Antoniu Sabina A
Clinic of Pulmonary Disease, 30 Dr I Cihac Street, 700115, Iasi, Romania.
Expert Opin Investig Drugs. 2006 Jul;15(7):823-8. doi: 10.1517/13543784.15.7.823.
Idiopathic pulmonary fibrosis (IPF) is an under-recognised, rare, progressive disease of the lungs with unknown aetiology and high mortality. The currently advocated pathogenic mechanism is represented by progressive multifocal fibrosis. It is diagnosed based on clinical, radiographic, physiological and histopathological criteria. Existing therapeutic guidelines recommend anti-inflammatory and immunosuppressive combinations, despite proven limited efficacy. There is no therapy approved specifically for IPF, but several antifibrotic agents are currently under development for this indication. Pirfenidone is an antifibrotic agent potentially effective for IPF therapy, and preclinical and available clinical data support its use in IPF. Future clinical studies are expected to provide more consistent information on survival benefit, lung function and health-related quality of life.
特发性肺纤维化（IPF）是一种认识不足、罕见的进行性肺部疾病，病因不明，死亡率高。目前所倡导的致病机制以进行性多灶性纤维化为代表。它是根据临床、影像学、生理学和组织病理学标准进行诊断的。尽管已证实现有治疗方法疗效有限，但现行治疗指南仍推荐使用抗炎和免疫抑制联合疗法。目前尚无专门批准用于IPF的治疗方法，但目前有几种抗纤维化药物正在针对这一适应症进行研发。吡非尼酮是一种可能对IPF治疗有效的抗纤维化药物，临床前和现有临床数据均支持其在IPF中的应用。未来的临床研究有望提供关于生存获益、肺功能和健康相关生活质量的更一致信息。
