Vol. 12. Núm. 29.
Páginas 1679-1689 (Abril 2017)

Actualización
Miopatías inflamatorias

Inflammatory myopathy

A. Selva O’CallaghanabE. Trallero AraguásacM. Labrador HorrilloadJ.M. Grau Junyente
Doi : https://10.1016/j.med.2017.03.001
Opciones
Resumen
Introducción

Las miopatías inflamatorias constituyen un grupo heterogéneo de enfermedades musculares adquiridas caracterizadas por la presencia de un infiltrado inflamatorio en la biopsia muscular. Actualmente se reconocen al menos 4 principales fenotipos clínicos que incluyen la dermatomiositis, polimiositis, miositis con cuerpos de inclusión y miopatía necrosante inmunomediada.

 Patogenia

Se consideran enfermedades sistémicas de probable origen autoinmune, detectándose en la biopsia muscular un infiltrado de linfocitos B y T CD4+ con depósito endotelial del complejo de ataque de membrana C5b9 en la dermatomiositis, el complejo linfocitos T CD8+-MHC-I en la polimiositis y miositis con cuerpos de inclusión y vacuolas ribeteadas con cuerpos de inclusión en esta última entidad. La necrosis muscular, la presencia de macrófagos y la expresión de MHC-I en las células musculares caracteriza a la miositis necrosante inmunomediada.

 Diagnóstico y tratamiento

La clínica, la biopsia muscular y el perfil inmunológico son herramientas útiles para alcanzar un diagnóstico correcto. El tratamiento inmunodepresor, las inmunoglobulinas intravenosas y las terapias biológicas, junto a la rehabilitación física constituyen el arsenal terapéutico para tratar estas enfermedades.

Palabras clave
Miopatía inflamatoria
Dermatomiositis
Polimiositis
Miositis con cuerpos de inclusión
Miopatía necrosante inmunomediada
Abstract
Introduction

Inflammatory myopathies are a heterogeneous group of acquired muscular disorders characterised by the presence of an inflammatory infiltrate in the muscle biopsy. Currently there are 4 main recognised clinical phenotypes, including dermatomyositis, polymyositis, inclusion body myositis and immune-mediated necrotising myopathy.

 Etiopathogenesis

They are considered systemic diseases of apparent autoimmune origin, where the muscle biopsy detects infiltrating B and CD4+ T lymphocytes with endothelial deposit of C5b-9 membrane attack complex in dermatomyositis, CD8+ T cells/MHC-I complex in polymyositis and inclusion body myositis and rimmed vacuoles in inclusion body myositis. Muscle necrosis, the presence of macrophages and the MHC-I expression in muscle cells characterise immune-mediated necrotising myositis.

 Diagnosis and treatment

Symptoms, muscle biopsy and immune profile are helpful tools to reach a correct diagnosis. Immunosuppressive therapies, intravenous immunoglobulins and biological agents, together with physical rehabilitation constitute the therapeutic arsenal to treat these conditions.

Keywords
Inflammatory myopathy
Dermatomyositis
Polymyositis
Inclusion body myositis
Immune-mediated necrotising myopathy

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Bibliografía
[1]
201703191219326088
201703191219326089
M.C. Dalakas.
Inflammatory muscle diseases.
N Engl J Med., (2015), 372
http://dx.doi.org/10.1056/NEJMoa1411480 | Medline
1734-47
[2]
2017031912193260810
H. Vargas-Leguás, A. Selva-O’Callaghan, M. Campins-Martí, E. Hermosilla Pérez, J.M. Grau-Junyent, X. Martínez Gómez, et al.
[Polymyositis-dermatomyositis: incidence in Spain (1997-2004)].
Med Clin (Barc)., (2007), 129
721-4
[3]
2017031912193260811
2017031912193260812
A. Bohan, J.B. Peter.
Polymyositis and dermatomyositis: parts 1 and 2.
N Engl J Med., (1975), 292
Medline
344-7; 403-407
[4]
2017031912193260813
M.C. Dalakas.
Polymyositis, dermatomyositis and inclusion-body myositis.
N Engl J Med., (1991), 325
1487-98
[5]
2017031912193260814
P. Basharat, L. Christopher-Stine.
Immune-mediated necrotizing myopathy: update on diagnosis and management.
Curr Rheumatol Rep., (2015), 17 pp. 72
http://dx.doi.org/10.1007/s11926-015-0548-6 | Medline
[6]
2017031912193260815
A.L. Mammen.
Necrotizing myopathies: beyond statins.
Curr Opin Rheumatol., (2014), 26
679-83
[7]
2017031912193260816
2017031912193260817
I.E. Lundberg, F.W. Miller, A. Tjärnlund, M. Bottai.
Diagnosis and classification of idiopathic inflammatory myopathies.
J Intern Med., (2016), 280
39-51
[8]
2017031912193260818
S. Okada, E. Weatherhead, I.N. Targoff, R. Wesley, F.W. Miller, International Myositis Collaborative Study Group.
Global surface ultraviolet radiation intensity may modulate the clinical and immunologic expression of autoinmune muscle disease.
Arthritis Rheum., (2003), 48
2285-93
[9]
2017031912193260819
L. Gan, F.W. Miller.
State of the art: what we know about infectious agents and myositis.
Curr Opin Rheumatol., (2011), 23
585-94
[10]
A. Selva-O’Callaghan, J.M. Tura, J.M. Grau-Junyent, M. Labrador-Horrillo, R. Solans-Laque, M. Vilardell-Tarrés.
Silicone gel filled breast implants and dermatomyositis.
Clin Exp Rheumatol., (2004), 22 pp. 376
Medline
[11]
A. Labirua-Iturburu, A. Selva-O’Callaghan, J.P. Zock, R. Orriols, X. Martínez-Gómez, M. Vilardell-Tarrés.
Occupational exposure in patients with the antisynthetase syndrome.
Clin Rheumatol., (2014), 33
221-5
[12]
2017031912193260820
A. Selva-O’Callaghan, M. Alvarado-Cardenas, A. Marin, I. Pinal-Fernández.
Statins and myositis: the role of anti-HMGCR antibodies.
Expert Rev Clin Immunol., (2015), 11
1277-9
[13]
2017031912193260821
A. Selva-O’Callaghan, E. Trallero-Araguás, J.M. Grau-Junyent, M. Labrador-Horrillo.
Malignancy and myositis: novel autoantibodies and new insights.
Curr Opin Rheumatol., (2010), 22
627-32
[14]
F.W. Miller, W. Chen, T.P. O’Hanlon, R.G. Cooper, J. Vencovsky, L.G. Rider, Myositis Genetics Consortium, et al.
Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes.
Genes Immun., (2015), 16
470-80
[15]
S. Rothwell, R.G. Cooper, I.E. Lundberg, F.W. Miller, P.K. Gregersen, J. Bowes, Myositis Genetics Consortium, et al.
Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups.
Ann Rheum Dis., (2016), 75
1558-66
[16]
2017031912193260822
F.W. Miller, R.G. Cooper, J. Vencovský, L.G. Rider, K. Danko, L.R. Wedderburn, Myositis Genetics Consortium, et al.
Genome-wide association study of dermatomyositis reveals genetic overlap with other autoimmune disorders.
Arthritis Rheum., (2013), 65
3239-47
[17]
M. Catalán-García, G. Garrabou, C. Morén, M. Guitart-Mampel, I. González-Casacuberta, A. Hernando, et al.
BACE-1, PS-1 and sAPPβ levels are increased in plasma from sporadic inclusion body myositis patients: surrogate biomarkers among inflammatory myopathies.
Mol Med., (2015), 21
817-23
[18]
2017031912193260823
A.L. Mammen, T. Chung, L. Christopher-Stine, P. Rosen, A. Rosen, K.R. Doering, et al.
Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy.
Arthritis Rheum., (2011), 63
713-21
[19]
2017031912193260824
A. Selva-O’Callaghan, E. Trallero-Araguás, M.A. Martínez, M. Labrador-Horrillo, I. Pinal-Fernández, J.M. Grau-Junyent, et al.
Inflammatory myopathy: diagnosis and clinical course, specific clinical scenarios and new complementary tools.
Expert Rev Clin Immunol., (2015), 11
737-47
[20]
J.C. Milisenda, P.I. Doti, S. Prieto-González, J.M. Grau.
Dermatomyositis presenting with severe subcutaneous edema: five additional cases and review of the literature.
Semin Arthritis Rheum., (2014), 44
228-33
[21]
2017031912193260825
E. Trallero-Araguás, J.M. Grau-Junyent, A. Labirua-Iturburu, F.J. García-Hernández, M. Monteagudo-Jiménez, G. Fraile-Rodríguez, IIM Study Group and Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI), et al.
Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group.
Semin Arthritis Rheum., (2016), 46
225-31
[22]
2017031912193260826
A. Labirua-Iturburu, E. Trallero Araguás, A. Selva O’Callaghan.
[Anti-synthetase syndrome].
Med Clin (Barc)., (2011), 137 pp. 77-83
http://dx.doi.org/10.1016/j.medcli.2011.02.026 | Medline
[23]
2017031912193260827
J. Tieu, I.E. Lundberg, V. Limaye.
Idiopathic inflammatory myositis.
Best Pract Res Clin Rheumatol., (2016), 30
http://dx.doi.org/10.1016/j.berh.2016.09.003 | Medline
149-68
[24]
K. Dankó, A. Ponyi, T. Constantin, G. Borgulya, G. Szegedi.
Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases.
Medicine (Baltimore)., (2004), 83 pp. 35-42
http://dx.doi.org/10.1097/01.md.0000109755.65914.5e | Medline
[25]
O. Miró, M. Laguno, J.R. Alonso, J. Casademont, C. Herrero, A. Selva, et al.
[Clinical course of idiopa thic inflammatory myopathies: complications, survival and prognostic factors].
Med Clin (Barc)., (1999), 112
521-6
[26]
I.J. Chen, W.P. Tsai, Y.J. Wu, S.F. Luo, H.H. Ho, L.B. Liou, et al.
Infections in polymyositis and dermatomyositis: analysis of 192 cases.
Rheumatology (Oxford)., (2010), 49
2429-37
[27]
2017031912193260828
P. Ungprasert, A. Sanguankeo.
Risk of venous thromboembolism in patients with idiopathic inflammatory myositis: a systematic review and meta-analysis.
Rheumatol Int., (2014), 34
1455-8
[28]
2017031912193260829
J.C. Milisenda, A. Selva-O’Callaghan, J.M. Grau.
The diagnosis and classification of polymyositis.
J Autoimmun., (2014), pp. 48-49
118-21
[29]
V.S. Vilela, S. Prieto-González, J.C. Milisenda, Selva-O. Callaghan A., J.M. Grau.
Polymyositis, a very uncommon isolated disease: clinical and histological re-evaluation after long-term follow-up.
Rheumatol Int., (2015), 35
915-20
[30]
A.A. Amato, R.C. Griggs.
Unicorns, dragons, polymyositis and other mythological beasts.
Neurology., (2003), 61
Medline
288-90
[31]
M.F.G. Van Der Meulen, I.M. Bronner, J.E. Hoogendijk, H. Burger, W.J. van Venrooij, A.E. Voskuyl, et al.
Polymyositis: an overdiagnosed entity.
Neurology., (2003), 61
Medline
316-21
[32]
2017031912193260830
M. Catalán, A. Selva-O’Callaghan, J.M. Grau.
Diagnosis and classification of sporadic inclusion body myositis (sIBM).
Autoimmun Rev., (2014), 13
363-6
[33]
2017031912193260831
R.C. Griggs, V. Askanas, S. DiMauro, A. Engel, G. Karpati, J.R. Mendell, et al.
Inclusion body myositis and myopathies.
Ann Neurol., (1995), 38
705-13
[34]
2017031912193260832
L.A. Love, R.L. Leff, D.D. Fraser, I.N. Targoff, M. Dalakas, P.H. Plotz, et al.
A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups.
Medicine (Baltimore)., (1991), 70
Medline
360-74
[35]
A. Selva-O’Callaghan, M. Labrador-Horrillo, R. Solans-Laque, C.P. Simeon-Aznar, X. Martínez-Gómez, M. Vilardell-Tarrés.
Myositis-specific and myositis-associated antibodies in a series of eighty-eight Mediterranean patients with idiopathic inflammatory myopathy.
Arthritis Rheum., (2006), 55
791-8
[36]
2017031912193260833
H.B. Larman, M. Salajegheh, R. Nazareno, T. Lam, J. Sauld, H. Steen, et al.
Cytosolic 5’-nucleotidase 1A autoimmunity in sporadic inclusion body myositis.
Ann Neurol., (2013), 73
408-18
[37]
2017031912193260834
M. Fujimoto, R. Watanabe, Y. Ishitsuka, N. Okiyama.
Recent advances in dermatomyositis-specific autoantibodies.
Curr Opin Rheumatol. En prensa, (2016),
[38]
M. Labrador-Horrillo, M.A. Martínez, A. Selva-O’Callaghan, E. Trallero-Araguás, J.M. Grau-Junyent, M. Vilardell-Tarrés, et al.
Identification of a novel myositis-associated antibody directed against cortactin.
Autoimmun Rev., (2014), 13
1008-12
[39]
A. Labirua-Iturburu, A. Selva-O’Callaghan, M. Vincze, K. Dankó, J. Vencovsky, B. Fisher, et al.
Anti-PL-7 (anti-threonyl-tRNA synthetase) antisynthetase syndrome: clinical manifestations in a series of patients from a European multicenter study (EUMYONET) and review of the literature.
Medicine (Baltimore)., (2012), 91
http://dx.doi.org/10.1136/sextrans-2014-051514 | Medline
206-11
[40]
M. Labrador-Horrillo, M.A. Martínez, A. Selva-O’Callaghan, E. Trallero-Araguas, E. Balada, M. Vilardell-Tarres, et al.
Anti-MDA5 antibodies in a large Mediterranean population of adults with dermatomyositis.
J Immunol Res., (2014), 2014
http://dx.doi.org/10.1155/2014/394127 | Medline
290797
[41]
2017031912193260835
M. Labrador-Horrillo, M.A. Martínez, A. Selva-O’Callaghan, E. Trallero-Araguás, E. Balada, M. Vilardell-Tarrés, et al.
Anti-TIF1γ antibodies (anti-p155) in adult patients with dermatomyositis: comparison of different diagnostic assays.
Ann Rheum Dis., (2012), 71
993-6
[42]
2017031912193260836
2017031912193260837
E. Trallero-Araguás, Rodrigo-Pendás JÁ, A. Selva-O’Callaghan, X. Martínez-Gómez, X. Bosch, M. Labrador-Horrillo, et al.
Usefulness of anti-p155 autoantibody for diagnosing cancer-associated dermatomyositis: a systematic review and meta-analysis.
Arthritis Rheum., (2012), 64
523-32
[43]
2017031912193260838
A. Selva-O’Callaghan, J.M. Grau, C. Gámez-Cenzano, A. Vidaller-Palacín, X. Martínez-Gómez, E. Trallero-Araguás, et al.
Conventional cancer screening versus PET/CT in dermatomyositis/polymyositis.
Am J Med., (2010), 123
558-62
[44]
A. Selva-O’Callaghan, V. Fonollosa-Pla, E. Trallero-Araguás, X. Martínez-Gómez, C.P. Simeon-Aznar, M. Labrador-Horrillo, et al.
Nailfold capillary microscopy in adults with inflammatory myopathy.
Semin Arthritis Rheum., (2010), 39 pp. 398-404
http://dx.doi.org/10.1016/j.semarthrit.2008.09.003 | Medline
[45]
2017031912193260839
E.H. Michelle, A.L. Mammen.
Myositis mimics.
Curr Rheumatol Rep., (2015), 17 pp. 63
http://dx.doi.org/10.1007/s11926-015-0541-0 | Medline
[46]
N. Nirmalananthan, J.L. Holton, M.G. Hanna.
Is it really myositis?. A consideration of the differential diagnosis.
Curr Opin Rheumatol., (2004), 16
684-91
[47]
2017031912193260840
A. Selva-O’Callaghan, A. Labirua-Iturburu.
Miopatías inflamatorias, importancia del diagnóstico diferencial.
Cuadernos de Autoinmunidad., (2011), 1
10-9
[48]
A. Selva-O’Callaghan, A. Redondo-Benito, E. Trallero-Araguás, X. Martínez-Gómez, E. Palou, M. Vilardell-Tarres.
Clinical significance of thyroid disease in patients with inflammatory myopathy.
Medicine (Baltimore)., (2007), 86
http://dx.doi.org/10.1128/JVI.05957-11 | Medline
293-8
[49]
2017031912193260841
2017031912193260842
International myositis assessment and clinical studies group (IMACS): global approaches to improved treatments and understanding of myositis.: National Institute of Environmental Health Sciences; [cited 2015]. Disponible en: http://www.niehs.nih.gov/research/resources/imacs/index.cfm.
[50]
H. Alexanderson.
Physical exercise as a treatment for adult and juvenile myositis.
J Intern Med., (2016), 280 pp. 75-96
http://dx.doi.org/10.1111/joim.12481 | Medline
[51]
2017031912193260843
Muscle Study, Group.
A randomized, pilot trial of etanercept in dermatomyositis.
Ann Neurol., (2011), 70
427-36
[52]
2017031912193260844
A. Selva-O’Callaghan, M. Ramos Casals, J.M. Grau Junyent.
[Biologic therapy in idiopathic inflammatory myopathy].
Med Clin (Barc)., (2014), 143
275-80
[53]
2017031912193260845
2017031912193260846
C.V. Oddis, A.M. Reed, R. Aggarwal, L.G. Rider, D.P. Ascherman, M.C. Levesque, RIM Study Group, et al.
Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial.
Arthritis Rheum., (2013), 65
314-24
[54]
2017031912193260847
R. Aggarwal, A. Bandos, A.M. Reed, D.P. Ascherman, R.J. Barohn, B.M. Feldman, R.I.M. et al, Study Group, C.V. Oddis.
Predictors of clinical improvement in rituximab-treated refractory adult and juvenile dermatomyositis and adult polymyositis.
Arthritis Rheumatol., (2014), 66
740-9
[55]
A. Labirua-Iturburu, A. Selva-O’Callaghan, X. Martínez-Gómez, E. Trallero-Araguás, M. Labrador-Horrillo, M. Vilardell-Tarrés.
Calcineurin inhibitors in a cohort of patients with antisynthetase-associated interstitial lung disease.
Clin Exp Rheumatol., (2013), 31
Medline
436-9
[56]
2017031912193260848
P.A. Gordon, J.B. Winer, J.E. Hoogendijk, E.H. Choy.
Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis.
Cochrane Database Syst Rev., (2012),
CD003643