Setting: Departmental treatment of SGS-GPA
Patients were included in this study from the joint ENT and vasculitis
multi-disciplinary team (MDT) database. The MDT consists of
otolaryngologists with sub-specialty expertise in vasculitis,
multi-organ vasculitis consultants and a vasculitis advanced nurse
practitioner. Those with suspected or confirmed vasculitis were reviewed
for ENT involvement in the joint vasculitis clinic. Patients with
significant SGS involvement were consented for airway assessment under
general anaesthesia (laryngotracheobronhcoscopy) with/without biopsy and
dilatation.
At surgery, high dose intravenous steroid (commonly 500mg - 1g
methylprednisolone) was administered at induction of general
anaesthesia. The subglottis was dilatated to its normal caliber using
either pulmonary balloons (Boston ScientificTM) or
serial dilatation with bougie.
A multi-organ vasculitis team review was arranged on diagnosis of
SGS-GPA.
Patients with a new diagnosis of active SGS-GPA inflammation received
induction IS with steroids and Cyclophosphamide or Rituximab.
Target date for the start of induction IS was a couple of months prior
to surgery where possible, however diagnostic delays and patients with
severe symptoms requiring urgent intervention delayed initiation of
induction IS in certain instances. Induction IS was administered in
accordance with European guidelines concerning Anti-Neutrophil
Cytoplasmic Antibodies (ANCA) associated vasculitis.6
Symptom review and nasendoscopy was undertaken on follow-up to assess
for SGS progression. Patients in remission or exhibiting fibrotic
non-active SGS-GPA received maintenance IS, usually consisting of either
Azathioprine or Rituximab maintenance therapy.