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静脉注射环磷酰胺成功治疗抗信号识别颗粒抗体阳性肌炎:一例报告

Successful treatment of anti-signal recognition particle antibody-positive myositis with intravenous cyclophosphamide: A case report.

作者信息

Temmoku Jumpei, Yoshida Shuhei, Tsuchihashi Kanae, Sumichika Yuya, Saito Kenji, Matsumoto Haruki, Fujita Yuya, Matsuoka Naoki, Asano Tomoyuki, Matsuda Nozomu, Sato Shuzo, Migita Kiyoshi

机构信息

Department of Rheumatology, Fukushima Medical University School of Medicine.

Department of Neurology, Fukushima Medical University School of Medicine.

出版信息

Fukushima J Med Sci. 2025 Jan 18;71(1):63-68. doi: 10.5387/fms.24-00013. Epub 2024 Dec 7.

Abstract

Myositis-specific autoantibodies play an important role on the disease phenotype of idiopathic inflammatory myopathies (IIMs). Anti-signal recognition particle (SRP) antibody-positive patients with IIMs may present with severe myopathy and highly elevated serum creatine kinase levels. These patients are often resistant to immunosuppressive therapy, but there is no established treatment strategy. A 51-year-old man referred to our department was diagnosed with IIM based on imaging and pathological findings. A high dose of corticosteroids followed by intravenous cyclophosphamide (IV-CY) treatment (750 mg three times) resulted in an improvement in clinical manifestations and functional outcomes, and recurrence did not occur. Our case suggests that IV-CY is an effective induction regimen for patients with anti-SRP antibody-positive IIMs.

摘要

肌炎特异性自身抗体在特发性炎性肌病(IIMs)的疾病表型中起重要作用。抗信号识别颗粒(SRP)抗体阳性的IIMs患者可能表现为严重肌病和血清肌酸激酶水平显著升高。这些患者通常对免疫抑制治疗耐药,但尚无既定的治疗策略。一名转诊至我科的51岁男性患者,根据影像学和病理检查结果被诊断为IIMs。大剂量皮质类固醇随后静脉注射环磷酰胺(IV-CY)治疗(750mg,共3次)使临床表现和功能预后得到改善,且未出现复发。我们的病例提示,IV-CY是抗SRP抗体阳性IIMs患者有效的诱导治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a2a/11799667/8b0abbaafdb1/2185-4610-71-063-g001.jpg

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