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免疫吸附作为炎性肌病挽救治疗的有效性和安全性:三例报告及文献综述

Effectiveness and safety of immunoadsorption as a rescue treatment of inflammatory myopathies: report of three cases and literature review.

作者信息

Nies Jasper F, Hendrix Claudia, Bartram Malte P, Spear Ryan, Hagmann Henning, Benzing Thomas, Kubacki Torsten

机构信息

Department II of Internal Medicine and Center for Molecular Medicine Cologne, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.

Department of Internal Medicine, Rush University Medical Center, Chicago, IL, USA.

出版信息

Ther Adv Musculoskelet Dis. 2024 May 17;16:1759720X241250238. doi: 10.1177/1759720X241250238. eCollection 2024.

Abstract

Idiopathic inflammatory myopathy (IIM) summarizes rare, systemic autoimmune conditions primarily characterized by inflammatory damage to the skeletal muscle. Although primary damage occurs to the muscle, these IIM-related conditions involve other organs, including the skin, lungs, upper gastrointestinal tract, joints, and heart. While many patients have an adequate response to immunosuppressive treatment, some patients develop rapidly progressive and treatment-resistant life-threatening courses. Treatment-resistant IIM is challenging for the treating physician and requires interdisciplinary and individualized treatment approaches. Extracorporeal therapy is one option for rescue therapy, with immunoadsorption (IA) having proven more effective than plasma exchange regarding the removal of circulating antibodies. Despite its efficacy and desirable safety profile, the clinical value of IA use in IIM is understudied with no controlled trials reported. Here, we present a review of the current knowledge regarding the management of treatment-resistant IIM and the cases of three patients with treatment-resistant IIM (two with dermatomyositis and one with immune-mediated necrotizing myopathy) who have successfully been treated with IA. All patients responded well to the therapy and experienced no IA-related complications. Taken together, we found IA to be a safe and effective treatment option in treatment-resistant IIM.

摘要

特发性炎性肌病(IIM)概括了罕见的全身性自身免疫性疾病,其主要特征是骨骼肌受到炎性损伤。虽然主要损伤发生在肌肉,但这些与IIM相关的疾病还累及其他器官,包括皮肤、肺、上消化道、关节和心脏。尽管许多患者对免疫抑制治疗反应良好,但一些患者会出现快速进展且对治疗耐药的危及生命的病程。治疗耐药的IIM对治疗医生来说具有挑战性,需要采用跨学科和个体化的治疗方法。体外治疗是挽救治疗的一种选择,就清除循环抗体而言,免疫吸附(IA)已被证明比血浆置换更有效。尽管IA具有疗效且安全性良好,但在IIM中使用IA的临床价值尚未得到充分研究,也没有报道过对照试验。在此,我们对治疗耐药IIM的管理的现有知识以及三名治疗耐药IIM患者(两名皮肌炎患者和一名免疫介导性坏死性肌病患者)成功接受IA治疗的病例进行综述。所有患者对治疗反应良好,且未出现与IA相关的并发症。综上所述,我们发现IA是治疗耐药IIM的一种安全有效的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d94/11102671/eabc2d325141/10.1177_1759720X241250238-fig1.jpg

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