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Patients with limited Wegener’s granulomatosis (WG) may experience a relapsing and remitting course. How such patients should be treated, particularly when they are refractory to standard of care therapies, is not clear. Rituximab is a monoclonal anti-CD20 antibody that has been used successfully to treat multiple forms of autoimmune and rheumatic diseases, but its role in the treatment of limited WG remains uncertain.

Eight patients with limited WG who were refractory to (or intolerant of) standard immuno-suppressive therapies were evaluated at the Johns Hopkins Hospital or the Mayo Clinic Rochester, and were treated with rituximab using a standard lymphoma protocol.

Four men and 4 women with limited WG were treated with rituximab. Patients’ mean age was 39 years. All patients had predominantly necrotizing granulomatous disease manifestations, including chronic sinusitis …