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

Author information
  • Jumpei Temmoku

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Shuhei Yoshida

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Kanae Tsuchihashi

    Department of Neurology, Fukushima Medical University School of Medicine

  • Yuya Sumichika

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Kenji Saito

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Haruki Matsumoto

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Yuya Fujita

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Naoki Matsuoka

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Tomoyuki Asano

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Nozomu Matsuda

    Department of Neurology, Fukushima Medical University School of Medicine

  • Shuzo Sato

    Department of Rheumatology, Fukushima Medical University School of Medicine

  • Kiyoshi Migita

    Department of Rheumatology, Fukushima Medical University School of Medicine

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.

The cintent of reseach paper

References

1. Ashton C, Paramalingam S, Stevenson B, Brusch A, Needham M. Idiopathic inflammatory myopathies:a review. Intern Med J, 51:845-852, 2021.


2. Malik A, Hayat G, Kalia JS, Guzman MA. Idiopathic Inflammatory Myopathies:Clinical Approach and Management. Front Neurol, 7:64, 2016.


3. Corona-Sanchez EG, Martínez-García EA, Lujano-Benítez AV, et al. Autoantibodies in the pathogenesis of idiopathic inflammatory myopathies:Does the endoplasmic reticulum stress response have a role? Front Immunol, 13:940122, 2022.


4. Teel A, Lu J, Park J, Singh N, Basharat P. The Role of Myositis-Specific Autoantibodies and the Management of Interstitial Lung Disease in Idiopathic Inflammatory Myopathies:A Systematic Review. Semin Arthritis Rheum, 57:152088, 2022.


5. Ma X, Bu BT. Anti-SRP immune-mediated necrotizing myopathy:A critical review of current concepts. Front Immunol, 13:1019972, 2022.


6. Valiyil R, Casciola-Rosen L, Hong G, Mammen A, Christopher-Stine L. Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies:a case series. Arthritis Care Res (Hoboken), 62:1328-1334, 2010.


7. Pinal-Fernandez I, Mammen AL. Spectrum of immune-mediated necrotizing myopathies and their treatments. Curr Opin Rheumatol, 28:619-624, 2016.


8. Suzuki S, Nishikawa A, Kuwana M, et al. Inflammatory myopathy with anti-signal recognition particle antibodies:case series of 100 patients. Orphanet J Rare Dis, 10:61, 2015.


9. Allenbach Y, Mammen AL, Benveniste O, Stenzel W. 224th ENMC International Workshop::Clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, The Netherlands, 14-16 October 2016. Neuromuscul Disord, 28:87-99, 2018.


10. Ascherman DP, Oriss TB, Oddis CV, Wright TM. Critical requirement for professional APCs in eliciting T cell responses to novel fragments of histidyl-tRNA synthetase (Jo-1) in Jo-1 antibody-positive polymyositis. J Immunol, 169:7127-7134, 2002.


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.


13. Carstens PO, Müllar LM, Wrede A, et al. Skeletal muscle fibers produce B-cell stimulatory factors in chronic myositis. Front Immunol, 14:1177721, 2023.


14. Fasano S, Gordon P, Hajji R, Loyo E, Isenberg DA. Rituximab in the treatment of inflammatory myopathies:a review. Rheumatology (Oxford), 56:26-36, 2017.


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.


17. Brummaier T, Pohanka E, Studnicka-Benke A, Pieringer H. Using cyclophosphamide in inflammatory rheumatic diseases. Eur J Intern Med, 24:590-596, 2013.


18. Maher TM, Tudor VA, Saunders P, et al. Rituximab versus intravenous cyclophosphamide in patients with connective tissue disease-associated interstitial lung disease in the UK (RECITAL):a double-blind, double-dummy, randomised, controlled, phase 2b trial. Lancet Respir Med, 11:45-54, 2023.


19. Moreno-Torres V, Martín-Iglesias D, Vivero F, et al. Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies. Semin Arthritis Rheum, 59:152164, 2023.


20. Quan XY, Chen HT, Liang SQ, et al. Revisited Cyclophosphamide in the Treatment of Lupus Nephritis. Biomed Res Int, 2022:8345737, 2022.


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.

Figures