乙酰半胱氨酸治疗特发性肺间质纤维化的进展

Recent progress of management of idiopathic pulmonary fibrosis with N-acetylcysteine

特发性肺间质纤维化是一种以纤维化及肺实质重塑为特征的渐进性疾病，目前尚无特效疗法，标准疗法是美国胸科协会推荐的治疗方案——皮质激素加免疫抑制剂。然而，尚无充分证据证明其有效性，且由于其不良反应大，常常限制了临床应用。近年来，大剂量乙酰半胱氨酸（600mg，3次／d）的使用为特发性肺间质纤维化的治疗带来了新的曙光，其具体机制包括：抗氧化，增加细胞内还原型谷胱甘肽；抗凋亡，保护肺泡上皮；抑制成纤维细胞的增殖及胶原合成等。现就近年的研究进展进行综述。

Idiopathic pulmonary fibrosis（IPF） is a progressive disease characterized with pulmonary fibrosis and parenchyma remodeling. There has been no satisfactory treatment for the disease. The current standard therapy recommended by American Thoracic Society for IPF involves treatment with corticosteroids and other immunosuppressive/cytotoxic agents. However, the beneficial effects of these agents are not well established, and adverse effects are often seen in the long treatment with glucocorticoids. Recently, the use of high-dose acetylcysteine （600 mg, 3 times per day） had shed high light on the treatment of IPF for its successfully preventing the decrease of vital capacity and diffusing capacity of the lung for carbon monoxide （DLco）. The mechanisms maybe contained anti-oxidation and increased the GSH in the cell,anti-apoptosis and protected alveolar epithelium, inhibited proliferation and collagen synthesis of fibroblast. The recent progress of these mechanisms will be reviewed in this article.