Bernard P, Charneux J
Service de dermatologie, CHU de Reims, 47 rue Serge-Kochman, Reims, France.
Ann Dermatol Venereol. 2011 Mar;138(3):173-81. doi: 10.1016/j.annder.2011.01.004. Epub 2011 Feb 16.
Bullous pemphigoid (BP) is the most common auto-immune bullous disorder. Its treatment is difficult due to high age and comorbidities of affected patients.
To assess the effects of treatments for BP.
Randomized therapeutic trials (RCTs) were identified using an automatic search on Pubmed et Embase until March 2009. Large retrospective series with homogeneous therapeutic management were also selected and analyzed.
Forty-four articles were selected and analyzed, which included nine RCTs with a total of 1007 participants (653 patients were included in two trials). Two RCTs comparing different modalities of systemic corticosteroid therapy failed to show differences in measure of disease control. The addition of plasma exchanges (one RCT) or azathioprine (one RCT) allowed to halve the amount of prednisone required for disease control. A further 3-arms RCT compared plasma exchange or azathioprine plus prednisone, but failed to show significant differences for disease control or mortality of BP. One study compared tetracycline plus nicotinamide with prednisolone, no significant difference for disease response was evidenced. A large controlled clinical trial demonstrated that high doses of very potent topical corticosteroids increased initial disease control and 1-year survival of patients with extensive BP, as compared with oral prednisone. Another RCT compared two regimens of potent topical corticosteroids and a non-inferior rate of BP control was obtained with the mild regimen. Finally, a study comparing two immunosuppressant drugs (azathioprine, mycophenolate mofetil) in addition to prednisone failed to show any difference for disease control, recurrence rate or the cumulated doses of prednisone.
Ultrapotent topical corticosteroids (clobetasol propionate; 20 to 40g/day) are effective treatments for BP with fewer systemic side-effects than oral high-dose corticosteroids. Systemic corticosteroids are effective but doses greater than 0.5mg/kg per day are associated with severe side-effects, including decreased survival. The effectiveness of the addition of plasma exchange or immunosuppressants (azathioprine, mycophenolate mofetil) to systemic corticosteroids has not been established. Combination treatment with tetracycline and nicotinamide needs further validation.
大疱性类天疱疮（BP）是最常见的自身免疫性大疱性疾病。由于受累患者年龄较大且合并多种疾病，其治疗较为困难。
评估BP的治疗效果。
通过在PubMed和Embase上自动检索，直至2009年3月，确定随机治疗试验（RCT）。还选择并分析了具有同质治疗管理的大型回顾性系列研究。
选取并分析了44篇文章，其中包括9项RCT，共有1007名参与者（两项试验纳入了653名患者）。两项比较全身用皮质类固醇不同治疗方式的RCT未显示出疾病控制指标上的差异。添加血浆置换（一项RCT）或硫唑嘌呤（一项RCT）可使疾病控制所需的泼尼松用量减半。另一项三臂RCT比较了血浆置换或硫唑嘌呤加泼尼松，但在BP的疾病控制或死亡率方面未显示出显著差异。一项研究比较了四环素加烟酰胺与泼尼松龙，未发现疾病反应有显著差异。一项大型对照临床试验表明，与口服泼尼松相比，高剂量的超强效外用皮质类固醇可提高广泛型BP患者的初始疾病控制率和1年生存率。另一项RCT比较了两种强效外用皮质类固醇方案，轻度方案获得了非劣效的BP控制率。最后，一项比较两种免疫抑制药物（硫唑嘌呤、霉酚酸酯）加泼尼松的研究在疾病控制、复发率或泼尼松累积剂量方面未显示出任何差异。
超强效外用皮质类固醇（丙酸氯倍他索；每天20至40克）是治疗BP的有效方法，与口服高剂量皮质类固醇相比，全身副作用更少。全身用皮质类固醇有效，但每天剂量大于0.5毫克/千克会伴有严重副作用，包括生存率降低。在全身用皮质类固醇基础上加用血浆置换或免疫抑制剂（硫唑嘌呤、霉酚酸酯）的有效性尚未得到证实。四环素和烟酰胺联合治疗需要进一步验证。
