Otolaryngological progression of granulomatosis with polyangiitis after systemic treatment with rituximab.

OBJECTIVE: Rituximab is used for the treatment of granulomatosis with polyangiitis (GPA), historically known as Wegener's granulomatosis. However, the otolaryngological progression of GPA after systemic treatment with rituximab (Rituxan) is unclear. We therefore examined the disease sequelae of patients with GPA who were treated with rituximab. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care medical center. SUBJECTS AND METHODS: Patients with a diagnosis of GPA who were treated with rituximab between 2006 and 2012 were included in this study. Systemic and otolaryngological symptomatology, prednisone usage, and procedural interventions following B-cell depletion were analyzed. RESULTS: We identified 11 patients who met our inclusion criteria. The average length of follow-up after treatment with rituximab was 23.5 months. After treatment with rituximab, there was a significant decrease in daily prednisone dose at 3, 12, and 18 months postinfusion (P < .05). However, there was no observed improvement in patients' otolaryngological complaints as measured by the Birmingham Vasculitis Activity Score. Furthermore, patients treated with rituximab underwent numerous otolaryngological interventions during follow-up. Patients with a history of subglottic stenosis (n = 6) underwent an average of 3.40 laryngoscopies and 0.58 dilations per year during rituximab remission, and patients with sinusitis also underwent multiple nasal endoscopies (4.54 per year, n = 9) and nasal debridements (1.34, n = 9). CONCLUSIONS: While rituximab has been shown to be noninferior to cyclophosphamide with respect to remission from systemic GPA, these patients continue to have chronic otolaryngological manifestations of their disease. Otolaryngologists must continue to play a supportive role throughout their maintenance period.