Effect of rituximab on refractory Wegener granulomatosis with predominant granulomatous disease.

Wegener granulomatosis is an autoimmune disorder with a double spectrum. Vasculitic manifestations are at one end of it, whereas granulomatous ones are at the other. Rituximab (RTX) is a chimeric monoclonal antibody that has been successfully used in this condition. However, the granulomatous forms have been reported to show a tendency to be less responsive to RTX than the vasculitic disease. We present 4 cases of predominantly Wegener granulomatosis that responded positively to RTX.