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Immunol Res. 2016 Jun;64(3):677-86. doi: 10.1007/s12026-015-8774-6.

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

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Department of Immunology, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, chemin du grand revoyet, 69495, Pierre-Benite Cedex, France.
Groupe d'Etude de l'Auto-Immunité (GEAI), 49933, Angers, France.
Department of Immunology, St Eloi Hospital, Montpellier University, CHRU de Montpellier, 34295, Montpellier Cedex 5, France.
Department of Internal and Vascular Medicine, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Université Lyon-1, 69495, Pierre-Benite Cedex, France.
Department of Immunology, CHU Henri Mondor - Service d'Immunologie Biologique, 94010, Créteil, France.
Department of Immunology, Hôpital Rangueil, 31059, Toulouse, Cedex 9, France.
Department of Immunology, CHU Dijon, 21079, Dijon, Cedex, France.
Laboratoire Luxembourgeois d'Immuno-Pathologie, 4149, Esch-Suralzette, Luxembourg.
Department of Immunology, Nouvel Hôpital Civil, 67091, Strasbourg, France.
Department of Medical Information, CHU de Montpellier, UMR Mistea, 34295, Montpellier, France.
Department of Biological Immunology, UMR-S 1076, Faculté de Pharmacie, LBM AP-HM et Aix-Marseille-Université, 13005, Marseille, France.
Department of Biological Immunology, UMR CNRS/AMU 7259, LBM AP-HM et Aix-Marseille-Université, 13005, Marseille, France.
Department of Immunology, AP-HP hôpital Saint-Antoine, UFR 967, Faculté de medecine, Université Pierre et Marie Curie, 75571, Paris Cedex 12, France.
Department of Immunology, CHU Bicetre, 94270, Kremlin Bicêtre, France.
Department of Immunology, Univ Lille Nord de France, 59000, Lille, France.
Hôpital Cardiovasculaire et pneumologique, Hospices Civils de Lyon, 69677, Bron, France.
Department of Microbiology and Immunology, Catholic University of Leuven, Louvain, Belgium.
Department of Laboratory Medicine, University Hospitals Leuven, Louvain, Belgium.
Department of Rheumatology, Centre national de référence des maladies auto-immunes systémiques rares, CHU Hautepierre, Université de Strasbourg, 67098, Strasbourg, France.
Departamento Universitário de Imunologia, Universidade Nova de Lisboa - Faculdade de Ciências Médicas, 1169-056, Lisbon, Portugal.
Department of Immunology and Allergology, CHU Hôpital Larrey, Institut de Biologie en Santé, 49933, Angers Cedex 9, France.
Department of Immunology, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, chemin du grand revoyet, 69495, Pierre-Benite Cedex, France.
Groupe d'Etude de l'Auto-Immunité (GEAI), 49933, Angers, France.


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.


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

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