Undifferentiated Connective Tissue-associated Interstitial Lung Disease Cohort

描述(申请人提供)：这是对布伦特·金德博士的K23奖项的申请，他是辛辛那提大学肺和重症护理的助理教授，正在以患者为导向的间质性肺疾病(ILD)临床研究确立自己的年轻研究员地位。K23奖项将为Kinder博士提供必要的支持，以实现以下目标：(1)成为间质性肺疾病临床和流行病学研究方面的专家；(2)建立第一个仔细定义的未分化结缔组织病(UCTD)相关ILD病例的预期队列；(3)应用先进的生物统计学方法分析UCTD-ILD进展的风险因素；(4)研究支气管肺泡灌洗液和血清表面活性物质蛋白的紊乱对疾病进展的影响；以及(5)发展独立的临床研究事业。为了实现这些目标，Kinder博士组建了一个指导团队，成员包括他的赞助人和主要导师Francis X.McCormack博士，辛辛那提大学肺部和重症监护科主任，负责弥漫性实质性肺疾病的临床和基础研究；以及共同导师小Talmadge E.King博士，他是加州大学旧金山分校医学教授和医学系主任，是间质性肺部疾病临床研究的国际公认领导者。ILD的发展现在是几种结缔组织疾病的主要死亡原因。Kinder博士将利用辛辛那提大学ILD中心和NHLBI赞助的罕见肺病联盟协调中心的资源，使用临床、放射、生理、生化和形态参数对精心定义的UCTD相关ILD病例进行前瞻性、纵向研究(目标1；)，确定ILD和UCTD患者的疾病进展的临床风险因素和演变到定义的CTD的速率(目标2；)，并确定血清和支气管肺泡灌洗液表面活性蛋白A和D与UCTD的ILD病程的关联(目标3)。这项研究将为Kinder博士提供必要的技能和初步数据，以设计一项关于UCTD-ILD的前瞻性、多中心、分子流行病学研究，该研究将在K奖结束前的R01拨款申请中提出。 公共卫生相关性： 在UCTD的背景下，提高我们对ILD发展的病程和结果的了解，对于制定证据基础至关重要，以此为基础设计对这种疾病的未来研究，这种疾病在年轻妇女的工作高峰期对她们造成不成比例的影响。(摘要结束)

DESCRIPTION (provided by applicant): This is an application for a K23 award for Dr. Brent Kinder, an Assistant Professor in Pulmonary and Critical Care at the University of Cincinnati, who is establishing himself as a young investigator in patient-oriented clinical research of interstitial lung diseases (ILD). This K23 award will provide Dr. Kinder with the support necessary to accomplish the following goals: (1) to become expert in clinical and epidemiologic research on interstitial lung disease; (2) to establish the first prospective cohort of carefully defined cases of Undifferentiated Connective Tissue Disease (UCTD)-associated ILD; (3) to implement advanced biostatistical methods to analyze the risk factors for progression of UCTD-ILD; (4) to examine the influence of derangement in bronchoalveolar lavage and serum surfactant proteins on disease progression; and (5) to develop an independent clinical research career. To achieve these goals, Dr. Kinder has assembled a mentoring team comprised of his sponsor and primary mentor, Dr. Francis X. McCormack, Chief of the Pulmonary and Critical Care Division at the University of Cincinnati, who conducts clinical and basic investigation in diffuse parenchymal lung disease, and a co-mentor, Dr. Talmadge E. King, Jr., Professor of Medicine and Chair of the Department of Medicine at the University of California, San Francisco, who is an internationally recognized leader in the clinical investigation of interstitial lung disease. The development of ILD is now the leading cause of death in several connective tissue diseases. Dr. Kinder will leverage the resources of the University of Cincinnati ILD Center and the coordinating center for the NHLBI-sponsored Rare Lung Disease Consortium to establish a prospective, longitudinal study of carefully defined cases of UCTD-associated ILD using clinical, radiological, physiological, biochemical, and morphological parameters (Aim 1;), to define the clinical risk factors for disease progression and rate of evolution to a defined CTD by American College of Rheumatology criteria in patients with ILD and UCTD (Aim 2;), and to define the association of serum and bronchoalveolar lavage surfactant proteins A and D with disease course of ILD in UCTD (Aim 3). This research will provide Dr. Kinder with the necessary skills and preliminary data to design a prospective, multi-center, molecular epidemiologic study of UCTD-ILD that will be proposed in an R01 grant application before the end of the K award. PUBLIC HEALTH RELEVANCE: Improving our understanding of the disease course and outcome of the development of ILD in the setting of UCTD is critical to developing an evidence-base on which to design futures studies of this disease that disproportionately impacts young women during their peak working years. (End of Abstract)