Rituximab induced remission in a patient with Wegener's granulomatosis

Abstract

Background: Wegener's granulomatosis is a form of systemic vasculitis typically involving the kidneys and upper and lower respiratory tract. Treatment employing cyclophosphamide and prednisone has improved prognosis, but relapses and treatment-induced side effects still cause severe morbidity and frequent mortality. There is therefore an urgent need to find new treatment modalities that are efficient and cause few side effects. Chimeric anti-CD20 (rituximab) may be one such treatment, apparently working in part by suppressing anti-PR3 production.

Patients and methods: We describe a 26-year-old man with two relapses while on azathioprine and mycophenolate necessitating a high cumulative dose of cyclophosphamide. He was treated with a single standard course of rituximab while continuing steroids and mycophenolate.

Results: After 4 months, rituximab led to resolution of pulmonary lesions and also rapidly caused normalization of elevated anti-PR3. He still remains in complete remission 11 months later with a negative anti-PR3 and normal kidney function. There were no side effects to rituximab.

Conclusion: Rituximab may be effective and seems to be safe in inducing remission in Wegener's granulomatosis. Studies are needed to establish the long-term benefit of this expensive treatment.