ILD). Six healthy nonsmoking volunteers served as control subjects. PALE concentrations of the marker of clotting activation, thrombin-antithrombin III complex (TAT), in patients with sarcoidosis and CVD-ILD were significantly greater than those in control subjects. PC levels in PALE were markedly higher in patients with IPF (610 +/- 150 ng/ml), sarcoidosis (680 +/- 170 ng/ml), and CVD-ILD (1,580 +/- 600 ng/ml) than in control subjects (230 +/- 140 ng/ml). PALE concentrations of activated PC-PC inhibitor (APC-PCI) complex were significantly decreased in IPF (0.46 +/- 0.16 ng/ml), sarcoidosis (0.43 +/- 0.11 ng/ml), and CVD-ILD (0.50 +/- 0.15 ng/ml) patients as compared with control subjects (1.08 +/- 0.23 ng/ml). APC-PCI/PC ratios were significantly lower in patients with IPF (2.70 +/- 1.74 ng/mu g), sarcoidosis (1.94 +/- 0.82 ng/mu g), and CVD-ILD (1.89 +/- 0.68 ng/mu g) than in control subjects (15.91 +/- 8.45 ng/mu g). Plasma levels of APC-PCI and the APC-PCI/PC ratio were also significantly decreased in patients with CVD-ILD as compared with control subjects. Overall, these findings suggest that decreased PC activation with increased procoagulant activity occurs in patients with ILD.