Brihaye B, Aouba A, Pagnoux C, Cohen P, Lacassin F, Guillevin L
French Vasculitis Study Group, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5-René-Descartes, Paris, France.
Clin Exp Rheumatol. 2007 Jan-Feb;25(1 Suppl 44):S23-7.
Rituximab, an anti-CD20 biotherapy, has been effective against refractory and/or relapsing Wegener's granulomatosis (WG). But the frequency of and time to responses to rituximab, and its effects on various clinical WG manifestations remain to be thoroughly evaluated.
Retrospective study of 8 patients with refractory/relapsing WG. In addition to their ongoing therapy, 7 patients received rituximab (375 mg/m2 weekly for 4 weeks) and another received 2 rituximab infusions (1 g on days 1 and 15). Disease activity was assessed using BVAS 2003 before and 6 months after the first rituximab infusion.
The median BVAS before rituximab was 14.3 (range 4-30). At 6 months, 5/8 patients had BVAS=0; 3/8 were in complete remission; 3/8 in partial remission (lung nodules persisted in 2 patients, scored 0 in BVAS); 2/8 did not respond. One patient relapsed 1 year after stopping rituximab and responded successfully to a second cycle. Dissociated responses of constitutional and 'vasculitis' symptoms, as opposed to granulomatous manifestations, were observed: the former regressed within days or weeks, while the latter regressed more slowly, over several months. Tolerance was good for 7 patients but 1 developed an urticarial rash during the last 3 infusions. Corticosteroids could be tapered in all patients.
Rituximab, when prescribed in conjunction with corticosteroids and immunosuppressants to treat refractory/relapsing WG, was able to improve clinical outcome. But the dissociation of response times in patients with predominantly granulomatous manifestations, as opposed to vasculitis symptoms, merits further study before an optimal rituximab regimen can be defined.
利妥昔单抗，一种抗CD20生物疗法，已被证明对难治性和/或复发性韦格纳肉芽肿（WG）有效。但利妥昔单抗的缓解频率、缓解时间及其对WG各种临床症状的影响仍有待全面评估。
对8例难治性/复发性WG患者进行回顾性研究。除了正在进行的治疗外，7例患者接受了利妥昔单抗治疗（375mg/m²，每周一次，共4周），另1例接受了2次利妥昔单抗输注（第1天和第15天各1g）。在首次输注利妥昔单抗前及输注后6个月，使用2003年BVAS评估疾病活动度。
利妥昔单抗治疗前BVAS中位数为14.3（范围4 - 30）。6个月时，5/8患者BVAS = 0；3/8患者完全缓解；3/8患者部分缓解（2例患者肺部结节持续存在，BVAS评分为0）；2/8患者无反应。1例患者在停用利妥昔单抗1年后复发，再次接受治疗后成功缓解。观察到全身症状和“血管炎”症状与肉芽肿表现的分离反应：前者在数天或数周内消退，而后者消退较慢，需数月时间。7例患者耐受性良好，但1例在最后3次输注期间出现荨麻疹皮疹。所有患者的皮质类固醇剂量均可逐渐减少。
利妥昔单抗与皮质类固醇和免疫抑制剂联合用于治疗难治性/复发性WG时，能够改善临床结局。但对于以肉芽肿表现为主的患者与血管炎症状患者在缓解时间上的分离反应，在确定最佳利妥昔单抗治疗方案前值得进一步研究。
