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用霉酚酸酯作为激素节约剂治疗寻常型天疱疮。

Treatment of pemphigus vulgaris with mycophenolate mofetil as a steroid-sparing agent.

作者信息

Esmaili Nafiseh, Chams-Davatchi Cheyda, Valikhani Mahin, Farshidfar Farshad, Parvaneh Nima, Tamizifar Banafshe

机构信息

Department of Dermatology, School of Medicine, Medical Sciences - University of Tehran, Tehran, Iran.

出版信息

Eur J Dermatol. 2008 Mar-Apr;18(2):159-64. doi: 10.1684/ejd.2008.0354.

Abstract

Pemphigus vulgaris is a rare autoimmune blistering disease. Estimation of the incidence in Iran is one patient per 100,000 of the population per year. Mycophenolate mofetil is an immunosuppressive drug and successful treatment of pemphigus vulgaris and bullous pemphigoid has been reported with it, in combination with high dose prednisone, or as monotherapy. The present study describes our experience of the adjuvant use of mycophenolate mofetil in the management of 31 patients with pemphigus vulgaris as an initial treatment. We evaluated the efficacy and safety of mycophenolate mofetil combined with prednisolone in this cohort. We also assessed the relationship between the demographic indices/disease severity factors, and the failure of this treatment. In this study, mycophenolate mofetil was of definite benefit in 21 cases (67.7%). Generalized forms; patients with higher sum of the clinical scores at presentation; severe involvement of the groin; chest; face and limbs and those who had nail dystrophy also appeared to have poorer responses. When we excluded patients with generalized forms, only four patients were included in the failure group and the response rate reached 83.3%. It can be concluded that, except for generalized diseases, mycophenolate mofetil can be used safely and effectively in patients with pemphigus vulgaris as a first line, steroid sparing agent.

摘要

寻常型天疱疮是一种罕见的自身免疫性水疱病。据估计,伊朗的发病率为每年每10万人口中有1例患者。霉酚酸酯是一种免疫抑制药物,已有报道称其与高剂量泼尼松联合使用或作为单一疗法成功治疗了寻常型天疱疮和大疱性类天疱疮。本研究描述了我们将霉酚酸酯作为初始治疗辅助药物用于治疗31例寻常型天疱疮患者的经验。我们评估了霉酚酸酯联合泼尼松龙在该队列中的疗效和安全性。我们还评估了人口统计学指标/疾病严重程度因素与该治疗失败之间的关系。在本研究中,霉酚酸酯在21例患者(67.7%)中具有明确疗效。泛发型患者;就诊时临床评分总和较高的患者;腹股沟、胸部、面部和四肢严重受累的患者以及患有甲营养不良的患者似乎反应较差。当我们排除泛发型患者时,失败组仅包括4例患者,有效率达到83.3%。可以得出结论,除泛发型疾病外,霉酚酸酯可作为一线药物安全有效地用于寻常型天疱疮患者,作为一种减少类固醇使用的药物。

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