两例治疗难治性寻常型天疱疮和严重基础疾病患者对新型IVIg制剂表现出良好的反应。

IF 3.5 3区 医学 Q1 DERMATOLOGY
Dermatology and Therapy Pub Date : 2025-01-01 Epub Date: 2025-01-03 DOI:10.1007/s13555-024-01326-6
Katharina Hogrefe, Julia K Winkler, Alexander H Enk
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引用次数: 0

摘要

寻常性天疱疮是一种严重且经常治疗抵抗的大疱性自身免疫性疾病。类固醇的标准治疗通常与另一种免疫抑制剂一起使用,并不是对所有患者都有效,或者对患有严重基础疾病的患者可能不是一个好的治疗选择。静脉注射免疫球蛋白(IVIgs)是一种治疗选择,通常表现出快速反应,可以减少伴随的免疫抑制。在这里,我们报告了两名患有严重寻常型天疱疮的复杂病史的患者,他们接受了一种新的IVIg制剂(Yimmugo®,每公斤体重每4周2克)的治疗。IVIg制剂在制造工艺上有所不同,并表现出不同的副作用。我们的两名患者都没有经历过ivig的任何副作用,皮肤和粘膜糜烂也有了明显的改善。需要更多关于静脉注射的报告,以帮助选择最佳的制剂和剂量,考虑到个体患者的耐受性和有效性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation.

Pemphigus vulgaris is a severe and often therapy-resistant bullous autoimmune disease. Standard therapy with steroids often administered together with another immunosuppressant does not respond in all patients or may not be a good therapeutic option in patients with severe underlying diseases. Intravenous immunoglobulins (IVIgs) represent a treatment alternative, often showing a rapid response which allows one to reduce concomitant immunosuppression. Here, we report on two patients with a complex disease history suffering from severe pemphigus vulgaris who received treatment with a new IVIg preparation (Yimmugo®, 2 g per kg body weight every 4 weeks). IVIg preparations differ regarding manufacturing process and show a varying side effect profile. Both of our patients did not experience any side effects from IVIgs and showed a significant improvement of skin and mucosal erosions. More reports on IVIgs are desirable to help in selecting the optimal preparation and dosing regarding tolerability and effectiveness for individual patients.

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来源期刊
Dermatology and Therapy
Dermatology and Therapy Medicine-Dermatology
CiteScore
6.00
自引率
8.80%
发文量
187
审稿时长
6 weeks
期刊介绍: Dermatology and Therapy is an international, open access, peer-reviewed, rapid publication journal (peer review in 2 weeks, published 3–4 weeks from acceptance). The journal is dedicated to the publication of high-quality clinical (all phases), observational, real-world, and health outcomes research around the discovery, development, and use of dermatological therapies. Studies relating to diagnosis, pharmacoeconomics, public health and epidemiology, quality of life, and patient care, management, and education are also encouraged. Areas of focus include, but are not limited to all clinical aspects of dermatology, such as skin pharmacology; skin development and aging; prevention, diagnosis, and management of skin disorders and melanomas; research into dermal structures and pathology; and all areas of aesthetic dermatology, including skin maintenance, dermatological surgery, and lasers. The journal is of interest to a broad audience of pharmaceutical and healthcare professionals and publishes original research, reviews, case reports/case series, trial protocols, and short communications. Dermatology and Therapy will consider all scientifically sound research be it positive, confirmatory or negative data. Submissions are welcomed whether they relate to an International and/or a country-specific audience, something that is crucially important when researchers are trying to target more specific patient populations. This inclusive approach allows the journal to assist in the dissemination of quality research, which may be considered of insufficient interest by other journals. The journal appeals to a global audience and receives submissions from all over the world.
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