Effectiveness of Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis (Wegener's)

Abstract

Objective. Ear, nose, and throat (ENT) involvement is the most prevalent manifestation of granulomatosis with polyangiitis (Wegener's) (GPA) and correlates with permanent damage and decreased quality of life. Although prior studies have evaluated the efficacy of rituximab (RTX) for granulomatous features of GPA, none have evaluated its efficacy solely for ENT manifestations. We compared the effectiveness of RTX to other therapies for the ENT manifestations of GPA in a large, well-characterized cohort. Methods. We performed a retrospective analysis of 975 visits from 99 GPA patients seen at a tertiary care ENT practice between 2003 and 2013. At each visit, subjects had a complete ENT examination, with ENT activity assessed by a single expert otolaryngologist. ENT disease activity during the observational period in subjects receiving RTX was compared to subjects receiving all other therapy. Results. In total, 48 subjects had never received RTX and 51 received RTX at least once. There was no active ENT disease during 92.4% of the observational period (days) for subjects receiving RTX, compared with 53.7% of the observational period for subjects not receiving RTX (odds ratio 11.0 [95% confidence interval 5.5-22.0], P < 0.0001). Subjects receiving RTX, compared with those receiving methotrexate, azathioprine, cyclophosphamide, or trimethoprim-sulfamethoxazole, were significantly more likely to have no active ENT disease (P < 0.0001 for each comparison). Conclusion. RTX is an effective treatment for ENT manifestations of GPA. Subjects treated with RTX were significantly less likely to have active ENT disease compared with those not receiving RTX. Patients being treated with RTX were 11 times less likely to have active ENT disease than patients being treated with other therapies.

Current Therapies for ANCA-Associated Vasculitis

“…This is a notoriously refractory disease feature, and radiographic differentiation between active disease and necrosis can be difficult. Recently, in the largest reported experience of RTX solely for otolaryngologic manifestations of GPA, patients receiving RTX for active ENT (ear nose and throat) disease were >10 times less likely to have active ENT disease compared to patients being treated with other therapies (50). Results of this retrospective study suggest RTX may be a useful therapy for granulomatous otolaryngologic disease in GPA.…”

“…In addition to frequent relapse, persistent, grumbling otolaryngologic disease is common and can lead to accrual of permanent damage such as nasal bridge collapse, hearing loss, and subglottic stenosis. The efficacy of RTX for the granulomatous compared to vasculitic manifestations of GPA is debated, with several case series offering conflicting results (46)(47)(48)(49)(50). Failure of several of these studies to demonstrate efficacy of RTX for granulomatous GPA was driven mostly by failure to improve orbital disease.…”

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

“…Our patients with refractory disease responded to rituximab therapy. It has been reported that rituximab is an effective treatment for ENT manifestations of GPA and patients treated with rituximab were 11 times less likely to have active ENT disease than patients treated with other therapies [28].…”

Current Landscape of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

“…Additionally, persistent, grumbling otolaryngologic disease is common and can lead to accrual of permanent damage, such as nasal bridge collapse, hearing loss, and subglottic stenosis, making treatment of these disease manifestations crucial. The efficacy of RTX for the granulomatous compared with vasculitic manifestations of GPA is debated, with several case series offering conflicting results [64][65][66][67][68] In the largest reported observational cohort looking at RTX solely for otolaryngologic manifestations of GPA, patients given RTX for active ENT disease were greater than 10 times less likely to have active ENT disease compared with patients treated with other therapies 68 ; results of this retrospective study suggest RTX may be a useful therapy for granulomatous otolaryngologic disease in GPA.…”