Inflammatory and immune dysregulation associated lung disease

炎症和免疫失调相关的肺部疾病

• 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
• 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' 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

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).

免疫或炎症调节障碍累及肺部的范围可以从原发性表现（如结节病）或相对轻微的表现（如对分枝杆菌病的孟德尔易感性免疫缺陷）。虽然这两者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。肺病临床医学科（PCMS）力求利用与临床传染病实验室以及NIAID和NIAMS内专注于这些疾病的其他分支机构的密切合作，描述已知和新出现的免疫和炎症疾病的肺部表现。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在改变气道清除中的作用。我们联合获得了一项从实验室到床边的资助，该资助用于收集和分析气道上皮细胞、支气管肺泡灌洗液、痰液和其他呼吸道标本，以及表征气道清除机制和潜在缺陷的床边措施。我们完成了对一种新型吸入式抗病毒药物安全性临床研究中收集的轻度哮喘患者痰液中肺囊虫PCR检测的分析（J investigation Med 2017）。我们还完成了对罕见纤毛病Alstrom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（pediatrics Pulmonol 2017）。