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Heterogeneous clinical spectrum of anti-SRP myositis and importance of the methods of detection of anti-SRP autoantibodies: a multicentric study

Immunologic Research volume 64, pages 677–686 (2016)Cite this article

Abstract

Anti-signal recognition particle (SRP) antibodies are important serological markers for the diagnosis and the prognosis of idiopathic inflammatory myopathy (IIM), especially to distinguish immune-mediated necrotizing myopathy (IMNM). This study was set up to investigate the phenotype associated with anti-SRP antibodies and to evaluate the methods for detecting these antibodies. Clinical and biological data were retrospectively obtained from 60 adult patients with anti-SRP antibodies detected by a dot immunoassay from 12 centers. Thirty-six (60 %) out of these 60 patients suffered from an IIM, and among them, 21 patients were diagnosed as IMNM. Among patients with a definite IIM, proximal weakness and myalgia were prominent symptoms at the time of diagnosis. Only few patients displayed severe extra-muscular symptoms such as cardiac involvement or severe myositis. Mean creatine kinase levels were high for all patients except for two of them. When testing by indirect immunofluorescence (IIF) on HEp2 cells, the fraction of patients displaying the typical anti-SRP fine speckled staining of the cytoplasm was higher in patients with IIM (30/36) (83 %) than in patients with non-IIM (3/24) (12.5 %) (p < 0.0001). Thirty (91 %) out of 33 patients with a positive immunodot and a characteristic IIF cytoplasmic staining suffered from a clinical definite myositis, whereas only 6 (22 %) out of 27 patients with a positive immunodot but a negative cytoplasmic pattern suffered from a myositis (p < 0.00001). This series highlights the strong heterogeneity of anti-SRP positivity that encompassed IMNM and non-IMNM and supports the necessity of considering both IIF and dot immunoassay to confirm the diagnosis of anti-SRP-associated myositis.

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Acknowledgments

We gratefully acknowledge the patients and the contributions made by the clinicians of all hospitals for the clinical data and especially: Pr JF Cordier JF, Pr. V. Cottin, Dr C. Khouatra, Dr J. Traclet, Pr J. Tebib, Pr J. Ninet, Dr G. Devouassoux, Dr B. Coppéré, Dr H. Gervais-Bernard, Dr E. Monard; Hospices Civils de Lyon; Dr C. Doche, CHG Chambéry, France; Pr J. Authier, neurology department, hôpital Henri Mondor, APHP, Créteil, France; Dr Steven Vanderschueren Catholic University of Leuven, University Hospitals Leuven; Belgique. We acknowledge Immunobiotec, CRB, HCL Lyon BB003300046.

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    Affiliations

    1. Department of Immunology, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, chemin du grand revoyet, 69495, Pierre-Benite Cedex, France

      Cécile Picard & Nicole Fabien

    2. Groupe d’Etude de l’Auto-Immunité (GEAI), 49933, Angers, France

      Thierry Vincent, Sophie Hue, Françoise Fortenfant, Daniela Lakomy, René-Louis Humbel, Joelle Goetz, Nathalie Bardin, Catherine Johanet, Pascale Chretien, Sylvain Dubucquoi, Sophie Desplat-Jégo, Xavier Bossuyt, Jean Sibilia, Isabelle Abreu, Alain Chevailler & Nicole Fabien

    3. Department of Immunology, St Eloi Hospital, Montpellier University, CHRU de Montpellier, 34295, Montpellier Cedex 5, France

      Thierry Vincent

    4. Department of Internal and Vascular Medicine, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Université Lyon-1, 69495, Pierre-Benite Cedex, France

      Jean-Christophe Lega

    5. Department of Immunology, CHU Henri Mondor - Service d’Immunologie Biologique, 94010, Créteil, France

      Sophie Hue

    6. Department of Immunology, Hôpital Rangueil, 31059, Toulouse, Cedex 9, France

      Françoise Fortenfant

    7. Department of Immunology, CHU Dijon, 21079, Dijon, Cedex, France

      Daniela Lakomy

    8. Laboratoire Luxembourgeois d’Immuno-Pathologie, 4149, Esch-Suralzette, Luxembourg

      René-Louis Humbel

    9. Department of Immunology, Nouvel Hôpital Civil, 67091, Strasbourg, France

      Joelle Goetz

    10. Department of Medical Information, CHU de Montpellier, UMR Mistea, 34295, Montpellier, France

      Nicolas Molinari

    11. Department of Biological Immunology, UMR-S 1076, Faculté de Pharmacie, LBM AP-HM et Aix-Marseille-Université, 13005, Marseille, France

      Nathalie Bardin

    12. Department of Biological Immunology, UMR CNRS/AMU 7259, LBM AP-HM et Aix-Marseille-Université, 13005, Marseille, France

      Daniel Bertin & Sophie Desplat-Jégo

    13. Department of Immunology, AP-HP hôpital Saint-Antoine, UFR 967, Faculté de medecine, Université Pierre et Marie Curie, 75571, Paris Cedex 12, France

      Catherine Johanet

    14. Department of Immunology, CHU Bicetre, 94270, Kremlin Bicêtre, France

      Pascale Chretien

    15. Department of Immunology, Univ Lille Nord de France, 59000, Lille, France

      Sylvain Dubucquoi

    16. Hôpital Cardiovasculaire et pneumologique, Hospices Civils de Lyon, 69677, Bron, France

      Nathalie Streichenberger

    17. Department of Microbiology and Immunology, Catholic University of Leuven, Louvain, Belgium

      Xavier Bossuyt

    18. Department of Laboratory Medicine, University Hospitals Leuven, Louvain, Belgium

      Xavier Bossuyt

    19. Department of Rheumatology, Centre national de référence des maladies auto-immunes systémiques rares, CHU Hautepierre, Université de Strasbourg, 67098, Strasbourg, France

      Jean Sibilia

    20. Departamento Universitário de Imunologia, Universidade Nova de Lisboa - Faculdade de Ciências Médicas, 1169-056, Lisbon, Portugal

      Isabelle Abreu

    21. Department of Immunology and Allergology, CHU Hôpital Larrey, Institut de Biologie en Santé, 49933, Angers Cedex 9, France

      Alain Chevailler

    Authors
    1. Cécile Picard
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    21. Nicole Fabien
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    Correspondence to Nicole Fabien.

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    Thierry Vincent and Jean-Christophe Lega have contributed equally to this work.

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    Picard, C., Vincent, T., Lega, JC. et al. Heterogeneous clinical spectrum of anti-SRP myositis and importance of the methods of detection of anti-SRP autoantibodies: a multicentric study. Immunol Res 64, 677–686 (2016). https://doi.org/10.1007/s12026-015-8774-6

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    Keywords

    • Anti-SRP antibodies
    • Immune-mediated necrotizing myopathy
    • Immunodot
    • Indirect immunofluorescence