Scharfman A, Hayem A, Davril M, Marko D, Hannothiaux M H, Lafitte J J
Unité INSERM No. 16, Lille, France.
Eur Respir J. 1989 Sep;2(8):751-7.
Pneumoconiosis is defined as the disease resulting from a chronic exposure to different inorganic dusts. In order to assess the lung defence against the effects of dust exposure, we studied the bronchoalveolar lavage (BAL) fluids from 30 silicotic patients (9 of them having a diagnosis of progressive massive fibrosis (PMF)) and 8 subjects with a diagnosis of asbestosis. Total protein content, N-acetyl-beta-D-glucosaminidase activity, free elastase-like activity, immunoreactive alpha 1-proteinase inhibitor (alpha 1PI) and neutrophil elastase inhibitory capacity (NEIC) were determined, and the values obtained were compared to those of 14 control BAL fluids. In all of the patients, our data showed a significant increase of total protein content and free elastase-like activity. In contrast, N-acetyl-beta-D-glucosaminidase activities did not reach statistical significance. Values concerning immunoreactive alpha 1PI and NEIC were significantly raised only in patients with PMF and with asbestosis. When the ratio NEIC/immunoreactive alpha 1PI was calculated, a significant difference was noticed in the asbestosis group; on the other hand, this ratio was significantly reduced in the group of PMF patients. After neutrophil elastase addition, an electrophoretic study by SDS-PAGE and immunoblotting was carried out; it showed more proteolysed alpha 1PI in the BAL fluids having a lowered NEIC/alpha 1PI ratio. These facts could be explained by the presence of inhibitors of neutrophil elastase different from alpha 1PI.
尘肺病被定义为长期接触不同无机粉尘所导致的疾病。为了评估肺部对粉尘暴露影响的防御能力，我们研究了30例矽肺患者（其中9例被诊断为进行性大块纤维化（PMF））和8例被诊断为石棉肺患者的支气管肺泡灌洗（BAL）液。测定了总蛋白含量、N - 乙酰 - β - D - 氨基葡萄糖苷酶活性、游离弹性蛋白酶样活性、免疫反应性α1 - 蛋白酶抑制剂（α1PI）和中性粒细胞弹性蛋白酶抑制能力（NEIC），并将所得值与14份对照BAL液的值进行比较。在所有患者中，我们的数据显示总蛋白含量和游离弹性蛋白酶样活性显著增加。相比之下，N - 乙酰 - β - D - 氨基葡萄糖苷酶活性未达到统计学显著性。仅在患有PMF和石棉肺的患者中，免疫反应性α1PI和NEIC的值显著升高。计算NEIC/免疫反应性α1PI比值时，在石棉肺组中发现有显著差异；另一方面，该比值在PMF患者组中显著降低。添加中性粒细胞弹性蛋白酶后，通过SDS - PAGE和免疫印迹进行电泳研究；结果显示，在NEIC/α1PI比值降低的BAL液中，α1PI的蛋白水解程度更高。这些事实可以通过存在不同于α1PI的中性粒细胞弹性蛋白酶抑制剂来解释。
