Inflammatory and immune dysregulation associated lung disease

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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.