Jasper F. Nies, Claudia Hendrix, Malte P. Bartram, Ryan Spear, Henning Hagmann, Thomas Benzing, Torsten Kubacki
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引用次数: 0
摘要
特发性炎症性肌病(IIM)是一种罕见的全身性自身免疫性疾病,主要特征是骨骼肌的炎症性损伤。虽然主要损害发生在肌肉上,但这些与 IIM 相关的疾病也会累及其他器官,包括皮肤、肺、上消化道、关节和心脏。虽然许多患者对免疫抑制治疗有充分的反应,但也有一些患者会出现快速进展和耐药的危及生命的病程。耐药的 IIM 对治疗医生来说具有挑战性,需要跨学科和个性化的治疗方法。体外疗法是抢救治疗的一种选择,在清除循环抗体方面,免疫吸附(IA)被证明比血浆置换更有效。尽管免疫吸附疗法具有良好的疗效和安全性,但对其在 IIM 中应用的临床价值研究不足,目前尚无对照试验报告。在此,我们回顾了目前有关治疗耐药IIM的知识,并介绍了三位成功接受IAM治疗的耐药IIM患者的病例(其中两位患有皮肌炎,一位患有免疫介导的坏死性肌病)。所有患者对治疗反应良好,没有出现与 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方案。
Effectiveness and safety of immunoadsorption as a rescue treatment of inflammatory myopathies: report of three cases and literature review
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.
期刊介绍:
Therapeutic Advances in Musculoskeletal Disease delivers the highest quality peer-reviewed articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of musculoskeletal disease.