The Liverpool Experience: The Role of Immunosuppression in treating
Vasculitic Subglottic Stenosis
Abstract
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.