Doukaki Spyridoula, Platamone Andrea, Alaimo Roberta, Bongiorno Maria Rita
Department of Dermatology, University of Palermo , Palermo, Sicily , Italy.
J Dermatolog Treat. 2015 Feb;26(1):67-72. doi: 10.3109/09546634.2014.880395. Epub 2014 Feb 13.
What is known and objective: Pemphigus is a severe, potentially life-threatening autoimmune blistering disease. The use of corticosteroids has dramatically improved the prognosis and changed its course. However, current morbidity of pemphigus is largely iatrogenic, caused by side effects of the long-term, high-dose corticosteroid therapy that is necessary to sustain disease control. In order to minimize side effects, a range of corticosteroid-sparing immunosuppressive agents have been introduced, including mycophenolate mofetil (MMF) and enteric-coated mycophenolate sodium (EC-MPS). A systematic review was performed to evaluate the effectiveness of MMF and EC-MPS in the treatment of pemphigus vulgaris and pemphigus foliaceus.
A retrospective literature search was conducted through multiple electronic databases (PubMed, Medline, The Cochrane database of systematic reviews) for reports on the use of mycophenolic acid (MPA) in the treatment of pemphigus vulgaris and pemphigus foliaceus.
Sixteen studies with a total of 239 patients have evaluated the treatment of pemphigus vulgaris and pemphigus foliac;eus with MPA. The majority of patients had refractory disease treated with corticosteroids as monotherapy or associated to adjuvant agents.
The results of this review suggest that MPA, as MMF or EC-MPS, may be a promising adjuvant or alternative therapy for the treatment of pemphigus vulgaris and pemphigus foliaceus. It appears safe, at least in the medium term and its adverse events seem to be dose dependent.
The use of mycophenolate is first-line adjuvant therapy in the treatment of pemphigus vulgaris and pemphigus foliaceus.
已知信息及目的:天疱疮是一种严重的、可能危及生命的自身免疫性水疱病。皮质类固醇的使用显著改善了预后并改变了病程。然而,目前天疱疮的发病率很大程度上是医源性的,由维持疾病控制所需的长期、高剂量皮质类固醇治疗的副作用引起。为了尽量减少副作用,已引入了一系列皮质类固醇节约型免疫抑制剂,包括霉酚酸酯(MMF)和肠溶包衣霉酚酸钠(EC-MPS)。进行了一项系统评价,以评估MMF和EC-MPS治疗寻常型天疱疮和落叶型天疱疮的有效性。
通过多个电子数据库(PubMed、Medline、Cochrane系统评价数据库)进行回顾性文献检索,以获取关于霉酚酸(MPA)治疗寻常型天疱疮和落叶型天疱疮的报告。
16项研究共239例患者评估了MPA治疗寻常型天疱疮和落叶型天疱疮的情况。大多数患者患有难治性疾病,接受皮质类固醇单药治疗或与辅助药物联合治疗。
本评价结果表明,MPA作为MMF或EC-MPS,可能是治疗寻常型天疱疮和落叶型天疱疮的一种有前景的辅助或替代疗法。至少在中期它似乎是安全的,其不良事件似乎与剂量有关。
霉酚酸酯的使用是治疗寻常型天疱疮和落叶型天疱疮的一线辅助疗法。