利妥昔单抗
是一种嵌合单克隆抗体,针对 B 细胞上表达的 CD20 抗原,但不存在于浆细胞和干细胞。治疗结果导致 B 细胞耗竭。由于皮肌炎是一种体液介导的疾病,肌肉活检发现存在丰富的 B 细胞,因此 B 细胞可作为较好的治疗靶点。[38]Dalakas MC. Immunopathogenesis of inflammatory myopathies. Ann Neurol. 1995;37:S74-S86.http://www.ncbi.nlm.nih.gov/pubmed/8968219?tool=bestpractice.com在对 6 名皮肌炎患者的开放性试验中,治疗 4 周内皮损有所改善。肌肉病变、肺功能和脱发也得到改善。4 名患者循环 B 细胞增加与肌肉症状加重有关,但 2 名患者在 1 年的随访中有改善。[173]Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum. 2005;52:601-607.http://onlinelibrary.wiley.com/doi/10.1002/art.20849/fullhttp://www.ncbi.nlm.nih.gov/pubmed/15692974?tool=bestpractice.com一些病例报告和小型系列报告称使用利妥昔单抗后皮肌炎的皮损和肌肉症状都有改善。[174]Rios Fernández R, Callejas Rubio JL, Sánchez Cano D, et al. Rituximab in the treatment of dermatomyositis and other inflammatory myopathies. A report of 4 cases and review of the literature. Clin Exp Rheumatol. 2009;27:1009-1016.http://www.ncbi.nlm.nih.gov/pubmed/20149324?tool=bestpractice.com但大多数病例报告描述的是在难治性皮肌炎中使用利妥昔单抗。因此,需要进行随机对照试验确定利妥昔单抗在皮肌炎治疗中的作用。
肿瘤坏死因子α (TNF-α) 受体阻滞剂
英夫利昔单抗和依那西普通过阻断促炎细胞因子 TNF-α的活动发挥作用。小型病例系列研究和病例报告称使用英利昔单抗后肌肉病变有改善,使用依那西普后皮损和肌肉病变均有改善。[175]Hengstman GJ, van den Hoogen FH, van Engelen BG. Treatment of dermatomyositis and polymyositis with anti-tumor necrosis factor-alpha: long-term follow up. Eur Neurol. 2004;52:61-63.http://www.ncbi.nlm.nih.gov/pubmed/15240986?tool=bestpractice.com[176]Miller M, Mendez E, Klein-Gitelman M, et al. Use of etanercept in juvenile dermatomyositis. Arthritis Rheum. 2002;46:S306.[177]Saadeh C. Etanercept is effective in the treatment of polymyositis/dermatomyositis which is refractory to conventional therapy. Arthritis Rheum. 2000;43:193.[178]Sprott H, Glatzel M, Michel BA. Treatment of myositis with etanercept (Enbrel), a recombinant human soluble fusion protein of TNF-alpha type II receptor and IgG1. Rheumatology (Oxford). 2004;43:524-526.http://rheumatology.oxfordjournals.org/cgi/content/full/43/4/524-ahttp://www.ncbi.nlm.nih.gov/pubmed/15024139?tool=bestpractice.com[179]Norman R, Greenberg RG, Jackson JM. Case reports of etanercept in inflammatory dermatoses. J Am Acad Dermatol. 2006;54(suppl 2):S139-S142.http://www.ncbi.nlm.nih.gov/pubmed/16488329?tool=bestpractice.com还需要进行随机对照试验,以进一步评估治疗皮肌炎的疗效和安全性。