Idiopathic Pulmonary Fibrosis Clinical Research Network

描述（由申请人提供）： 特发性肺纤维化（IPF）是最常见的病因不明的间质性肺病，其特征是不断进展至死亡-只有20 - 30%的IPF患者在诊断后存活5年或更长时间。 IPF的常规管理主要基于抑制炎症将防止进展为纤维化的概念。 显然，这种方法已经失败;目前没有有效的治疗方法。 本申请的目的是参与IPF临床研究网络（IPF-CRN），其目标是评价新的和现有的IPF管理方法，并将这些研究产生的结果传播给医学界。 为了支持这一申请，我们提出了两个方案，解决了重要的临床问题，测试了IPF的新治疗方法，并需要一个多中心的学术研究网络才能完成。 具体目标：1）组建一个由临床和基础科学家组成的多学科小组，作为多中心IPF临床研究网络的一部分，合作设计和实施治疗新诊断IPF患者的治疗试验;和2）使用UCSF间质性肺疾病卓越中心（ILD-COE）建立的临床研究基础设施在IPF-CRN的主持下实施多中心临床试验。 方案1：泼尼松联合硫唑嘌呤治疗IPF的随机、双盲、安慰剂对照、多中心试验。 方案2：干扰素γ-1b联合吡非尼酮治疗IPF的随机、双盲、安慰剂对照、多中心试验。 (End摘要）

DESCRIPTION (provided by applicant): Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease of unknown etiology and is characterized by a relentless progression to death - only 20 to 30% of IPF patients survive 5 years or longer after their diagnosis. Conventional management of IPF has been primarily based on the concept that suppressing inflammation would prevent progression to fibrosis. Clearly, this approach has failed; there is no effective treatment currently in use. The purpose of this application is to participate in the IPF Clinical Research Network (IPF-CRN) whose goal is to evaluate new and existing approaches for the management of IPF and to disseminate the findings generated from these studies to the medical community. In support of this application, we present two protocols that address important clinical questions, test novel therapeutic approaches for IPF, and require a multicenter, academic research network for completion. Specific Aims: 1) to assemble a multidisciplinary group of clinical and basic scientists to collaborate in the design and implementation of therapeutic trials for the treatment of patients with newly diagnosed IPF, as part of the multicenter IPF Clinical Research Network; and 2) to use the clinical research infrastructure established by the UCSF Interstitial Lung Disease Center of Excellence (ILD-COE) to implement multicenter clinical trials conducted under the auspices of IPF-CRN. Protocol 1: A randomized, double-blind, placebo-controlled, multicenter trial of prednisone plus azathioprine in the treatment of IPF. Protocol 2: A randomized, double blind, placebo controlled, multicenter trial of interferon gamma-1b plus pirfenidone in the treatment of IPF. (End of Abstract)