利妥昔单抗是否能作为皮肌炎的一线治疗用药?

利妥昔单抗是否能作为皮肌炎的一线治疗用药?

刘 蕊

      摘要(爱尔兰):  B细胞在皮肌炎（DM）的发病机制中起到了关键的作用，文献报道提出针对B细胞的靶向治疗对于DM患者是切实可行的。2007年一篇文章报道了1例20岁的女性患者，因近端肌无力数周入院，其指关节可见Gottron's征，她的炎症指标是正常的，且自身抗体指标也是阴性的。她的CPK值为7,000 U/L (参考值 0-170) ， LDH 是 1,300 U/L (参考值266-500)。肌电图和肌活检均提示肌炎活动。肺功能检查正常，高分辨肺CT也没有发现间质性肺病。最初给予她60mg糖皮质激素(1 mg/kg)，临床症状明显好转，但是一旦激素减量，症状即会加重。向她推荐了多种免疫抑制剂，包括实验性应用利妥昔单抗，她由于不愿意接受长时间的系统治疗，所以选择了利妥昔单抗治疗。当她接受了在第0和第14天2次应用利妥昔单抗1000mg后的几周，她所应用的糖皮质激素开始逐渐减量。现在已经是利妥昔单抗应用后的第24个月，她的临床症状及实验室检查均处于完全缓解状态，而且未用糖皮质激素及利妥昔单抗以外的免疫抑制剂。经过应用一个疗程的利妥昔单抗清除外周血B细胞的治疗，患者不仅肌肉及皮肤病变彻底好转，而且摆脱了糖皮质激素在内的所有免疫抑制剂治疗。

      附原文： Abstract : B cells may play a pivotal role in the pathophysiology of DM, and reports have claimed that targeting B cells is a viable treatment option in patients with dermatomyositis. A 20-year-old girl presented in October 2007, with few weeks' history of proximal muscle weakness. Gottron's papules were noted on her knuckles. She had normal inflammatory markers and negative autoantibody screen. Her CPK was 7,000 U/L (normal range 0-170) with an LDH of 1,300 U/L (normal range 266-500). EMG and muscle biopsy was consistent with active myositis. She had normal pulmonary function tests. HRCT showed no interstitial lung disease. She was started with 60 mg glucocorticoids (1 mg/kg), with a good clinical response. However, any attempt to taper down the steroid dose led to recurrence of her symptoms. The options of available immunosuppressive therapies, including the experimental usage of rituximab, were discussed with her; averse to long-term systemic treatments, she opted to try a course of rituximab. She had rituximab 1,000 mg on days 0 and 14, and her glucocorticoids were tapered in next few weeks. Now, 24 months since her rituximab infusions, she remains in complete clinical and biochemical remission and is naive to other immunosuppressive agents apart from glucocorticoids and rituximab. Depleting peripheral B cells with rituximab (one course) in our patient has led not only to complete resolution of muscle and skin disease (induction) but also remains off all immunosuppressives including glucocorticoids.

   引自: Haroon M, Devlin J. Rituximab as a first-line agent for the treatment of dermatomyositis. Rheumatol Int. 2010 Mar 26. [Epub ahead of print]