Dupuy Alain, Viguier Manuelle, Bédane Christophe, Cordoliani Florence, Blaise Sophie, Aucouturier Françoise, Bonnetblanc Jean-Marie, Morel Patrice, Dubertret Louis, Bachelez Hervé
Institut de Recherche sur la Peau, Institut National de la Santé et de la Recherche Médicale Unité 532, and Service de Dermatologie 2, Laboratoire d'Immunologie, Hôpital Saint-Louis Assistance Publique-Hôpitaux de Paris, France.
Arch Dermatol. 2004 Jan;140(1):91-6. doi: 10.1001/archderm.140.1.91.
Pemphigus vulgaris (PV) is a severe antibody-mediated autoimmune blistering disease. Because some patients with PV do not enter into remission, despite the use of high-dose corticosteroid therapy and immunosuppressive adjuvant treatments, new effective and safer agents are warranted to treat refractory PV. Rituximab, a monoclonal anti-CD20 antibody, induces depletion of B cells in vivo and has shown efficacy in patients with refractory antibody-mediated autoimmune disorders. We describe herein 3 patients treated with rituximab for severe PV.
Three patients with refractory PV were treated with rituximab, resulting in a clinical response in all patients, which was complete in 2 patients. A decline in titers of circulating antiepidermis autoantibodies paralleled disease activity, while circulating B cells remained undetectable for 6 to 10 months. Two patients experienced bacterial infection in the weeks following the rituximab course. A clinical relapse occurred in 2 patients, at 6 and 10 months. A second course of rituximab controlled the disease in one of them.
These patients' response suggests that rituximab may be a valuable treatment for refractory PV and warrants further studies to evaluate the risk-benefit ratio in patients with PV showing resistance to classic therapy.
寻常型天疱疮（PV）是一种严重的抗体介导的自身免疫性水疱病。由于一些PV患者即使使用高剂量皮质类固醇疗法和免疫抑制辅助治疗也无法缓解，因此需要新的有效且更安全的药物来治疗难治性PV。利妥昔单抗是一种单克隆抗CD20抗体，可在体内诱导B细胞耗竭，并已在难治性抗体介导的自身免疫性疾病患者中显示出疗效。我们在此描述3例接受利妥昔单抗治疗的重度PV患者。
3例难治性PV患者接受了利妥昔单抗治疗，所有患者均有临床反应，其中2例完全缓解。循环抗表皮自身抗体滴度的下降与疾病活动平行，而循环B细胞在6至10个月内一直无法检测到。2例患者在利妥昔单抗疗程后的几周内发生了细菌感染。2例患者分别在6个月和10个月时出现临床复发。其中1例患者接受第二次利妥昔单抗疗程后疾病得到控制。
这些患者的反应表明，利妥昔单抗可能是治疗难治性PV的一种有价值的疗法，有必要进一步研究以评估其在对经典疗法耐药的PV患者中的风险效益比。
