摘要
纤维化性肺泡炎合并系统性硬化(FASSc)比单纯隐源性纤维化肺泡炎(CFA)具有更好的预后。我们想要确定这种改善的预后是否与下呼吸道中不同的中性粒细胞迁移和激活有关。因此,我们比较了FASSc、CFA和正常个体的支气管肺泡灌洗(BAL)中性粒细胞数量和中性粒细胞源性酶水平。对45例患者进行支气管肺泡灌洗(FASSc n = 20;CFA n = 15;法线n = 10);测定细胞计数和中性粒细胞源性酶、髓过氧化物酶、弹性酶(总弹性酶和弹性酶/ α - 1抗胰蛋白酶复合物)、胶原酶和乳铁蛋白水平。对纤维性肺泡炎患者进行肺功能测试。三组BAL液中胶原酶、髓过氧化物酶和弹性酶/ α - 1-抗胰蛋白酶复合物水平存在显著差异。CFA患者的中性粒细胞百分比、胶原酶和髓过氧化物酶水平明显高于FASSc患者。 Disease extent, as judged by lung volumes and gas transfer, was comparable in the CFA and FASSc groups. Forced vital capacity (% predicted) was significantly lower in patients with evidence of increased neutrophil enzyme release than those without. We conclude that: 1) increased neutrophil migration to the lung is accompanied by release both of primary and secondary granule enzymes in cryptogenic fibrosing alveolitis; and 2) the lower amounts of neutrophil products in fibrosing alveolitis complicating systemic sclerosis may account for the improved prognosis, even when disease is as extensive as in cryptogenic fibrosing alveolitis.