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Serum levels of anti-SRP54 antibodies reflect disease activity of necrotizing myopathy in a child treated effectively with combinatorial methylprednisolone pulses and plasma exchanges followed by intravenous cyclophosphamide

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Modern Rheumatology

Abstract

We describe the effectiveness of combinatorial therapy with plasma exchanges and methylprednisolone pulses followed by intravenous cyclophosphamide in a young girl with anti-signal recognition particle 54 (SRP54) antibody-associated myopathy. We also use a newly described quantitative assay to demonstrate the close association between the titers of anti-SRP54 antibodies and disease activity. This is the first report of a pediatric patient indicating that the serum levels of anti-SRP54 antibodies are also beneficial for monitoring the disease activity of progressive necrotizing myopathy.

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References

  1. Brouwer R, Hengstman GJ, Vree Egberts W, Ehrfeld H, Bozic B, Ghirardello A, Grøndal G, Hietarinta M, Isenberg D, Kalden JR, Lundberg I, Moutsopoulos H, Roux-Lombard P, Vencovsky J, Wikman A, Seelig HP, van Engelen BG, van Venrooij WJ. Autoantibody profiles in the sera of European patients with myositis. Ann Rheum Dis. 2001;60:116–23.

    Article  PubMed  CAS  Google Scholar 

  2. Kao AH, Lacomis D, Lucas M, Fertig N, Oddis CV. Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy. Arthritis Rheum. 2004;50:209–15.

    Article  PubMed  CAS  Google Scholar 

  3. Rider LG, Miller FW. Classification and treatment of the juvenile idiopathic inflammatory myopathies. Rheum Dis Clin North Am. 1997;23:619–55.

    Article  PubMed  CAS  Google Scholar 

  4. Wedderburn LR, McHugh NJ, Chinoy H, et al. HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis–scleroderma overlap. Rheumatology. 2007;46:1786–91.

    Article  PubMed  CAS  Google Scholar 

  5. Love LA, Leff RL, Fraser DD, et al. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine. 1990;70:360–74.

    Google Scholar 

  6. Targoff IN, Johnson AE, Miller FW. Antibody to signal recognition particle in polymyositis. Arthritis Rheum. 1990;33:1361–70.

    Article  PubMed  CAS  Google Scholar 

  7. Kawabata T, Komaki H, Saito T, Saito Y, Nakagawa E, Sugai K, Sasaki M, Hayashi YK, Nishino I, Momomura M, Kizawa T, Imagawa T, Yokota S. A pediatric patient with myopathy associated with antibodies to a signal recognition particle. Brain Dev. 2012; 34(10):877–80.

    Google Scholar 

  8. Benveniste O, Drouot L, Jouen F, Charuel J, Bloch-Queyrat C, Behin A, Amoura Z, Marie I, Guiguet M, Eymard B, Gilbert D, Tron F, Herson S, Musset L, Boyer O. Correlation of anti-signal recognition particle autoantibody levels with creatine kinase activity in patients with necrotizing myopathy. Arthritis Rheum. 2011;63(7):1961–71.

    Article  PubMed  CAS  Google Scholar 

  9. Arlet JB, Dimitri D, Pagnoux C, Boyer O, Maisonobe T, Authier FJ, Bloch-Queyrat C, Goulvestre C, Heshmati F, Atassi M, Guillevin L, Herson S, Benveniste O, Mouthon L. Marked efficacy of a therapeutic strategy associating prednisone and plasma exchange followed by rituximab in two patients with refractory myopathy associated with antibodies to the signal recognition particle (SRP). Neuromuscul Disord. 2006;16:334–6.

    Article  PubMed  Google Scholar 

  10. Gherardi RK. Pathogenic aspects of dermatomyositis, polymyositis and overlap myositis. Presse Med. 2011;40(4 Pt 2):e209–18.

    Article  PubMed  Google Scholar 

  11. Brewer EJ Jr, Giannini EH, Rossen RD, et al. Plasma exchange therapy of a childhood onset dermatomyositis patient. Arthritis Rheum. 1980;23:509–13.

    Article  PubMed  Google Scholar 

  12. Anderson L, Ziter FA. Plasmapheresis via central catheter in dermatomyositis: a new method for selected pediatric patients. J Pediatr. 1981;98:240–1.

    Article  PubMed  CAS  Google Scholar 

  13. Miller FW, Leitman SF, Cronin ME, Hicks JE, Leff RL, Wesley R, Fraser DD, Dalakas M, Plotz PH. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med. 1992;326(21):1380–4.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

This work was supported in part by a grant from the Association Française Contre les Myopathies.

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Correspondence to Takako Miyamae.

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Momomura, M., Miyamae, T., Nozawa, T. et al. Serum levels of anti-SRP54 antibodies reflect disease activity of necrotizing myopathy in a child treated effectively with combinatorial methylprednisolone pulses and plasma exchanges followed by intravenous cyclophosphamide. Mod Rheumatol (2012). https://doi.org/10.1007/s10165-012-0792-9

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  • DOI: https://doi.org/10.1007/s10165-012-0792-9

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