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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Intravenous immunoglobulin therapy in patients with multiple mucosal involvement in mucous membrane pemphigoid.
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Intravenous immunoglobulin therapy in patients with multiple mucosal involvement in mucous membrane pemphigoid.

机译：多粘膜累及粘膜类天疱疮患者的静脉内免疫球蛋白治疗。

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Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is an autoimmune mucocutaneous, blistering disease which can lead to blindness and/or death from sudden asphyxiation, secondary to a scarring process. Conventional therapy for the treatment of MMP consists of high-dose systemic corticosteroids and/or immunosuppressive agents. Some patients do not respond to these treatments and develop multiple serious side effects, which can be potentially fatal. In such patients, alternative treatment modalities are needed. This study presents the use of intravenous immunoglobulin (IVIg) therapy in 15 patients with severe MMP whose disease was nonresponsive to the prolonged use of high-dose systemic corticosteroids and immunosuppressive agents and who developed multiple side effects to them. All 15 patients received an IVIg dose of 1-2 g/kg/cycle. The following objective parameters were used to assess the clinical outcome pre- and post-IVIg therapy: number of side effects, frequencies of recurrences and relapses, duration and total dosage of prednisone therapy, and the quality of life. The differences in these variables between the pre- and post-IVIg data were statistically analyzed using the SAS UNIVARIATE software running the two-sided Wilcoxon signed-rank and sign tests. A statistically significant difference was observed between pre- and post-IVIg therapy data when comparing the aforementioned variables. All 15 patients had an effective clinical response, were able to discontinue previous systemic therapies, and eventually achieved a prolonged clinical remission. IVIg improved the quality of life in all 15 patients and demonstrated a steroid-sparing effect. No serious side effects were observed. IVIg therapy is a safe and effective alternative modality in the treatment of patients with nonresponsive and progressive MMP and can induce a sustained clinical remission. (c)2001 Elsevier Science.
机译：粘膜类天疱疮（MMP），也称为瘢痕类天疱疮（CP），是一种自身免疫性的皮肤粘膜，起泡性疾病，可因瘢痕形成而导致突然窒息导致失明和/或死亡。用于治疗MMP的常规疗法包括高剂量全身性皮质类固醇和/或免疫抑制剂。一些患者对这些疗法无反应，并发展出多种严重的副作用，这可能是致命的。在此类患者中，需要其他治疗方式。这项研究提出了在15名重症MMP患者中使用静脉免疫球蛋白（IVIg）治疗，这些患者的疾病对长时间使用大剂量全身性皮质类固醇和免疫抑制剂无反应，并对他们产生多种副作用。所有15位患者均接受1-2 g / kg /周期的IVIg剂量。以下客观参数用于评估IVIg治疗前后的临床结局：副作用数量，复发和复发的频率，泼尼松治疗的持续时间和总剂量以及生活质量。使用运行双面Wilcoxon符号秩和符号测试的SAS UNIVARIATE软件，对IVIg前后数据之间这些变量的差异进行了统计分析。比较上述变量时，在IVIg治疗前后的数据之间观察到统计学上的显着差异。所有15例患者均具有有效的临床反应，能够中止先前的全身疗法，并最终实现了长期的临床缓解。 IVIg改善了所有15例患者的生活质量，并显示了类固醇保护作用。没有观察到严重的副作用。 IVIg疗法是治疗无反应和进行性MMP的患者的一种安全有效的替代方法，可以诱导持续的临床缓解。 （c）2001 Elsevier科学。

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