Daniel Ebenezer, Thorne Jennifer E
Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
Curr Opin Ophthalmol. 2008 Jul;19(4):292-7. doi: 10.1097/ICU.0b013e328302cc61.
The aim of this article is to review articles on mucous membrane pemphigoid published in the English literature from 2006 to 2007.
The risk of ocular disease among patients with oral only mucous membrane pemphigoid is estimated at 15-20% at 5 years. Biopsy with direct immunofluorescent testing is essential to confirm the diagnosis because histopathology even with electron microscopy does not appear to be specific enough to confirm the diagnosis of mucous membrane pemphigoid with consistency. Autoantibodies to IgG and IgG4 appear to be the most common findings observed on direct immunofluorescent testing in patients with mucous membrane pemphigoid. Treatment for mucous membrane pemphigoid typically involves immunosuppressive drugs, including biologic therapy, as well as intravenous immunoglobulin, although results have been mixed and the majority of data reported still are from case series and anecdotal evidence.
Mucous membrane pemphigoid continues to be a potentially life and sight threatening disease. Therapy for this disease continues to show varied results although benefits are clearly seen in some patients. The immunosuppressive drugs used for treating mucous membrane pemphigoid and certain definitive categories of mucous membrane pemphigoid continue to evolve.
本文旨在回顾2006年至2007年发表于英文文献中关于黏膜类天疱疮的文章。
仅有口腔黏膜类天疱疮的患者发生眼部疾病的风险在5年时估计为15% - 20%。活检及直接免疫荧光检测对于确诊至关重要，因为即使结合电子显微镜检查，组织病理学表现似乎也不足以特异性地确诊黏膜类天疱疮。在黏膜类天疱疮患者的直接免疫荧光检测中，针对IgG和IgG4的自身抗体似乎是最常见的发现。黏膜类天疱疮的治疗通常涉及免疫抑制药物，包括生物治疗以及静脉注射免疫球蛋白，尽管结果不一，且大多数报道的数据仍来自病例系列和轶事证据。
黏膜类天疱疮仍然是一种可能危及生命和视力的疾病。尽管在部分患者中明显可见益处，但该疾病的治疗效果仍存在差异。用于治疗黏膜类天疱疮的免疫抑制药物以及某些特定类型的黏膜类天疱疮仍在不断发展。
