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.
MeSH Keywords: laryngostenosis, acquired subglottic stenosis,
Granulomatosis with Polyangiitis, immunosuppressants, Antineutrophil
Cytoplasmic Antibodies