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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

Cécile Picard 1, 2 Thierry Vincent Jean-Christophe Lega 3 Sophie Hue 4 Françoise Fortenfant Daniel Lakomy 5 René-Louis Humbel Joelle Goetz Nicolas Molinari 6 Nathalie Bardin 7, 8 Daniel Bertin 7 Catherine Johanet 9 Pascale Chretien Sylvain Dubucquoi 10 Nathalie Streichenberger 3 Sophie Desplat-Jégo 11 Xavier Bossuyt Jean Sibilia 12 Isabelle Abreu Alain Chevailler 13 Nicole Fabien 3
1 EFS - Alpes-Méditerranée - Etablissement Français du Sang - Alpes-Méditerranée
2 ADES - Anthropologie bio-culturelle, Droit, Ethique et Santé
3 HCL - Hospices Civils de Lyon
4 Service d'immunologie biologique
5 CHU Dijon
6 IMAG - Institut Montpelliérain Alexander Grothendieck
7 Laboratoire d'Immunologie [Hôpital de la Conception - APHM]
8 VRCM - Vascular research center of Marseille
9 Physiopathologie et traitement des maladies du foie
10 CHU Bicêtre
11 NICN - Neurobiologie des interactions cellulaires et neurophysiopathologie - NICN
12 Physiopathologie des arthrites
13 CHU Angers - Centre Hospitalier Universitaire d'Angers
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.
Keywords : Immunodot Indirect immunofluorescence Anti-SRP antibodies Immune-mediated necrotizing myopathy
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Journal articles
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https://hal-amu.archives-ouvertes.fr/hal-01455442
Contributor : Françoise Dignat-George <>
Submitted on : Friday, February 3, 2017 - 3:25:36 PM
Last modification on : Wednesday, October 14, 2020 - 4:08:27 AM

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UNIV-AMU | CNRS | INRA | HCL | I3M_UMR5149 | UNAM | UPEC-UPEM | IMAG-MONTPELLIER | MIPS | UNIV-MONTPELLIER | INP | INP-NISP | ANTHROPO-MARSEILLE | UPEC | INRAE | SITE-ALSACE

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Cécile Picard, Thierry Vincent, Jean-Christophe Lega, Sophie Hue, Françoise Fortenfant, et al.. Heterogeneous clinical spectrum of anti-SRP myositis and importance of the methods of detection of anti-SRP autoantibodies: a multicentric study. Immunologic Research, Humana Press, 2016, 64 (3), pp.677-686. ⟨10.1007/s12026-015-8774-6⟩. ⟨hal-01455442⟩

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