Atzmony Lihi, Hodak Emmilia, Gdalevich Michael, Rosenbaum Omer, Mimouni Daniel
Department of Dermatology, Rabin Medical Center, Beilinson Hospital, 39 Jabotinski St., 49100, Petach Tikva, Israel.
Am J Clin Dermatol. 2014 Dec;15(6):503-15. doi: 10.1007/s40257-014-0101-9.
No optimal therapeutic approach has been established for pemphigus.
Our objective was to evaluate the efficacy, steroid-sparing effect, and safety of available treatment modalities.
PubMed, LILACS (up to July 2014), the Cochrane Central Register of Controlled Trials (CENTRAL, issue 5 of 12, May 2014), and the ClinicalTrials.gov registry and reference lists were searched for randomized controlled trials of any treatment modality for pemphigus vulgaris and pemphigus foliaceus. Data were extracted independently by two authors using predefined appraisal criteria and data fields.
A total of 20 studies (826 participants) were included. Most were small and open-labeled; all but seven were not concealed for allocation. Owing to the variability in intervention arms, five meta-analyses were performed, each pooling the data of two to three trials. Studies excluded from the meta-analyses were described quantitatively. Azathioprine had a steroid-sparing effect but did not increase remission rate. Mycophenolate mofetil induced sustained remission more quickly than did placebo and delayed time to relapse but did not have a steroid-sparing effect or favorable remission rate. Cyclophosphamide had a steroid-sparing effect, though less than azathioprine, but did not affect the remission rate or time-to-disease control. Intravenous immunoglobulin had more favorable short-term efficacy than did placebo. Topical epidermal growth factor hastened lesion healing.
Although some of the available therapeutic modalities for pemphigus are beneficial in terms of steroid-sparing, hastening response, or delaying relapse, none were found to increase the complete response rate compared with glucocorticoids alone, currently the mainstay of treatment. Multicenter randomized controlled trials and case control studies with uniform outcome measures are warranted.
天疱疮尚无最佳治疗方法。
我们的目的是评估现有治疗方式的疗效、激素节省作用和安全性。
检索了PubMed、LILACS（截至2014年7月）、Cochrane对照试验中心注册库（CENTRAL，2014年5月第12期第5号）以及ClinicalTrials.gov注册库和参考文献列表，以查找寻常型天疱疮和落叶型天疱疮任何治疗方式的随机对照试验。两名作者使用预先确定的评估标准和数据字段独立提取数据。
共纳入20项研究（826名参与者）。大多数研究规模较小且为开放标签；除7项研究外，其余均未采用随机分配隐藏。由于干预组的变异性，进行了5项荟萃分析，每项荟萃分析汇总了两到三项试验的数据。对排除在荟萃分析之外的研究进行了定量描述。硫唑嘌呤具有激素节省作用，但未提高缓解率。霉酚酸酯比安慰剂更快地诱导持续缓解，并延迟复发时间，但不具有激素节省作用或良好的缓解率。环磷酰胺具有激素节省作用，尽管不如硫唑嘌呤，但不影响缓解率或疾病控制时间。静脉注射免疫球蛋白的短期疗效比安慰剂更有利。外用表皮生长因子可加速皮损愈合。
虽然天疱疮的一些现有治疗方式在激素节省、加快反应或延迟复发方面有益，但与目前作为主要治疗方法的单独使用糖皮质激素相比，未发现任何一种治疗方式能提高完全缓解率。有必要开展多中心随机对照试验和具有统一结局指标的病例对照研究。
