Successful treatment of refractory tracheal stenosis complicating anti-neutrophil cytoplasm antibody-associated vasculitis with sirolimus

Abstract

Laryngotracheal granulomatous inflammation is a clinical entity that may complicate either localized or systemic disorders. It can result in life-threatening airway compromise, requiring urgent medical or surgical intervention. We report the case of a patient presenting with recurrent tracheal stenosis secondary to anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis, refractory to conventional treatments. Despite multiple endoscopic dilatations and surgical resection of tracheal rings, disease control remained suboptimal. Introduction of sirolimus led to a sustained improvement of the stenotic lesion, as demonstrated by clinical and instrumental assessment. Sirolimus may be considered a valuable therapeutic option for severe subglottic inflammatory manifestations in ANCA-associated vasculitis.