RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis

Background: Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a...

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Autores: Suarez-Calvet, X, Gallardo, E, Pinal-Fernandez, I, De Luna, N, Lleixa, C, Diaz-Manera, J, Rojas-Garcia, R, Castellvi, I, Martinez, MA, Grau, JM, Selva-O'Callaghan, A, Illa, I
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2017
País:España
Institución:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
Repositorio:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
OAI Identifier:oai:iibsantpau.fundanetsuite.com:p14192
Acceso en línea:https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=14192
Access Level:acceso abierto
Palabra clave:Inflammatory myopathies
Dermatomyositis
Biomarker
Muscle biopsy
Perifascicular atrophy
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spelling RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositisSuarez-Calvet, XGallardo, EPinal-Fernandez, IDe Luna, NLleixa, CDiaz-Manera, JRojas-Garcia, RCastellvi, IMartinez, MAGrau, JMSelva-O'Callaghan, AIlla, IInflammatory myopathiesDermatomyositisBiomarkerMuscle biopsyPerifascicular atrophyBackground: Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a hallmark of DM. However, perifascicular atrophy is not observed in all patients with DM and, conversely, perifascicular atrophy can be observed in other myositis such as antisynthetase syndrome (ASS), complicating DM diagnosis. Retinoic acid inducible-gene I (RIG-I), a receptor of innate immunity that promotes type I interferon, was observed in perifascicular areas in DM. We compared the value of RIG-I expression with perifascicular atrophy as a biomarker of DM. Methods: We studied by immunohistochemical analysis the expression of RIG-I and the presence of perifascicular atrophy in 115 coded muscle biopsies: 44 patients with DM, 18 with myositis with overlap, 8 with ASS, 27 with non-DM inflammatory myopathy (16 with polymyositis, 6 with inclusion body myositis, 5 with immune-mediated necrotizing myopathy), 8 with muscular dystrophy (4 with dysferlinopathy, 4 with fascioscapulohumeral muscle dystrophy) and 10 healthy controls. Results: We found RIG-I-positive fibers in 50% of DM samples vs 11% in non-DM samples (p < 0.001). Interestingly, RIG-I staining identified 32% of DM patients without perifascicular atrophy (p = 0.007). RIG-I sensitivity was higher than perifascicular atrophy (p < 0.001). No differences in specificity between perifascicular atrophy and RIG-I staining were found (92% vs 88%). RIG-I staining was more reproducible than perifascicular atrophy (K coefficient 0.52 vs 0.37). Conclusions: The perifascicular pattern of RIG-I expression supports the diagnosis of DM. Of importance for clinical and therapeutic studies, the inclusion of RIG-I in the routine pathological staining of samples in inflammatory myopathy will allow us to gather more homogeneous subgroups of patients in terms of immunopathogenesis.BMC2017info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=14192ARTHRITIS RESEARCH & THERAPYISSN: 14786354ISSNe: 14786362reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pauinstname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)Inglésinfo:eu-repo/semantics/openAccessoai:iibsantpau.fundanetsuite.com:p141922026-06-14T12:41:47Z
dc.title.none.fl_str_mv RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
title RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
spellingShingle RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
Suarez-Calvet, X
Inflammatory myopathies
Dermatomyositis
Biomarker
Muscle biopsy
Perifascicular atrophy
title_short RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
title_full RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
title_fullStr RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
title_full_unstemmed RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
title_sort RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
dc.creator.none.fl_str_mv Suarez-Calvet, X
Gallardo, E
Pinal-Fernandez, I
De Luna, N
Lleixa, C
Diaz-Manera, J
Rojas-Garcia, R
Castellvi, I
Martinez, MA
Grau, JM
Selva-O'Callaghan, A
Illa, I
author Suarez-Calvet, X
author_facet Suarez-Calvet, X
Gallardo, E
Pinal-Fernandez, I
De Luna, N
Lleixa, C
Diaz-Manera, J
Rojas-Garcia, R
Castellvi, I
Martinez, MA
Grau, JM
Selva-O'Callaghan, A
Illa, I
author_role author
author2 Gallardo, E
Pinal-Fernandez, I
De Luna, N
Lleixa, C
Diaz-Manera, J
Rojas-Garcia, R
Castellvi, I
Martinez, MA
Grau, JM
Selva-O'Callaghan, A
Illa, I
author2_role author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Inflammatory myopathies
Dermatomyositis
Biomarker
Muscle biopsy
Perifascicular atrophy
topic Inflammatory myopathies
Dermatomyositis
Biomarker
Muscle biopsy
Perifascicular atrophy
description Background: Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a hallmark of DM. However, perifascicular atrophy is not observed in all patients with DM and, conversely, perifascicular atrophy can be observed in other myositis such as antisynthetase syndrome (ASS), complicating DM diagnosis. Retinoic acid inducible-gene I (RIG-I), a receptor of innate immunity that promotes type I interferon, was observed in perifascicular areas in DM. We compared the value of RIG-I expression with perifascicular atrophy as a biomarker of DM. Methods: We studied by immunohistochemical analysis the expression of RIG-I and the presence of perifascicular atrophy in 115 coded muscle biopsies: 44 patients with DM, 18 with myositis with overlap, 8 with ASS, 27 with non-DM inflammatory myopathy (16 with polymyositis, 6 with inclusion body myositis, 5 with immune-mediated necrotizing myopathy), 8 with muscular dystrophy (4 with dysferlinopathy, 4 with fascioscapulohumeral muscle dystrophy) and 10 healthy controls. Results: We found RIG-I-positive fibers in 50% of DM samples vs 11% in non-DM samples (p < 0.001). Interestingly, RIG-I staining identified 32% of DM patients without perifascicular atrophy (p = 0.007). RIG-I sensitivity was higher than perifascicular atrophy (p < 0.001). No differences in specificity between perifascicular atrophy and RIG-I staining were found (92% vs 88%). RIG-I staining was more reproducible than perifascicular atrophy (K coefficient 0.52 vs 0.37). Conclusions: The perifascicular pattern of RIG-I expression supports the diagnosis of DM. Of importance for clinical and therapeutic studies, the inclusion of RIG-I in the routine pathological staining of samples in inflammatory myopathy will allow us to gather more homogeneous subgroups of patients in terms of immunopathogenesis.
publishDate 2017
dc.date.none.fl_str_mv 2017
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=14192
url https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=14192
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv BMC
publisher.none.fl_str_mv BMC
dc.source.none.fl_str_mv ARTHRITIS RESEARCH & THERAPY
ISSN: 14786354
ISSNe: 14786362
reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
instname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
instname_str Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
reponame_str r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
collection r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
repository.name.fl_str_mv
repository.mail.fl_str_mv
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