免疫介导的坏死性肌病,坏死肌纤维中有C5b-9沉积,经高剂量糖皮质激素、他克莫司和静脉注射免疫球蛋白联合强化治疗成功

IF 2.7 Q3 IMMUNOLOGY
T. Shimada, M. Higashida-Konishi, M. Akiyama, S. Hama, K. Izumi, S. Matsubara, H. Oshima, Y. Okano
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引用次数: 1

摘要

摘要目前,尚未建立免疫介导的坏死性肌病(IMNM)的标准治疗策略。在此,我们介绍了一例IMNM,该病例通过大剂量糖皮质激素、他克莫司和静脉注射免疫球蛋白的强化联合治疗获得了成功。她的肌肉无力迅速加重,入院一周后便卧床不起。她并发吞咽困难,血清肌源性酶升高,心室舒张功能障碍,间质性肺病。血清抗SRP抗体呈阳性,她的肌肉活检显示许多坏死纤维,炎症程度很低。进一步的组织学分析表明,吞噬巨噬细胞浸润,坏死肌纤维中沉积膜攻击复合物(C5b-9),提示补体途径和巨噬细胞的激活是该疾病的病理机制。她被诊断为IMNM,并立即开始了上述联合治疗,这导致了显著的临床改善。最近的研究表明,静脉注射免疫球蛋白和他克莫司可以抑制补体途径和巨噬细胞的激活。我们目前的病例表明,早期开始强化联合治疗,包括静脉注射免疫球蛋白和他克莫司,可能通过破坏IMNM中补体和巨噬细胞的致病性激活,有效预防不可逆的肌肉损伤。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Immune-mediated necrotizing myopathy which showed deposition of C5b-9 in the necrotic muscle fibers and was successfully treated with intensive combined therapy with high-dose glucocorticoids, tacrolimus, and intravenous immunoglobulins
Abstract Currently, no standard treatment strategy has been established for immune-mediated necrotizing myopathy (IMNM). Here we present a case of IMNM which was successfully treated with intensive combined therapy with high-dose glucocorticoids, tacrolimus, and intravenous immunoglobulins. Her muscle weakness was rapidly progressive and severe so that she became bedridden one week after admission. She was complicated with dysphagia and had serum myogenic enzymes elevation, ventricular diastolic dysfunction, and interstitial lung disease. Serum anti-SRP antibody was positive and her muscle biopsy revealed many necrotic fibers with minimal inflammation. Further histological analysis demonstrated infiltration of phagocytic macrophages with deposition of membrane attack complex (C5b-9) in the necrotic muscle fibers, suggesting activation of complement pathway and macrophages as a pathomechanism of this disease. She was diagnosed as IMNM and was immediately initiated a combination therapy described above, which led to dramatic clinical improvements. Recent studies suggest that intravenous immunoglobulins and tacrolimus can inhibit the activation of complement pathway and macrophages. Our present case suggests that early initiation of intensive combined therapy including intravenous immunoglobulins and tacrolimus might be effective for preventing irreversible muscle damages by disrupting a pathogenic activation of complement and macrophages in IMNM.
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来源期刊
Immunological Medicine
Immunological Medicine Medicine-Immunology and Allergy
CiteScore
7.10
自引率
2.30%
发文量
19
审稿时长
19 weeks
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