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).

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3
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2017
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U.S. National Heart Lung and Blood Inst
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Hsu, Denise C; Breglio, Kimberly F; Pei, Luxin et al. (2018) Emergence of Polyfunctional Cytotoxic CD4+ T Cells in Mycobacterium avium Immune Reconstitution Inflammatory Syndrome in Human Immunodeficiency Virus-Infected Patients. Clin Infect Dis 67:437-446
Sanchez, Gina A Montealegre; Reinhardt, Adam; Ramsey, Suzanne et al. (2018) JAK1/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies. J Clin Invest 128:3041-3052
Davey, Emma L; Colombo, Rhonda E; Fiorentino, Charles et al. (2017) Pneumocystis colonization in asthmatic patients not receiving oral corticosteroid therapy. J Investig Med 65:800-802
Boerwinkle, Caroline; Marshall, Jan D; Bryant, Joy et al. (2017) Respiratory manifestations in 38 patients with Alström syndrome. Pediatr Pulmonol 52:487-493
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Colombo, Rhonda E; Fiorentino, Charles; Dodd, Lori E et al. (2016) A phase 1 randomized, double-blind, placebo-controlled, crossover trial of DAS181 (Fludase®) in adult subjects with well-controlled asthma. BMC Infect Dis 16:54
Kuhns, Douglas B; Fink, Danielle L; Choi, Uimook et al. (2016) Cytoskeletal abnormalities and neutrophil dysfunction in WDR1 deficiency. Blood 128:2135-2143
Lau, Chuen-Yen; Mihalek, Andrew D; Wang, Jing et al. (2016) Pulmonary Manifestations of the Autoimmune Lymphoproliferative Syndrome. A Retrospective Study of a Unique Patient Cohort. Ann Am Thorac Soc 13:1279-88
Mihalek, Andrew D; Haney, Carissa; Merino, Maria et al. (2016) Exercise-induced haemoptysis as a rare presentation of a rare lung disease. Thorax 71:865-8
Ferre, Elise M N; Rose, Stacey R; Rosenzweig, Sergio D et al. (2016) Redefined clinical features and diagnostic criteria in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. JCI Insight 1:

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