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Idiopathic subglottic stenosis (iSGS) is a debilitating extrathoracic obstruction involving the lower laryngeal and upper tracheal airway. It arises without a known antecedent injury or associated disease process. iSGS is a fibrotic disease marked histologically by excessive accumulation of fibrous connective tissue components of the extracellular matrix (ECM, i.e., collagen and fibronectin) in inflamed tissue, which leads to airway obstruction and clinical dyspnea. Diverse diseases in divergent organ systems are associated with fibrosis, suggesting common pathogenic pathways. One of the most common is sustained host inflammation. Recent investigations focusing on the inflammatory response associated with iSGS have sought to characterize the immunophenotype and cytokine profile of the airway scar in iSGS. While the role of the immune response as inciting event in iSGS remains unresolved, the centrality of an active immune response to the observed subglottic tissue remodeling is becoming more defined.

The role of inflammatory cytokines in the development of idiopathic subglottic stenosis