Presentations: A modified version of the article was presented at the European League Against Rheumatism (EULAR) Annual Congress – Paris, France 11–14 June 2008.
B-cell depletion with rituximab for refractory head and neck Wegener’s granulomatosis: a cohort study
Article first published online: 23 JUL 2009
© 2009 Blackwell Publishing Ltd
Volume 34, Issue 4, pages 328–335, August 2009
How to Cite
Martinez Del Pero, M., Chaudhry, A., Jones, R.B., Sivasothy, P., Jani, P. and Jayne, D. (2009), B-cell depletion with rituximab for refractory head and neck Wegener’s granulomatosis: a cohort study. Clinical Otolaryngology, 34: 328–335. doi: 10.1111/j.1749-4486.2009.01968.x
- Issue published online: 23 JUL 2009
- Article first published online: 23 JUL 2009
- Accepted for publication 10 May 2009
Objectives: This study aimed to evaluate the response of refractory Wegener’s granulomatosis affecting the ear, nose and throat and granulomatous eye disease to B-cell depletion with rituximab.
Design: A retrospective case note review.
Setting: Tertiary Centre.
Participants: All patients who received rituximab for refractory Wegener’s granulomatosis affecting the head and neck were included.
Main outcome measures: Demographic and follow-up data at five time points were recorded. Response was measured using change in the Birmingham Vasculitis Activity Score and prednisolone dose. Secondary outcomes included changes in additional immunomodulators and anti-neutrophil cytoplasm antibodies serology. Adverse events were recorded for the duration of follow-up.
Results: Thirty-four patients were included in the analysis. The median age was 47, the male to female ratio was 3 : 2 and the overall median follow-up was 25.5 months. At six months, nine (26%) patients had a partial response, twenty-one (62%) were in remission and four (12%) did not respond. All four non-responders went into remission after a second course of rituximab. Total Birmingham Vasculitis Assessment score decreased after rituximab at all time points (P < 0.001). Four of five patients with retro-orbital involvement responded well to treatment. Two patients were considered secondary failures requiring alternative therapy after an initial response. Adverse events included four major chest infections, two cancers and six infusion reactions.
Conclusions: Our cohort derived considerable benefit from rituximab permitting a reduction in immunosuppressive exposure and prednisolone dose with few major adverse effects. There was an 80% (4/5) response in patients with retro-orbital granulomas. The effect of rituximab was most noticeable in the first 6 months (88% response).