B-cell depletion with rituximab for refractory head and neck Wegener's granulomatosis: a cohort study.
OBJECTIVE
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.


METHODS
A retrospective case note review.


METHODS
Tertiary Centre.


METHODS
All patients who received rituximab for refractory Wegener's granulomatosis affecting the head and neck were included.


METHODS
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).
Martinez Del Pero
M
M
Department of Otolaryngology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK. marcos@doctors.org.uk
Chaudhry
A
A
Jones
R B
RB
Sivasothy
P
P
Jani
P
P
Jayne
D
D
D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D000911:Antibodies, Monoclonal / Q000009:adverse effects / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001402:B-Lymphocytes / Q000187:drug effects*; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007155:Immunologic Factors / Q000009:adverse effects / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008212:Lymphocyte Depletion / Q000379:methods*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010038:Otorhinolaryngologic Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D011239:Prednisolone / Q000009:adverse effects / Q000627:therapeutic use; D012008:Recurrence; D019233:Retreatment; D000069283:Rituximab; D055815:Young Adult
D019268:Antibodies, Antineutrophil Cytoplasmic; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide; D011239:Prednisolone