Marzano Angelo V, Fanoni Daniele, Venegoni Luigia, Berti Emilio, Caputo Ruggero
Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena of Milan, Milan, Italy.
Dermatology. 2007;214(4):310-8. doi: 10.1159/000099591.
Pemphigus is a severe blistering disorder caused by autoantibodies to desmogleins 1 and 3. Because some patients with pemphigus never enter into remission, new immunosuppressants are warranted. Rituximab is a chimeric monoclonal antibody binding to the CD20 antigen on B cells, which proved to be effective in recalcitrant pemphigus.
To evaluate the efficacy and safety of rituximab in refractory pemphigus and to investigate its effects on the autoantibody profile.
Six patients with recalcitrant pemphigus were treated. Rituximab was administered intravenously at a dosage of 375 mg/m2 body surface once weekly for 4 weeks.
Three pemphigus foliaceus patients and 1 with mucocutaneous pemphigus vulgaris (PV) showed complete response over a follow-up period of up to 18 months. In one oral PV, control of the disease was achieved using pulse therapy with cyclophosphamide following rituximab withdrawal. In one PV with vegetating features, good improvement was obtained after 6 rituximab infusions. All patients tolerated the treatment well. Anti-desmoglein autoantibodies significantly decreased only in pemphigus foliaceus.
This study highlights that rituximab is a valuable drug for refractory pemphigus, although the response of mucous membranes and cutaneous folds may be delayed.
天疱疮是一种由针对桥粒芯糖蛋白1和3的自身抗体引起的严重水疱性疾病。由于一些天疱疮患者从未进入缓解期，因此需要新的免疫抑制剂。利妥昔单抗是一种与B细胞上的CD20抗原结合的嵌合单克隆抗体，已被证明对难治性天疱疮有效。
评估利妥昔单抗治疗难治性天疱疮的疗效和安全性，并研究其对自身抗体谱的影响。
对6例难治性天疱疮患者进行治疗。利妥昔单抗以375mg/m²体表面积的剂量静脉注射，每周1次，共4周。
3例落叶型天疱疮患者和1例黏膜皮肤寻常型天疱疮（PV）患者在长达18个月的随访期内显示完全缓解。1例口腔PV患者在停用利妥昔单抗后使用环磷酰胺脉冲治疗实现了疾病控制。1例具有增殖性特征的PV患者在输注6次利妥昔单抗后有明显改善。所有患者对治疗耐受性良好。仅在落叶型天疱疮患者中，抗桥粒芯糖蛋白自身抗体显著降低。
本研究强调，利妥昔单抗是治疗难治性天疱疮的一种有价值的药物，尽管黏膜和皮肤褶皱处的反应可能会延迟。
