■天疱疮(PF)与寻常型天疱疮(PV)的不同之处在于它仅影响皮肤,而不涉及粘膜。天疱疮通常用全身性皮质类固醇和免疫抑制剂(ISA)治疗。最近,生物制剂已被使用。当前关于生物治疗的文献通常将PF与PV的治疗相结合,因此,临床医生通常很难从PV中分离出PF的治疗方法。这篇综述的目的是提供有关当前生物疗法使用的信息,特别是PF。
■搜索PubMed,Embase,其他数据库使用关键词天疱疮(PF)进行,利妥昔单抗(RTX),静脉注射免疫球蛋白(IVIg),和生物制品。41项研究包括在这篇综述中,产生了105名PF患者,用RTX处理,IVIg,或两者的组合。85名患者接受了RTX治疗,8例IVIg患者,和12接受RTX和IVIg。
■本综述中的大多数患者患有对常规免疫抑制疗法(CIST)无反应的PF,并有明显的副作用。RTX治疗导致63.2%的完全缓解(CR),复发率为39.5%,感染率为19.7%,死亡率为3.9%。淋巴瘤(LP)方案的复发大于类风湿性关节炎(RA)方案(p<0.0001)。IVIg导致62.5%的患者出现CR,没有复发或感染。接受两种生物制剂的患者在首次使用RTX时都经历了更好的结果,然后是IVIg。接受RTX的患者的随访持续时间,IVIg,分别为22.1、24.8和35.7个月,分别。
■在对常规免疫抑制疗法无反应的天疱疮患者中,或在对CIST有明显副作用的患者中,RTX和IVIg似乎是有用的试剂。临床反应概况,以及复发,感染,接受RTX治疗的PF患者的死亡率与PV患者的死亡率相似。数据表明,特定于PF的协议可能会产生更好的结果,更少的不利影响,提高了生活质量。
Pemphigus foliaceus (PF) differs from pemphigus vulgaris (PV) in that it affects only the skin and mucous membranes are not involved. Pemphigus is commonly treated with systemic corticosteroids and immunosuppressive agents (ISAs). More recently, biologics have been used. The current literature on biologic therapy often combines treatment of PF with PV, hence it is often difficult for clinicians to isolate the treatment of PF from PV. The purpose of this
review was to provide information regarding the use of current biological therapy, specifically in PF.
A search of PubMed, Embase, and other databases was conducted using keywords pemphigus foliaceus (PF), rituximab (RTX), intravenous immunoglobulin (IVIg), and biologics. Forty-one studies were included in this
review, which produced 105 patients with PF, treated with RTX, IVIg, or a combination of both. Eighty-five patients were treated with RTX, eight patients with IVIg, and 12 received both RTX and IVIg.
Most patients in this
review had PF that was nonresponsive to conventional immunosuppressive therapies (CIST), and had significant side effects from their use. RTX treatment resulted in complete remission (CR) in 63.2%, a relapse rate of 39.5%, an infection rate of 19.7%, and a mortality rate of 3.9%. Relapse was greater in the lymphoma (LP) protocol than the rheumatoid arthritis (RA) protocol (p<0.0001). IVIg led to CR in 62.5% of patients, with no relapses or infections. Patients receiving both biologics experienced better outcomes when RTX was first administered, then followed by IVIg. Follow-up durations for patients receiving RTX, IVIg, and both were 22.1, 24.8, and 35.7 months, respectively.
In pemphigus foliaceus patients nonresponsive to conventional immunosuppressive therapy or in those with significant side effects from CIST, RTX and IVIg appear to be useful agents. Profile of clinical response, as well as relapse, infection, and mortality rates in PF patients treated with RTX were similar to those observed in PV patients. The data suggests that protocols specific for PF may produce better outcomes, less adverse effects, and improved quality of life.