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首页> 外文期刊>Modern rheumatology >Treatment of refractory retrobulbar granuloma with rituximab in a patient with ANCA-negative Wegener's granulomatosis: a case report.
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Treatment of refractory retrobulbar granuloma with rituximab in a patient with ANCA-negative Wegener's granulomatosis: a case report.

机译：利妥昔单抗治疗ANCA阴性韦格纳肉芽肿患者难治性球后肉芽肿：病例报告。

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Retrobulbar granuloma is one of the serious complications in Wegener's granulomatosis and often shows resistance to conventional therapy during long-term treatment. The outcome of this complication includes visual loss, orbital and facial deformity, fistula formation, as well as infection. There has been increasing evidence that shows the efficacy of rituximab, a chimeric anti-B cell mAb, for the treatment of autoimmune diseases including Wegener's granulomatosis. We present a 22-year-old Japanese woman who was diagnosed with Wegener's granulomatosis complicated by refractory retrobulbar granuloma. She was admitted to our hospital with pain of the right eye and right proptosis during treatment with monthly IVCY for Wegener's granulomatosis. We diagnosed refractory retrobulbar granuloma by computed tomography (CT) scan and biopsy. She showed a refractory growth of retrobulbar granuloma in spite of negative ANCA. She was also complicated with pulmonary granulomatous lesions in bilateral apices. After approval by an institutional ethical committee and informed consent of this patient, rituximab 375 mg/m(2) was intravenously administered weekly four times. Concomitant prednisolone 0.5 mg/kg was also administered for 2 weeks and gradually tapered. Treatment of rituximab resulted in prompt relief of symptoms in this case and the reduction of the granuloma. BVAS score also improved from 6 to 0 at 3 months and was kept in remission for 12 months. Circulating CD19-positive cells were kept less than 0.1% during the follow-up. There were no serious adverse events. This case suggests that rituximab is effective for refractory retrobulbar granuloma complicated in Wegener's granulomatosis even when ANCA titers are negative.
机译：球后肉芽肿是韦格纳肉芽肿的严重并发症之一，在长期治疗中通常表现出对常规治疗的抵抗力。这种并发症的结果包括视力丧失，眼眶和面部畸形，瘘管形成以及感染。越来越多的证据表明，利妥昔单抗（一种嵌合的抗B细胞mAb）可用于治疗自身免疫性疾病，包括韦格纳肉芽肿病。我们介绍了一名22岁的日本妇女，她被诊断出患有韦格纳肉芽肿病并伴有难治性后球肉芽肿。她在每月进行韦格纳肉芽肿病的IVCY治疗期间因右眼和右突的疼痛而入院。我们通过计算机断层扫描（CT）扫描和活检诊断为难治性球后肉芽肿。尽管ANCA阴性，但她仍显示出球后肉芽肿难治性生长。她还伴有双侧肺部肉芽肿性病变。经机构伦理委员会批准并征得该患者的知情同意后，每周四次静脉给药375 mg / m（2）利妥昔单抗。还给予0.5 mg / kg泼尼松龙2周，并逐渐减量。利妥昔单抗的治疗可在这种情况下迅速缓解症状并减少肉芽肿。 BVAS评分在3个月时也从6改善到0，并保持缓解12个月。在随访期间，循环中的CD19阳性细胞保持低于0.1％。没有严重的不良事件。该病例表明，即使当ANCA滴度为负值时，利妥昔单抗对难治性韦格纳肉芽肿中并发的难治性球后肉芽肿也有效。

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