Power C, O'Connor C M, MacFarlane D, O'Mahoney S, Gaffney K, Hayes J, FitzGerald M X
Department of Medicine, University College Dublin, Ireland.
Am J Respir Crit Care Med. 1994 Sep;150(3):818-22. doi: 10.1164/ajrccm.150.3.8087357.
The potential role of neutrophil elastase in causing lung damage and exacerbating the inflammatory response in cystic fibrosis (CF) has received considerable attention. Although another potent neutrophil-derived enzyme, collagenase, is implicated in tissue destruction in several interstitial lung disorders, there has been no reference to this enzyme in CF. The objective of this study was to determine whether neutrophil collagenase is present in active form in CF sputum and, if so, whether it is related to disease severity. High levels of active collagenase were detected in sputum from patients with CF, and the majority of the enzyme present was of neutrophil origin. In a group of 16 patients with CF, negative relations between sputum collagenase activity and Shwachman score (r = -0.55, p < 0.05) and FEV1 (r = -0.59, p < 0.02) were noted, indicating an association between high collagenase activity and severity of disease. A positive correlation was observed between sputum collagenase and elastase activity (r = 0.62, p < 0.05). These results suggest that both neutrophil elastase and collagenase may play a significant role in lung destruction in CF.
中性粒细胞弹性蛋白酶在导致囊性纤维化（CF）患者肺损伤及加剧炎症反应方面的潜在作用已受到广泛关注。尽管另一种由中性粒细胞产生的强效酶——胶原酶，在几种间质性肺病的组织破坏中起作用，但在CF中尚未提及该酶。本研究的目的是确定CF痰液中是否存在活性形式的中性粒细胞胶原酶，如果存在，它是否与疾病严重程度相关。在CF患者的痰液中检测到高水平的活性胶原酶，且所检测到的大部分酶来源于中性粒细胞。在一组16例CF患者中，观察到痰液胶原酶活性与Shwachman评分（r = -0.55，p < 0.05）及第一秒用力呼气容积（FEV1）（r = -0.59，p < 0.02）呈负相关，表明胶原酶活性高与疾病严重程度相关。痰液胶原酶与弹性蛋白酶活性之间存在正相关（r = 0.62，p < 0.05）。这些结果表明，中性粒细胞弹性蛋白酶和胶原酶可能在CF的肺破坏中起重要作用。
