Inflammatory and immune dysregulation associated lung disease

炎症和免疫失调相关的肺部疾病

基本信息

批准号：
9572321
负责人：
Kenneth Olivier
金额：
$ 13.45万
依托单位：
NATIONAL HEART, LUNG, AND BLOOD INSTITUTE
依托单位国家：
美国
项目类别：
财政年份：
资助国家：
美国
起止时间：
至
项目状态：
未结题

来源：
https://reporter.nih.gov/project-details/9572321
关键词：
Alstrom syndrome Antiviral Agents Autoimmune Diseases Award Breathing Bronchoalveolar Lavage Chronic Granulomatous Disease Cilia Clinical Clinical Medicine Collaborations Collection Communicable Diseases Defect Deficiency Diseases Detection Disease Epithelial Cells Functional disorder Funding Genetic Goals Grant Granulomatous Host Defense IgE Immune Immune System Diseases Infection Inflammation Inflammatory Job&apos s Syndrome Laboratories Link Lung Lung diseases Measures Minor Mutation National Institute of Allergy and Infectious Disease National Institute of Arthritis and Musculoskeletal and Skin Diseases North Carolina Pathway interactions Pneumocystis Predisposition Regulation Research Personnel Role STAT3 gene Safety Sarcoidosis Specimen Sputum Universities asthmatic bench to bedside ciliopathy clinical investigation improved insight mycobacterial novel respiratory

项目摘要

The pulmonary involvement in disorders of immune or inflammatory regulation can range from a primary manifestation such as with sarcoidosis or a relatively minor manifestations such as seen with the Mendelian Susceptibility to Mycobacterial Disease immune deficiencies. While both of these can be associated with granulomatous inflammation in the lung, the clinical pulmonary manifestations are quite different as are the management strategies. The Pulmonary Clinical Medicine Section (PCMS) has sought to capitalize on the close collaboration with the Laboratory of Clinical Infectious Diseases and other branches within the NIAID and NIAMS focused on these disorders to describe the pulmonary manifestations of known and emerging immune and inflammatory diseases. Over the past year we have continued collaboration with investigators at the University of North Carolina seeks to better define the role of STAT3 in altered airway clearance. We were jointly awarded a Bench-to-Bedside grant that has funded collection and analysis of airway epithelial cells, bronchoalveolar lavage, sputum, and other respiratory specimen and bedside measures for characterization of the airway clearance mechanisms and potential defects. We completed analysis of Pneumocystis PCR detection in sputum from mild asthmatics collected as part of a previous clinical investigation of safety of a novel inhaled antiviral agent (J Investig Med 2017). We also completed analysis of respiratory manifestations of the rare ciliopathy, Alstrom Syndrome, which suggests possible overlap with respiratory cilia dysfunction (Pediatr Pulmonol 2017).

免疫或炎症调节疾病中肺部受累的范围可以是主要表现（例如结节病）或相对较小的表现（例如分枝杆菌病孟德尔易感性免疫缺陷）。虽然这两种情况都可能与肺部肉芽肿性炎症有关，但临床肺部表现和治疗策略却截然不同。肺临床医学科 (PCMS) 寻求利用与临床传染病实验室以及 NIAID 和 NIAMS 内其他分支机构的密切合作，重点关注这些疾病，以描述已知和新出现的免疫和炎症性疾病的肺部表现。在过去的一年里，我们继续与北卡罗来纳大学的研究人员合作，寻求更好地定义 STAT3 在气道清除改变中的作用。我们共同获得了一项从实验室到床边的资助，用于资助气道上皮细胞、支气管肺泡灌洗液、痰和其他呼吸道标本的收集和分析，以及用于表征气道清除机制和潜在缺陷的床边措施。我们完成了对轻度哮喘患者痰液中肺孢子菌 PCR 检测的分析，该痰液是先前新型吸入抗病毒药物安全性临床研究的一部分（J Investig Med 2017）。我们还完成了对罕见纤毛病阿尔斯特罗姆综合征的呼吸道表现的分析，这表明可能与呼吸纤毛功能障碍重叠（Pediatr Pulmonol 2017）。