Adjunction of rituximab to steroids and immunosuppressants for refractory/relapsing Wegener's granulomatosis: a study on 8 patients.

OBJECTIVE: Rituximab, an anti-CD20 biotherapy, has been effective against refractory and/or relapsing Wegener's granulomatosis (WG). But the frequency of and time to responses to rituximab, and its effects on various clinical WG manifestations remain to be thoroughly evaluated.

METHODS: Retrospective study of 8 patients with refractory/relapsing WG. In addition to their ongoing therapy, 7 patients received rituximab (375 mg/m2 weekly for 4 weeks) and another received 2 rituximab infusions (1 g on days 1 and 15). Disease activity was assessed using BVAS 2003 before and 6 months after the first rituximab infusion.

RESULTS: The median BVAS before rituximab was 14.3 (range 4-30). At 6 months, 5/8 patients had BVAS=0; 3/8 were in complete remission; 3/8 in partial remission (lung nodules persisted in 2 patients, scored 0 in BVAS); 2/8 did not respond. One patient relapsed 1 year after stopping rituximab and responded successfully to a second cycle. Dissociated responses of constitutional and 'vasculitis' symptoms, as opposed to granulomatous manifestations, were observed: the former regressed within days or weeks, while the latter regressed more slowly, over several months. Tolerance was good for 7 patients but 1 developed an urticarial rash during the last 3 infusions. Corticosteroids could be tapered in all patients.

CONCLUSION: Rituximab, when prescribed in conjunction with corticosteroids and immunosuppressants to treat refractory/relapsing WG, was able to improve clinical outcome. But the dissociation of response times in patients with predominantly granulomatous manifestations, as opposed to vasculitis symptoms, merits further study before an optimal rituximab regimen can be defined.