Inflammatory and immune dysregulation associated lung disease

肺部受累的免疫或炎症调节障碍可从最初的表现，如结节病，或相对轻微的表现，如孟德尔易感分支杆菌病免疫缺陷。虽然这两者都可能与肺部的肉芽肿炎症有关，但临床上的肺部表现以及治疗策略都有很大的不同。慢性呼吸道感染实验室寻求利用与临床免疫学和微生物学实验室以及NIAID和NIAMS内其他分支机构的密切合作，重点研究这些疾病，以描述已知和新出现的免疫性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，以更好地确定STAT3在改变呼吸道清除中的作用。作为这项合作的一部分，北卡罗来纳大学的合作者利用收集的呼吸道细胞来表征呼吸道上皮细胞与新型SARS CoV-2病毒的相互作用，为其从上呼吸道到下呼吸道的传播提供了可信的解释。骨髓间充质基质细胞在体外可以调节与结节病相关的巨噬细胞相关的炎症，为该疾病的潜在治疗干预奠定了基础。肺功能的特点是两种罕见的情况，多发性内分泌肿瘤2B和先天性角化不良。最后，描述了一种新的免疫调节紊乱，PI3KGamma缺乏症在一名以淋巴细胞为主的复发性肺浸润物患者中被描述，并从一个新的小鼠模型中收集了对炎症途径的见解。

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 Laboratory of Chronic Airway Infection (LCAI) has sought to capitalize on the close collaboration with the Laboratory of Clinical Immunology and Microbiology 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 to better define the role of STAT3 in altered airway clearance. Airway cells collected as part of this collaboration were utilized by UNC collaborators to characterize the airway epithelial interaction with the novel SARS CoV-2 virus to provide a plausible explanation for its transit from the upper to lower respiratory tract. Bone marrow derived mesenchymal stromal cells were shown to modulate in vitro the inflammation associated with sarcoidosis associated macrophage, setting the stage for a potential therapeutic intervention in this disease. Pulmonary function was characterised in 2 rare conditions, Multiple Endocrine Neoplasia 2B and dyskeratosis congenita. Finally, a new disorder of immune regulation, PI3Kgamma deficiency was described in a patient with recurring lymphocyte-predominant lung infiltrates and insights into the inflammatory pathway were gleaned from a novel mouse model.