The Liverpool experience: The role of immunosuppression in treating vasculitic subglottic stenosis

Five Key Points • Subglottic stenosis (SGS) is the commonest tracheobronchial manifestation of granulomatosis with polyangiitis (GPA), with early recognition and treatment key to preventing its vasculitic progression and fibrosis. • Previous studies have shown SGS to be the first feature of GPA in 4% of cases. It is not uncommon to see negative biochemical (10% ANCA negative) and negative histological biopsies (77% of head and neck specimens are negative). • Our management strategy emphasized rapid SGS-GPA treatment with limited surgical manipulation of the airway and systemic immunosuppression (IS) to prevent evolution of SGS & concurrent systemic vasculitic relapse. • In our study early multi-disciplinary team involvement to deliver induction IS in the presence of active SGS-GPA led to a procedure free interval (PFI) of 31.3 months. This is a significant increase compared to other published studies. • Nineteen percent (4/21) of the cohort did not require any surgical input following induction IS.