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.
Abstract/References
Successful treatment of anti-signal recognition particle antibody-positive myositis with intravenous cyclophosphamide: A case report
Jumpei Temmoku, Shuhei Yoshida, Kanae Tsuchihashi, Yuya Sumichika, Kenji Saito, Haruki Matsumoto, Yuya Fujita, Naoki Matsuoka, Tomoyuki Asano, Nozomu Matsuda, Shuzo Sato, Kiyoshi Migita
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Jumpei Temmoku
Department of Rheumatology, Fukushima Medical University School of Medicine
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Shuhei Yoshida
Department of Rheumatology, Fukushima Medical University School of Medicine
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Kanae Tsuchihashi
Department of Neurology, Fukushima Medical University School of Medicine
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Yuya Sumichika
Department of Rheumatology, Fukushima Medical University School of Medicine
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Kenji Saito
Department of Rheumatology, Fukushima Medical University School of Medicine
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Haruki Matsumoto
Department of Rheumatology, Fukushima Medical University School of Medicine
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Yuya Fujita
Department of Rheumatology, Fukushima Medical University School of Medicine
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Naoki Matsuoka
Department of Rheumatology, Fukushima Medical University School of Medicine
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Tomoyuki Asano
Department of Rheumatology, Fukushima Medical University School of Medicine
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Nozomu Matsuda
Department of Neurology, Fukushima Medical University School of Medicine
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Shuzo Sato
Department of Rheumatology, Fukushima Medical University School of Medicine
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Kiyoshi Migita
Department of Rheumatology, Fukushima Medical University School of Medicine
Abstract
References
2. Malik A, Hayat G, Kalia JS, Guzman MA. Idiopathic Inflammatory Myopathies:Clinical Approach and Management. Front Neurol, 7:64, 2016.
11. Malmström V, Venalis P, Albrecht I. T cells in myositis. Arthritis Res Ther, 14:230, 2012.
12. Aggarwal R, Oddis CV, Goudeau D, et al. Autoantibody levels in myositis patients correlate with clinical response during B cell depletion with rituximab. Rheumatology (Oxford), 55:991-999, 2016.
15. Zhen C, Hou Y, Zhao B, Ma X, Dai T, Yan C. Efficacy and safety of rituximab treatment in patients with idiopathic inflammatory myopathies:A systematic review and meta-analysis. Front Immunol, 13:1051609, 2022.
16. Pinal-Fernandez I, Parks C, Werner JL, et al. Longitudinal Course of Disease in a Large Cohort of Myositis Patients With Autoantibodies Recognizing the Signal Recognition Particle. Arthritis Care Res (Hoboken), 69:263-270, 2017.
21. Ysermans R, Busch MH, Aendekerk JP, Damoiseaux J, van Paassen P. Adding low dose cyclophosphamide to rituximab for remission-induction may prolong relapse-free survival in patients with ANCA vasculitis:A retrospective study. J Transl Autoimmun, 6:100178, 2023.
22. Mecoli CA, Lahouti AH, Brodsky RA, Mammen AL, Christopher-Stine L. High-dose cyclophosphamide without stem cell rescue in immune-mediated necrotizing myopathies. Neurol Neuroimmunol Neuroinflamm, 4:e381, 2017.