Undifferentiated Connective Tissue-associated Interstitial Lung Disease Cohort

描述（由申请人提供）：这是Brent Kinder博士的K23奖申请，他是辛辛那提大学肺病和重症监护助理教授，他是一名以患者为导向的间质性肺疾病（ILD）临床研究的年轻研究者。K23奖将为Kinder博士提供必要的支持，以实现以下目标：(1)成为间质性肺疾病的临床和流行病学研究专家；(2)建立第一个仔细定义的未分化结缔组织病（UCTD）相关ILD病例的前瞻性队列；(3)采用先进的生物统计学方法分析UCTD-ILD进展的危险因素；(4)探讨支气管肺泡灌洗液和血清表面活性蛋白紊乱对疾病进展的影响；(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，由他的赞助人和主要导师Francis X. McCormack博士和共同导师Talmadge E. King, Jr.博士组成，Francis X. McCormack博士是辛辛那提大学肺病和重症监护科的主任，他对弥漫性肺实质疾病进行临床和基础研究，Talmadge E. King, Jr.博士是加州大学旧金山分校医学教授和医学系主任。他是国际公认的间质性肺疾病临床研究的领导者。ILD的发展现在是几种结缔组织疾病死亡的主要原因。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，利用临床、放射学、生理、生化和形态学参数，对仔细定义的uctd相关ILD病例进行前瞻性、纵向研究（Aim 1;）。根据美国风湿病学会的标准，确定ILD和UCTD患者疾病进展和演变为CTD的临床危险因素（目的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患者ILD病程的关系（目的3）。这项研究将为Kinder博士提供必要的技能和初步数据，以设计一项前瞻性的、多中心的、UCTD-ILD的分子流行病学研究，该研究将在K奖结束前提交R01资助申请。

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)