Mordelet-Dambrine M, Lafuma C, Stanislas-Leguern G, Robert L, Chrétien J, Hornebeck W
INSERM U 214, Hôpital Laennec, Paris, France.
Eur Respir J. 1988 Aug;1(8):748-57.
Elastase activities were investigated in bronchoalveolar lavage (BAL) cells from healthy controls (C, n = 8) and patients with untreated mediastinopulmonary sarcoidosis at different stages: I (n = 5), II (n = 7), IIIA (n = 3), IIIB (n = 4). Elastase activities (EA and ELA) were measured against a synthetic substrate, succinyl-trialanine-paranitroanilide (SLAPN) and radiolabelled ligamentum nuchae 3H insoluble elastin (specific radioactivity 3.7 MBq.mg-1). A significant increase of ELA of BAL cells was observed in patients (S III), in particular those exhibiting fibrotic roentgenographic patterns and a higher number of polymorphonuclear cells. On the contrary, EA of BAL cells showed no variation between different stages of sarcoidosis. ELA was never detected in the macrophage culture medium and appeared to be mainly associated with non-adherent macrophages, whereas EA appeared to be preferentially associated with adherent macrophages. The inhibitory profiles of ELA and EA were investigated using general proteinase inhibitors and also compounds interacting specifically with leucocyte elastase. ELA was mainly due to a serine protease, which has a sensitivity to inhibitors similar to leucocyte elastase and is increased in advanced stages of sarcoidosis. EA appears to be only slightly modified, if at all, in sarcoidosis and is probably related to the action of both a serine protease and a metalloenzyme. Thus, only ELA yields diagnostic information concerning stage III of sarcoidosis associated with a fibrotic or bullous roentgenographic pattern.
对健康对照者（C组，n = 8）以及处于不同阶段的未经治疗的纵隔肺结节病患者（I期，n = 5；II期，n = 7；IIIA期，n = 3；IIIB期，n = 4）的支气管肺泡灌洗（BAL）细胞中的弹性蛋白酶活性进行了研究。针对合成底物琥珀酰 - 丙氨酰 - 对硝基苯胺（SLAPN）和放射性标记的项韧带³H不溶性弹性蛋白（比放射性为3.7 MBq.mg⁻¹）测定弹性蛋白酶活性（EA和ELA）。在患者（III期）中观察到BAL细胞的ELA显著增加，尤其是那些呈现纤维化影像学模式且多形核细胞数量较多的患者。相反，BAL细胞的EA在结节病的不同阶段之间未显示出变化。在巨噬细胞培养基中从未检测到ELA，且似乎主要与非贴壁巨噬细胞相关，而EA似乎优先与贴壁巨噬细胞相关。使用一般蛋白酶抑制剂以及与白细胞弹性蛋白酶特异性相互作用的化合物研究了ELA和EA的抑制谱。ELA主要归因于一种丝氨酸蛋白酶，其对抑制剂的敏感性与白细胞弹性蛋白酶相似，且在结节病晚期增加。EA在结节病中似乎即使有变化也非常轻微，可能与丝氨酸蛋白酶和金属酶的作用都有关。因此，只有ELA能提供与纤维化或大疱性影像学模式相关的结节病III期的诊断信息。
