Interest of therapeutic plasmapheresis in a chronic hemodialysis patient with severe bullous pemphigoid

IF 1.4 4区 医学 Q4 HEMATOLOGY Journal of Clinical Apheresis Pub Date : 2024-06-16 DOI:10.1002/jca.22133
Pedram Ahmadpoor, Mathilde Beck, Moise Michel, Emilie Pambrun, Pierre Stoebner, Olivier Moranne
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Abstract

Bullous pemphigoid is the most common autoimmune blistering disease induced by autoantibodies against basement membrane anchoring proteins (anti-BP-180 and anti-BP-230). The disease generally appears after the age of 70 and is associated with a 23.5% 1-year mortality, especially in diabetics, or in the presence of ischemic heart disease and high anti-BP-180. Treatment starts with topical steroids but some patients may require oral steroids and systemic immunosuppression. We, hereby, discuss a diabetic patient on chronic hemodialysis, with severely relapsed bullous pemphigoid under biotherapy with omalizumab, who was successfully treated with five sessions of double filtration plasmapheresis, thus avoiding the need for systemic steroids.

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一名患有严重大疱性类天疱疮的慢性血液透析患者对治疗性浆膜穿刺的兴趣。
大疱性类天疱疮是由针对基底膜锚定蛋白(抗-BP-180 和抗-BP-230)的自身抗体诱发的最常见的自身免疫性水疱病。该病一般出现在 70 岁以后,1 年死亡率为 23.5%,尤其是糖尿病患者或患有缺血性心脏病和高抗 BP-180 的患者。治疗首先从局部类固醇开始,但有些患者可能需要口服类固醇和全身免疫抑制剂。我们在此讨论一名长期血液透析的糖尿病患者,他患有严重复发的大疱性类天疱疮,在使用奥马珠单抗进行生物治疗后,成功接受了五次双滤过性浆细胞分离治疗,从而避免了全身使用类固醇的需要。
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来源期刊
CiteScore
2.80
自引率
13.30%
发文量
70
审稿时长
>12 weeks
期刊介绍: The Journal of Clinical Apheresis publishes articles dealing with all aspects of hemapheresis. Articles welcomed for review include those reporting basic research and clinical applications of therapeutic plasma exchange, therapeutic cytapheresis, therapeutic absorption, blood component collection and transfusion, donor recruitment and safety, administration of hemapheresis centers, and innovative applications of hemapheresis technology. Experimental studies, clinical trials, case reports, and concise reviews will be welcomed.
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