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 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).