We report a case of thrombotic thrombocytopenic purpura (TTP) secondary to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis treated by rituximab. TTP secondary to ANCA-associated vasculitis is very rare and has a high mortality rate. We employed rituximab and successfully treated TTP secondary to ANCA-associated vasculitis, because standard therapies, such as steroid therapy, intravenous pulse cyclophosphamide, and repeated plasma exchange (PE), did not suppress her disease activity. This is the first report to suggest that rituximab can achieve complete remission of TTP secondary to ANCA-associated vasculitis.
2010 by The Japanese Society of Internal Medicine