Pirfenidone treatment of idiopathic pulmonary fibrosis.
Pirfenidone treatment of idiopathic pulmonary fibrosis.
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DOI：
10.2147/tcrm.s12209
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发表时间：
2011-02-08
影响因子：
2.8
通讯作者：
Gomer RH
中科院分区：
文献类型：
--
作者：
Gan Y;Herzog EL;Gomer RH
特发性肺纤维化（IPF）是一种离散的临床病理实体，其定义为高分辨率CT扫描和/或开放式肺活检中通常存在间质性肺炎，并且缺乏替代诊断或暴露来解释这些发现。目前没有fda批准的治疗方法可用于治疗这种疾病，5年死亡率为80%。吡啶酮衍生物吡非尼酮已被广泛研究作为一种可能的治疗剂用于这种致命的疾病。这篇综述将介绍治疗IPF患者的医生所遇到的独特的临床特征和管理问题，包括对常规治疗的不良反应。将讨论吡非尼酮的生物化学和临床前疗效，并对临床疗效、安全性、副作用和以患者为中心的焦点（如生活质量和耐受性）进行全面回顾。希望这一信息将有助于深入了解围绕在IPF中使用吡非尼酮的复杂问题，并进一步研究这种药物作为这种毁灭性疾病的可能治疗方法。
Idiopathic pulmonary fibrosis (IPF) is a discrete clinicopathologic entity defined by the presence of usual interstitial pneumonia on high-resolution CT scan and/or open lung biopsy and the absence of an alternate diagnosis or exposure explaining these findings. There are currently no FDA-approved therapies available to treat this disease, and the 5-year mortality is ∼80%. The pyridone derivative pirfenidone has been studied extensively as a possible therapeutic agent for use in this deadly disease. This review will present the unique clinical features and management issues encountered by physicians caring for IPF patients, including the poor response to conventional therapy. The biochemistry and preclinical efficacy of pirfenidone will be discussed along with a comprehensive review of the clinical efficacy, safety, and side effects and patient-centered foci such as quality of life and tolerability. It is hoped that this information will lend insight into the complex issues surrounding the use of pirfenidone in IPF and lead to further investigation of this agent as a possible therapy in this devastating disease.