Kusume K
Department of Medicine and Clinical Immunology, Kyoto University, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Oct;29(10):1254-60.
Idiopathic pulmonary fibrosis (IPF) is thought to develop through slowly progressing lung injury, in which fibrosis occurs as a result of abnormal repair processes. Lung injury in emphysema, in which the normal extracellular matrix is destroyed, is considered to occur mainly because of protease-antiprotease imbalance. In order to examine whether the pathogenesis of IPF involves the proteolytic mechanism of enzymes as in emphysema, concentrations of plasma neutrophil elastase and serum alpha 1-protease inhibitor were measured in patients with IPF, and compared with the levels in patients with emphysema and in normal individuals. In some patients with IPF, the blood concentration of neutrophil elastase was much higher than normal and the degree of imbalance between neutrophil elastase and alpha 1-protease inhibitor was significantly great than in patients with emphysema. In these patients, many years had passed since the onset of the disease, the number of leukocytes and neutrophils and the concentration of LDH in peripheral blood were significantly higher than normal, and the concentration of CEA-II was slightly increased. These data suggest that chronic, massive lung injury had occurred. The blood concentration of neutrophil elastase and alpha 1-protease inhibitor ratio may be useful in assessing the degree of lung injury.
特发性肺纤维化（IPF）被认为是通过缓慢进展的肺损伤发展而来，其中纤维化是异常修复过程的结果。肺气肿中的肺损伤，正常细胞外基质被破坏，被认为主要是由于蛋白酶 - 抗蛋白酶失衡所致。为了研究IPF的发病机制是否像肺气肿那样涉及酶的蛋白水解机制，我们测定了IPF患者血浆中性粒细胞弹性蛋白酶浓度和血清α1 - 蛋白酶抑制剂浓度，并与肺气肿患者和正常个体的水平进行比较。在一些IPF患者中，中性粒细胞弹性蛋白酶的血浓度远高于正常水平，且中性粒细胞弹性蛋白酶与α1 - 蛋白酶抑制剂之间的失衡程度明显大于肺气肿患者。在这些患者中，疾病发作已过去多年，外周血白细胞、中性粒细胞数量及乳酸脱氢酶浓度显著高于正常水平，且癌胚抗原 - II浓度略有升高。这些数据表明发生了慢性、广泛性肺损伤。中性粒细胞弹性蛋白酶血浓度和α1 - 蛋白酶抑制剂比值可能有助于评估肺损伤程度。
