Genomic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran; Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran..
Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Int Immunopharmacol. 2018 Jan;54:131-138. doi: 10.1016/j.intimp.2017.11.005. Epub 2017 Nov 10.
Pemphigus vulgaris (PV) is a rare autoimmune disease due to the production of pathogenic autoantibodies directed against desmoglein 1 and 3, usually affecting both skin and mucous membranes. Recently, rituximab, a chimeric IgG1 monoclonal antibody which targets the CD20 molecules have been regarded as a promising treatment for PV. In this study, a systematic review was conducted to conclude on how and which PV patients could benefit from rituximab infusion. Search in PubMed results in 114 relevant studies, which met the criteria. Total of 1085 PV patients with different conditions, including unresponsive childhood/juvenile or adult PV patients, women of childbearing age, those with chronic infections with the risk of reactivation have been evaluated. Although the majority of these patients well responded to rituximab, some of them did not respond, and the paucity of patients experienced exacerbation of disease. In addition to the rituximab monotherapy or its combination with conventional therapies, different novel combination therapies of rituximab with immunoadsorption and/or IVIg have shown promising results. Moreover, using rituximab as the first-line treatment has emerged recently. Pneumocystis carinii pneumonia and septicemia were found as the two fatal and serious adverse events associated with rituximab. Moreover, development or reactivation of herpes simplex and herpes zoster and cytomegalovirus should be warned. Similar to the adults, those with childhood and juvenile PV could be successfully treated with rituximab. Although rituximab seems to trigger reactivation of chronic infections, such as viral hepatitis and HIV infection, no related report was found. Administration of rituximab in approximately ten months before conception also was found safe and effective for a successful pregnancy. In conclusion, rituximab is very effective in adult and childhood/juvenile PV. However, there is a risk of not responding, exacerbation of disease and development of fatal infections. Moreover, it seems to be a promising first-line treatment for refractory PV.
寻常型天疱疮（PV）是一种罕见的自身免疫性疾病，由针对桥粒芯糖蛋白 1 和 3 的致病性自身抗体引起，通常同时累及皮肤和黏膜。最近，利妥昔单抗（一种针对 CD20 分子的嵌合 IgG1 单克隆抗体）已被认为是治疗 PV 的一种有前途的方法。本研究进行了系统评价，以总结哪些 PV 患者以及如何从利妥昔单抗输注中受益。在 PubMed 中进行检索得到 114 项相关研究，这些研究均符合纳入标准。共纳入了 1085 例不同病情的 PV 患者，包括对儿童/青少年或成人 PV 患者无效的患者、育龄期妇女、有慢性感染风险且易复发的患者等。尽管大多数患者对利妥昔单抗反应良好，但也有部分患者无反应，极少数患者出现疾病恶化。除了利妥昔单抗单药治疗或与常规治疗联合治疗外，利妥昔单抗与免疫吸附和/或 IVIg 的不同新型联合治疗方案也显示出良好的效果。此外，最近还出现了将利妥昔单抗作为一线治疗的方法。卡氏肺孢子虫肺炎和败血症是与利妥昔单抗相关的两种致命且严重的不良事件。此外，还应警惕单纯疱疹和带状疱疹以及巨细胞病毒的发展或复发。与成人一样，儿童和青少年 PV 患者也可成功接受利妥昔单抗治疗。尽管利妥昔单抗似乎会引发慢性感染（如病毒性肝炎和 HIV 感染）的复发，但目前尚未发现相关报道。在受孕前大约 10 个月使用利妥昔单抗也被发现是安全有效的，可成功妊娠。总之，利妥昔单抗对成人和儿童/青少年 PV 均非常有效。然而，存在无反应、疾病恶化和致命感染发展的风险。此外，它似乎是一种有前途的难治性 PV 的一线治疗方法。
