Hamada H, Sakatani M, Nishioka M, Akira M, Yamamoto S, Ueda E
Department of Internal Medicine, National Kinki-Chuo Hospital for Chest Diseases, Osaka, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1997 Nov;35(11):1238-44.
A 63-year-old female with acure exacerbution of idiopathic interstitial pneumonia (IIP) showed high levels of elastolytic activity in bronchoalveolar lavage fluid (BALF). She was admitted to our hospital with progressive dyspnea. Arterial blood gas analysis and pulmonary function tests were abnormal. Examination of the bronchoalveolar lavage fluid revealed an increased numbers of neutrophils. Despite the administration of corticosteroids, the patient died of respiratory failure. A high level of elastolytic activity was present in the BALF. Western immunoblot analysis, using an anti-alpha 1-protease inhibitor (PI) antibody, revealed a truncated alpha 1-PI in the BALF. These findings suggest that an imbalance between protease and PI in the lower respiratory tract contribute to lung tissue damage in patients with IIP.
一名63岁患有特发性间质性肺炎（IIP）急性加重期的女性，其支气管肺泡灌洗液（BALF）中显示出高水平的弹性蛋白酶活性。她因进行性呼吸困难入住我院。动脉血气分析和肺功能测试均异常。支气管肺泡灌洗液体检查显示中性粒细胞数量增加。尽管给予了皮质类固醇治疗，患者仍死于呼吸衰竭。BALF中存在高水平的弹性蛋白酶活性。使用抗α1-蛋白酶抑制剂（PI）抗体进行的蛋白质免疫印迹分析显示，BALF中存在截短的α1-PI。这些发现表明，下呼吸道中蛋白酶和PI之间的失衡导致了IIP患者的肺组织损伤。
