Tamura Naoto, Matsudaira Ran, Hirashima Mika, Ikeda Makoto, Tajima Michiko, Nawata Masuyuki, Morimoto Shinji, Kaneda Kazuhiko, Kobayashi Shigeto, Hashimoto Hiroshi, Takasaki Yoshinari
Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan.
Intern Med. 2007;46(7):409-14. doi: 10.2169/internalmedicine.46.6156. Epub 2007 Apr 2.
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.
韦格纳肉芽肿的传统治疗方法，即使用类固醇和环磷酰胺，在一些难治性患者中无法控制疾病活动，且具有治疗相关毒性。使用利妥昔单抗（一种嵌合抗CD20单克隆抗体）进行B细胞清除疗法，已被证明对某些自身免疫性疾病有效，包括抗中性粒细胞胞浆抗体（ANCA）相关的系统性血管炎。我们报告了两例韦格纳肉芽肿难治性病例：一例有支气管和肺部受累及眶后肉芽肿，另一例有眶后肉芽肿和肥厚性硬脑膜炎导致严重头痛。利妥昔单抗在两例中均有效，肉芽肿缩小，ANCA滴度降低，使得类固醇能够减量。未检测到不良反应。
