Bédane C, Doffoel Hantz V
Service de dermatologie, hôpital Dupuytren, 2 avenue Martin-Luther-King, Limoges cedex, France.
Ann Dermatol Venereol. 2011 Mar;138(3):201-8. doi: 10.1016/j.annder.2011.01.012. Epub 2011 Feb 22.
Mucous membrane pemphigoid is a rare autoimmune bullous disorder. Numerous treatment regimens have been proposed in the literature.
To assess the efficacy and tolerance of treatment regimens proposed in mucous membrane pemphigoid (MMP), from a systematic review of the literature.
Randomized control trials have been identified using the PubMed and Embase databases up to April 2009. Uncontrolled prospective and retrospective studies have also been analyzed.
Literature analysis confirms that clinical and therapeutic trials are very uncommon in MMP; only retrospective series or case reports are available and have been analyzed. Therefore, the level of evidence is usually weak. Twenty-four series have been analyzed in this review. Dapsone remains the first line treatment in non-ocular forms of MMP. Sulfasalazine or cyclins can be used when dapsone is not tolerated or effective. Corticosteroids can be used to control inflammatory flares of the disease. Immunosuppressants are not used as the first line of treatment and can be added to anti-inflammatory drugs for a better control of MMP. Cyclophophamide or mycophenolate mofetil can be used, especially in the elderly. In ocular forms of the disease, the severity and chronicity of ocular involvement is the main therapeutical target. Non-scarring conjunctivitis can be treated by dapsone monotherapy. Ocular flares of the disease can be treated with systemic corticosteroids or cyclophosphamide. Many immunomodulating drugs are under evaluation. Intravenous immunoglobulins, etanercept or rituximab can be proposed when cyclophosphamide is not able to control the disease.
Data from the literature did not allow identifying the best therapeutic regimen, mainly because of the lack of prospective comparative studies. Dapsone remains the first line treatment in MMP. Immunosuppressive or immunomodulating drugs should be discussed patient by patient.
黏膜类天疱疮是一种罕见的自身免疫性大疱性疾病。文献中已提出众多治疗方案。
通过对文献的系统综述，评估黏膜类天疱疮（MMP）治疗方案的疗效和耐受性。
截至2009年4月，利用PubMed和Embase数据库检索随机对照试验。还分析了非对照的前瞻性和回顾性研究。
文献分析证实，MMP的临床和治疗试验非常少见；仅有回顾性系列研究或病例报告可供分析。因此，证据水平通常较低。本综述分析了24个系列研究。氨苯砜仍是非眼部型MMP的一线治疗药物。当不能耐受氨苯砜或其无效时，可使用柳氮磺胺吡啶或环磷酰胺。糖皮质激素可用于控制疾病的炎症发作。免疫抑制剂不作为一线治疗药物，可添加到抗炎药物中以更好地控制MMP。可使用环磷酰胺或霉酚酸酯，尤其适用于老年人。在眼部型疾病中，眼部受累的严重程度和慢性化是主要治疗目标。非瘢痕性结膜炎可用氨苯砜单一疗法治疗。疾病的眼部发作可用全身性糖皮质激素或环磷酰胺治疗。许多免疫调节药物正在评估中。当环磷酰胺无法控制疾病时，可使用静脉注射免疫球蛋白、依那西普或利妥昔单抗。
文献数据无法确定最佳治疗方案，主要是因为缺乏前瞻性比较研究。氨苯砜仍是MMP的一线治疗药物。免疫抑制或免疫调节药物应根据患者个体情况讨论使用。
