Idiopathic Pulmonary Fibrosis Clinical Research Network

描述（由申请人提供）： 特发性肺纤维化（IPF）是一种进行性肺部疾病，通常导致呼吸衰竭和死亡，诊断后中位生存期为3年。 最近的研究表明，由皮质类固醇和细胞毒性药物组成的传统治疗对提高生存率无效。 目前没有有效的治疗这种疾病。 IPF发病机制基础研究的最新进展和新型治疗药物的开发增加了对合作临床研究网络的需求，以评估新的管理策略。 一个已建立的临床研究者小组（马约间质性肺疾病小组）对IPF有着长期的兴趣和跟踪记录，建议在马约诊所建立一个临床中心，以参与NIH资助的IPF临床研究网络，进行合作治疗试验。 这些临床研究者及其肺部临床研究中心的研究人员在招募和保留IPF和其他肺部疾病临床试验受试者方面具有丰富的经验。 该临床中心提出了两项治疗研究供网络考虑。 第一项研究将检查伊马替尼和喷替福林联合方案与安慰剂相比治疗IPF的效果。 第二个项目将研究秋水仙碱和N-乙酰半胱氨酸联合治疗与安慰剂相比的效果。 为了开展这些和其他IPF临床研究网络研究，该临床中心将从马约诊所罗切斯特每年就诊的200 - 250例新发IPF患者中抽取样本。 马约间质性肺疾病诊所登记研究和计算机化数据库将允许识别和参与既往在该临床中心就诊的其他IPF受试者。 该临床研究中心还可以接触到在马约诊所杰克逊维尔（FL）和马约诊所斯科茨代尔（AZ）就诊的其他潜在参与者。 (End摘要）

DESCRIPTION (provided by applicant): Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease that commonly results in respiratory failure and death with a median survival of three years after diagnosis. Recent studies demonstrate traditional therapy consisting of corticosteroids and cytotoxic agents to be ineffective in improving survival. There is currently no effective treatment for this disorder. Recent advances in basic research on the pathogenesis of IPF and the development of novel therapeutic agents have increased the need for a cooperative clinical research network in evaluating new management strategies. An established group of clinical investigators (Mayo Interstitial Lung Disease Group) with a longstanding interest and track record in IPF proposes the development of a clinical center at Mayo Clinic to participate in the NIH-funded IPF Clinical Research Network for conducting collaborative therapeutic trials. These clinical investigators and their research staff of the Pulmonary Clinical Research Center have considerable experience in recruitment and retention of subjects for clinical trials in IPF and other lung diseases. This clinical center proposes two treatment studies for consideration by the network. The first study will examine the effects of combined imatinib and pentoxifylline regimen compared to placebo in the treatment of IPF. The second project will study the effects of combined colchicines and N-acetylcysteine therapy compared to placebo. To conduct these and other IPF Clinical Research Network studies, this clinical center will draw from 200-250 new patients with IPF seen each year at Mayo Clinic Rochester. The Mayo Interstitial Lung Disease Clinic registry and computerized databases will allow identification and participation of other subjects with IPF previously seen at this clinical center. This clinical center will also have access to other potential participants seen at Mayo Clinic Jacksonville (FL) and Mayo Clinic Scottsdale (AZ). (End of Abstract)