Matsumoto Aki, Azuma Arata
Fourth Department of Internal Medicine, Nippon Medical School.
Nihon Rinsho. 2006 Jul;64(7):1354-60.
Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disease that prognosis (INF) has not improved by available therapy. Recently, much attention has focused on the utility of the T-helper type 1 cytokine interferon (IFN)-gamma as a treatment for IPF. The rationale for its use has been based on observations of its properties as an inhibitor of fibroblast proliferation, collagen synthesis and deposition, and expression of profibrotic cytokines. The clinical trial is advanced aiming at the prognosis improvement of IPF patients. Small-scale controlled clinical study in 1999, IFN-gamma has improved the respiratory functions of IPF, which was resistant to steroid therapy. Be based on these results, phase III large-scale study mainly in North America, the lagged effect of respiratory function aggravation was expected in IPF, in which pulmonary functions are mildly or moderately deteriolated. But in progressive cases, interferon gamma-1b did not affect progression-free survival, pulmonary function, or the quality of life.
特发性肺纤维化（IPF）是一种进行性致命疾病，现有治疗方法并未改善其预后。最近，1型辅助性T细胞细胞因子干扰素（IFN）-γ作为IPF治疗方法的效用受到了广泛关注。使用它的理论依据基于其作为成纤维细胞增殖、胶原蛋白合成与沉积以及促纤维化细胞因子表达抑制剂的特性观察。针对改善IPF患者预后的临床试验正在推进。1999年的小规模对照临床研究中，IFN-γ改善了对类固醇治疗耐药的IPF患者的呼吸功能。基于这些结果，主要在北美的III期大规模研究预计，在肺功能轻度或中度恶化的IPF患者中会出现呼吸功能加重的滞后效应。但在进展性病例中，干扰素γ-1b并未影响无进展生存期、肺功能或生活质量。
