Inflammatory and immune dysregulation associated lung disease

肺部参与免疫或炎症调节的疾病可以从原发性表现如结节病或相对较小的表现如孟德尔易感性分枝杆菌病免疫缺陷。 虽然这两种疾病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完全不同。肺部临床医学科（PCMS）试图利用与临床感染性疾病实验室和NIAID和NIAMS内其他分支机构的密切合作，专注于这些疾病，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州大学的研究人员合作，寻求更好地确定STAT3在改变气道清除率中的作用。我们共同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床边测量，以表征气道清除机制和潜在缺陷。 我们完成了对轻度哮喘患者痰液中肺孢子虫PCR检测的分析，这是既往一项新型吸入性抗病毒剂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Alstrom综合征的呼吸系统表现的分析，这表明可能与呼吸纤毛功能障碍重叠（Pediatr Pulmonol 2017）。

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).