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.