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

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 immunological (10% ANCA negative) and non-specific histological biopsies (77% of head and neck specimens). 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 33.3 months. This is a significant increase compared to other published studies. 20% (4/20) of the cohort did not require any surgical input following induction IS during the study's timeframe.