title: Two cases of refractory Wegener's granulomatosis successfully treated with rituximab.
abstract: Conventional therapy for Wegener's granulomatosis, steroid and cyclophosphamide, fails to control disease activity in some refractory patients and has treatment-related toxicity. B cell depletion therapy using rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective for certain autoimmune diseases including antineutrophil cytoplasmic antibody (ANCA) -associated systemic vasculitis. We report two refractory cases of Wegener's granulomatosis: one with bronchial and pulmonary involvement and retroorbital granuloma, the other with retroorbital granuloma and hypertrophic pachymeningitis causing severe headache. Rituximab was effective in both cases, with diminished granuloma and reduced ANCA titers, allowing steroids to be tapered. No adverse effects were detected.
authors.affiliation: Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan. tnaoto@med.juntendo.ac.jp <tnaoto@med.juntendo.ac.jp>
authors.forename: Naoto
authors.initials: N
authors.lastname: Tamura
authors.forename: Ran
authors.initials: R
authors.lastname: Matsudaira
authors.forename: Mika
authors.initials: M
authors.lastname: Hirashima
authors.forename: Makoto
authors.initials: M
authors.lastname: Ikeda
authors.forename: Michiko
authors.initials: M
authors.lastname: Tajima
authors.forename: Masuyuki
authors.initials: M
authors.lastname: Nawata
authors.forename: Shinji
authors.initials: S
authors.lastname: Morimoto
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Kaneda
authors.forename: Shigeto
authors.initials: S
authors.lastname: Kobayashi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hashimoto
authors.forename: Yoshinari
authors.initials: Y
authors.lastname: Takasaki
mesh_terms: D000293:Adolescent; D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D008279:Magnetic Resonance Imaging; D011241:Prednisone / Q000627:therapeutic use; D012074:Remission Induction / Q000379:methods; D018570:Risk Assessment; D000069283:Rituximab; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D011241:Prednisone