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静脉注射免疫球蛋白治疗自身免疫性大疱性疾病:来自中欧转诊中心的病例系列。

Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center.

机构信息

Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, 60-355 Poznan, Poland.

Cutaneous Histopathology and Immunopathology Section, Department of Dermatology, Poznan University of Medical Sciences, 60-355 Poznan, Poland.

出版信息

Medicina (Kaunas). 2023 Jul 7;59(7):1265. doi: 10.3390/medicina59071265.

DOI:10.3390/medicina59071265
PMID:37512078
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10385677/
Abstract

: Autoimmune bullous diseases (AIBDs) may be treated with intravenous immunoglobulin (IVIG) infusions. This study aimed to evaluate the benefits and safety profiles of high-dose IVIG therapy in AIBD patients, as determined by clinical remission, the glucocorticosteroid-sparing effect, and adverse events at 12 months follow-up in a Central European university dermatology department setting. : Our case series included 10 patients: five patients with pemphigus vulgaris, one with pemphigus herpetiformis, one with pemphigus foliaceus, one with bullous pemphigoid, two with epidermolysis bullosa acquisita. They underwent 4-12 monthly cycles of IVIG therapy at a dose of 2 g/kg per cycle. : The prednisone dosage reduction after 2, 6, and 12 months following the final IVIG course was 65.45%, 70.91%, and 76.37%, respectively. During the 12-month observation period, disease relapse was observed in 20% of patients, while others achieved complete or partial remission without or with minimal therapy. Side effects were seen in 80% of patients; they were transient and did not necessitate discontinuation of IVIG. : IVIG demonstrates effectiveness as a treatment with a favorable safety profile. Nevertheless, its high cost remains a significant drawback, particularly in low-income countries. IVIG should be considered, especially in patients opposed to standard therapies or with contraindications to their use.

摘要

自身免疫性大疱性疾病(AIBD)可采用静脉注射免疫球蛋白(IVIG)治疗。本研究旨在评估高剂量 IVIG 治疗在中欧大学皮肤科环境下 AIBD 患者中的疗效和安全性,主要评估指标为临床缓解、糖皮质激素节省效应以及 12 个月随访时的不良事件。

本病例系列纳入了 10 名患者:5 名寻常型天疱疮患者、1 名疱疹样天疱疮患者、1 名落叶型天疱疮患者、1 名大疱性类天疱疮患者、2 名获得性大疱性表皮松解症患者。他们接受了 4-12 个月的 IVIG 治疗,剂量为 2 g/kg/周期。

在最后一次 IVIG 疗程结束后 2、6 和 12 个月,泼尼松剂量分别减少了 65.45%、70.91%和 76.37%。在 12 个月的观察期内,20%的患者疾病复发,而其他患者则实现了完全或部分缓解,无需或仅需最低限度的治疗。80%的患者出现了副作用,但均为一过性,无需停止 IVIG 治疗。

IVIG 作为一种治疗方法具有疗效确切、安全性良好的特点。然而,其高昂的费用仍然是一个显著的缺点,尤其是在低收入国家。因此,IVIG 应被考虑用于治疗,尤其是在那些反对标准治疗或存在这些治疗方法禁忌的患者中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/9780a9719200/medicina-59-01265-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/f11f609bf353/medicina-59-01265-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/78edf0b6baed/medicina-59-01265-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/390d87db9b0c/medicina-59-01265-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/9780a9719200/medicina-59-01265-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/f11f609bf353/medicina-59-01265-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/78edf0b6baed/medicina-59-01265-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/390d87db9b0c/medicina-59-01265-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/835d/10385677/9780a9719200/medicina-59-01265-g004.jpg

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Front Immunol. 2022 Dec 19;13:1103375. doi: 10.3389/fimmu.2022.1103375. eCollection 2022.
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An Bras Dermatol. 2022 Jul-Aug;97(4):409-423. doi: 10.1016/j.abd.2021.09.010. Epub 2022 Jun 11.
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