PT-杂志文章Au -Cailes，JB Au -O'Connor，C Au -Pantelidis，P au -Southcott，Am au -Au -Fitzgerald，MX Au -Black，CM Au -du Bois，RM Ti- ti -rm ti -rm ti -rm ti -ti -ti -rm粒细胞激活肺炎肺炎肺炎：孤独的隐晶性纤维化肺泡炎和系统性硬化症DP的比较 - 1996年5月01日-TA-欧洲呼吸杂志PG -992--999​​ VI -9 IP -5 4099 -http：//www.qdcxjkg.com/content.com/content/9/5//9/5/992. SHORT 4100 -http：//www.qdcxjkg.com/content/9/5/992.full so -eur Respir J1996 5月1日；9 AB-纤维肺炎复杂性的全身性硬化症（FASSC）的预后比孤独的隐秘纤维化肺泡炎（CFA）更好。我们想确定这种改善的预后是否与下呼吸道中的嗜中性粒细胞迁移和激活有关。因此，我们比较了FASSC，CFA和正常个体中嗜中性粒细胞衍生酶的中性粒细胞数量和中性粒细胞的水平。对45名受试者（FASSC n = 20; CFA n = 15;正常n = 10）进行支气管肺泡灌洗。测量了中性粒细胞衍生酶，髓过氧化物酶，弹性酶（总弹性酶和弹性酶/α1抗胰蛋白酶复合物），胶原酶和乳铁蛋白的水平。在患有纤维肺泡炎的受试者中进行肺功能测试。来自三组的BAL液中存在胶原酶，髓过氧化物酶/α1-抗抑制蛋白蛋白酶复合物的胶原酶水平显着差异。CFA患者的中性粒细胞百分比和胶原酶和髓过氧化物酶的水平明显高于FASSC。 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.