Emory Clinical Research Center in IPF

描述（由申请人提供）： 特发性肺纤维化（IPF）是一种特发性间质性肺炎，其特征是肺实质快速被纤维化替代，导致肺功能进行性恶化。 这个过程往往导致呼吸衰竭和死亡。 虽然有几种药物正在研究中，但目前还没有治疗这种毁灭性疾病的有效方法。 本提案是为了响应NIH发起的名为“IPF临床研究网络”的RFA而提交的，目的是成为该网络中的临床研究中心之一。 我们的研究中心将设在埃默里间质性肺疾病治疗和研究中心（埃默里ILD中心）。 Emory ILD中心被认为是IPF和相关疾病患者诊断、治疗和研究的首要中心，因为：1）它目前是在五家埃默里医院中的任何一家医院、亚特兰大市区的几个学术机构中发现的间质性肺病患者的转诊基地，并通过遍布格鲁吉亚州和该国东南部地区的私人执业医生网络; 2）作为IPF和其他间质性肺病相关临床研究的中心，在IPF相关临床试验和转化研究方面积累了丰富的经验; 3）由临床研究者、翻译研究者、研究协调员和其他具有IPF患者诊断和管理专业知识的工作人员组成的多学科团队;和4）它由本领域公认的研究人员团队指导，他们的工作是阐明肺组织重塑和纤维增生的机制。 该提案描述了网络将考虑的两项临床试验，目标是促纤维化途径和肺成纤维细胞/肌成纤维细胞，这是负责肺纤维化中纤维增生和组织重塑的关键效应细胞。 它还包括临床研究技能核心，指导初级研究者发展必要的技能，成为参与IPF相关研究的独立临床科学家。 领导临床研究中心和临床研究技能核心的研究者团队目前正在从事与IPF相关的临床研究，并在指导年轻研究者进入学术界方面享有盛誉。 (End摘要）

DESCRIPTION (provided by applicant): Idiopathic Pulmonary Fibrosis (IPF) is an idiopathic interstitial pneumonia characterized by the rapid replacement of the lung parenchyma with fibrosis which results in progressive deterioration of lung function. This process often leads to respiratory failure and death. Although several drugs are under investigation, currently there are no effective means of treating this devastating disease. This proposal is submitted in response to the NIH-sponsored RFA titled "IPF Clinical Research Network" with the intention of becoming one of the clinical research centers within this network. Our site will be based at the Emory Center for the Treatment and Study of Interstitial Lung Disease (The Emory ILD Center). The Emory ILD Center is considered a premier center for the diagnosis, treatment, and research of patients with IPF and related disorders because: 1) it currently serves as a referral base for patients with interstitial lung disease identified at any of five Emory hospitals, at several academic institutions in metro Atlanta, and through a network of private practice physicians located throughout the state of Georgia and the Southeastern region of the country; 2) it has served as a site for clinical-based investigations related to IPF and other interstitial lung diseases, and as such has developed extensive experience in clinical trials and translational research related to IPF; 3) it is staffed by a multidisciplinary team of clinical investigators, translational researchers, research coordinators, and other staff with expertise in the diagnosis and management of patients with IPF; and 4) it is directed by a team of investigators recognized in the field for their work in elucidating the mechanisms of lung tissue remodeling and fibroproliferation. The proposal describes two clinical trials to be considered by the Network that target pro-fibrotic pathways and the lung fibroblast/myofibroblast, the key effector cell responsible for fibroproliferation and tissue remodeling in pulmonary fibrosis. It also includes a Clinical Research Skills Core that will mentor junior investigators into developing the necessary skills to become independent clinician scientists involved in research related to IPF. The team of investigators leading the clinical research center and the Clinical Research Skills Core are currently engaged in clinical research related to IPF and have developed a reputation for mentoring young investigators into careers in academia. (End of Abstract)