Engineer L, Ahmed A R
Department of Oral Medicine and Diagnostic Sciences, Harvard School of Dental Medicine, Boston, MA 02115, USA.
J Am Acad Dermatol. 2001 Jan;44(1):83-8. doi: 10.1067/mjd.2000.112288.
Conventional therapy of severe bullous pemphigoid (BP) relies on the use of high-dose systemic corticosteroids with or without adjuvants, notably immunosuppressive agents. This approach can result in debilitating and potentially fatal side effects, hence the need to explore alternative therapies. Intravenous immunoglobulin (IVIg) therapy is emerging as one possibility.
Our purpose was to determine whether any preliminary conclusion can be drawn about the potential for the use of IVIg to treat patients with severe BP.
A literature search was done to identify reports, in English-language peer-reviewed journals, on the use of IVIg to treat patients with BP. Reports were examined for information on disease duration, severity, therapies used before and after IVIg, dose and frequency of IVIg administration, and its immediate and long-term effects.
Data on treatment of 17 patients indicated that 12 patients (70%) experienced a beneficial clinical response to IVIg. In 5 patients (30%) no clinical benefit was observed. A minimum dose of 2 g/kg per cycle at monthly intervals for 3 months has been the most common approach. However, this should not be perceived as a "standard dose" at the present time. In some patients the use of IVIg appears to permit a systemic corticosteroid-sparing effect. Longer use has achieved sustained clinical remission in some patients. Lack of response was observed in patients who received low-dose IVIg or who received a single infusion only. Minimal side effects in the form of headaches and nausea and vomiting were observed in some patients.
The present experience consists only of open uncontrolled trials in a few patients and does not allow for definitive conclusions. However, at this time IVIg appears to be a promising agent for the treatment of BP, especially for patients who do not respond to conventional therapy. Adequate doses for longer periods may be required to induce and maintain sustained clinical remissions. Large-scale controlled studies with defined entry criteria, objectives, end points, and long-term follow-up are necessary to determine the specific role of IVIg in the overall management of BP.
重度大疱性类天疱疮（BP）的传统治疗依赖于使用大剂量全身用糖皮质激素，可联合或不联合辅助药物，尤其是免疫抑制剂。这种方法可能导致使人虚弱甚至有潜在致命风险的副作用，因此需要探索替代疗法。静脉注射免疫球蛋白（IVIg）治疗正成为一种可能的选择。
我们的目的是确定能否就使用IVIg治疗重度BP患者的潜力得出任何初步结论。
进行文献检索，以识别英文同行评审期刊上关于使用IVIg治疗BP患者的报告。检查报告中有关疾病持续时间、严重程度、IVIg治疗前后使用的疗法、IVIg给药剂量和频率及其即时和长期效果的信息。
17例患者的治疗数据表明，12例患者（70%）对IVIg有良好的临床反应。5例患者（30%）未观察到临床获益。最常用的方法是每3个月每月间隔1次，每次每周期最低剂量为2 g/kg。然而，目前不应将此视为“标准剂量”。在一些患者中，使用IVIg似乎可以产生节省全身用糖皮质激素的效果。在一些患者中，更长时间的使用已实现持续临床缓解。接受低剂量IVIg或仅接受单次输注的患者未观察到反应。一些患者出现了以头痛、恶心和呕吐形式表现的轻微副作用。
目前的经验仅包括少数患者的开放非对照试验，无法得出明确结论。然而，目前IVIg似乎是治疗BP的一种有前景的药物，特别是对于那些对传统治疗无反应的患者。可能需要足够剂量并持续更长时间以诱导和维持持续临床缓解。需要进行具有明确入选标准、目标、终点和长期随访的大规模对照研究，以确定IVIg在BP整体管理中的具体作用。
