Successful treatment of a patient with refractory Wegener's granulomatosis by rituximab

Abstract

  We herein report the successful use of rituximab in a 55-year-old woman with refractory PR3-ANCA-associated Wegener's granulomatosis. She responded to treatments with high dose methylprednisolone (mPSL), oral and intravenous cyclophosphamide, intravenous gammaglobulin, immunoabsorption, oral prednisolone (PSL), and oral ciclosporin, although she frequently relapsed with various symptoms, such as perforation of the small intestine, scleritis, orbital granuloma, complete AV block, multiple lung nodules, cerebellar infarction, and pharyngeal granuloma with a high level of PR3-ANCA. During her latest relapse, she was given high dose mPSL and 2 infusions of 600 mg (375 mg/m²) of anti-CD20 monoclonal antibody rituximab with an interval of 4 weeks, followed by a tapering of the administered PSL. The patient's PR3-ANCA levels normalized within 2 months, but at 6 months after the beginning of the administration of rituximab, the PR3-ANCA level gradually increased without any clinical symptoms. Therefore, two more infusions of rituximab were again administered and thereafter the PR3-ANCA level finally normalized. At present, at 14 months after the initial rituximab treatment, the patient's remission continues to be maintained. The successful treatment of this patient by rituximab therefore suggests that it is an effective and new therapeutic approach for the treatment of refractory Wegener's granulomatosis.