Antisignal Recognition Particle Antibodies–Related Cardiomyopathy
2013; Lippincott Williams & Wilkins; Volume: 127; Issue: 5 Linguagem: Inglês
10.1161/circulationaha.112.118877
ISSN1524-4539
AutoresMathilde Thiébaut, Benjamin Terrier, Samia Menacer, A. Berezné, Guillaume Bussone, Claire Goulvestre, Rémi Bellance, Loı̈c Guillevin, O. Vignaux, Luc Mouthon,
Tópico(s)Immunodeficiency and Autoimmune Disorders
ResumoHomeCirculationVol. 127, No. 5Antisignal Recognition Particle Antibodies–Related Cardiomyopathy Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessEditorialPDF/EPUBAntisignal Recognition Particle Antibodies–Related Cardiomyopathy Mathilde Thiébaut, MD, Benjamin Terrier, MD, PhD, Samia Menacer, MD, Alice Berezne, MD, Guillaume Bussone, MD, PhD, Claire Goulvestre, MD, PhD, Rémi Bellance, MD, Loïc Guillevin, MD, Olivier Vignaux, MD, PhD and Luc Mouthon, MD, PhD Mathilde ThiébautMathilde Thiébaut From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Benjamin TerrierBenjamin Terrier From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Samia MenacerSamia Menacer From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Alice BerezneAlice Berezne From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Guillaume BussoneGuillaume Bussone From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Claire GoulvestreClaire Goulvestre From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Rémi BellanceRémi Bellance From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Loïc GuillevinLoïc Guillevin From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). , Olivier VignauxOlivier Vignaux From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). and Luc MouthonLuc Mouthon From the Department of Internal Medicine (M.T., B.T., A.B., G.B., L.G.), the Department of Cardiology (S.M.), the Laboratory of Immunology (C.G.), and the Department of Radiology and Cardiovascular Imaging (O.V.), Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France; and the Caribbean Reference Center for Neuromuscular Disorders, University Hospital, La Meynard, Fort-de-France, Martinique, France (R.B.). Originally published5 Feb 2013https://doi.org/10.1161/CIRCULATIONAHA.112.118877Circulation. 2013;127:e434–e436IntroductionA 63-year-old woman was referred to our center for the workup of a 6-month history of diffuse myalgia and severe proximal weakness. Physical examination did not find any sign of heart failure. The patient had a medical history of diabetes mellitus. Serum creatine kinase and aldolase levels were increased at 2262 U/L (normal <195 U/L) and 19.9 U/L (normal <7.6 U/L), respectively. ECG showed sinus rhythm at 91 beats per minute with frequent ventricular extrasystoles and normal repolarization. Muscle biopsy of the quadriceps revealed necrotizing myopathy (Figure 1A) and antisignal recognition particle (SRP) antibodies were identified in serum (Figure 1B), consistent with the diagnosis of anti-SRP necrotizing myopathy. Cardiac involvement was documented at diagnosis with increased troponin level at 451 pg/mL (normal <14 pg/mL), and transmural gadolinium enhancement and hypokinesia of the anteroseptal wall on cardiac magnetic resonance (CMR) imaging (Movie I in the online-only Data Supplement). Coronary angiography was normal. The patient received 1 mg/kg/d of prednisone, plasma exchanges, and rituximab with a good efficacy. Clinical and biological relapse occurred 5 months later, leading to adjunction of methotrexate with a remission of the disease.Download figureDownload PowerPointFigure 1. Histology of the muscle and indirect immunofluorescence assay in the serum. A, Muscle biopsy of the quadriceps revealed necrotizing myopathy with regenerating and degenerating fibers contrasting with the absence of inflammatory infiltrates. B, Classic indirect immunofluorescence assay on HEp-2 cells in the serum showed a characteristic cytoplasmic staining pattern. Dot-blot analysis of the serum showed specific reactivity against the signal recognition particle (SRP).Six months later, after discontinuating all treatments by herself, she presented a new relapse of myopathy with proximal weakness, chest pain, and increased serum creatine kinase (1710 U/L) and troponin levels (616 pg/mL). ECG remained stable with sinus rhythm, flattened T waves in apical and lateral regions, and frequent ventricular extrasystoles (Figure 2A and 2B). Cardiac ultrasonography disclosed mild global hypokinesia predominating in anterior and septal territories with 40% left ventricular ejection fraction (Movies II and III in the online-only Data Supplement) associated with septal thickening and infiltration (Figure 3A and 3B) and transmural infiltration of anteroseptal wall (Figure 3C). CMR showed severe global hypokinesia with 23% left ventricular ejection fraction (Movie IV), with an early and late transmural gadolinium enhancement of the anterolateral and anteroseptal walls (Figure 4A and 4B and Movie V in the online-only Data Supplement), associated with circumferential subendocardial enhancement and pericardial effusion (Figure 3C). Combination therapy with rituximab, prednisone, and methotrexate was reintroduced, with an improvement of muscle proximal weakness and the normalization of creatine kinase (145 U/L) and troponine (<14 pg/mL) levels.Download figureDownload PowerPointFigure 2. ECG. A, Sinus rhythm and flattened T waves in apical and lateral regions. B, frequent ventricular extrasystoles.Download figureDownload PowerPointFigure 3. Echocardiographic views. A, Parasternal long axis view of the heart. B, Four-chamber view. C, Short axis image. Cardiac ultrasonography disclosed mild global hypokinesia predominating in anterior and septal territories associated with septal thickening and transmural infiltration.Download figureDownload PowerPointFigure 4. Delayed enhancement cardiac MRI (CMR) images. A, Short axis view. B, Vertical long axis view. CMR imaging showed severe global hypokinesia, with an early and late transmural gadolinium enhancement of the anterolateral and anteroseptal walls. C, Axial 2-chamber view. CMR imaging revealed circumferential subendocardial enhancement and pericardial effusion.Cardiac involvement is recognized as a major clinical manifestation and 1 of the most frequent causes of death in patients with inflammatory or necrotizing myopathies, although its actual frequency is still uncertain, ranging from 6 to 75%.1,2 Anti-SRP antibodies represent one of the muscle-specific autoantibodies, and was associated in previous studies with a more acute and severe form of necrotizing myopathy and more frequent cardiac involvement.3 This particular form of myopathy is characterized by a necrotizing myopathy with regenerating and degenerating fibers contrasting with the absence of inflammatory infiltrates. In contrast to these earlier studies, recent findings revealed that the frequency of cardiac involvement did not differ between patients with necrotizing myopathy associated with anti-SRP antibodies and those with other inflammatory myopathies.4 However, no description of the specific cardiac involvement in patients with anti-SRP necrotizing myopathy is available in the literature. In the present case, we report for the first time findings from cardiac ultrasonography and CMR imaging in a patient with anti-SRP necrotizing myopathy, showing a severe specific cardiomyopathy with severe global hypokinesia, early and late transmural gadolinium enhancement of the anterolateral and anteroseptal wall, and circumferential subendocardial enhancement. Transmural infarction is usually associated with atherosclerosis involving a major coronary artery, what was excluded in the present case as indicated by normal coronary angiography. In contrast, this transmural involvement could be the consequence of the necrosis of myocardial fibers that could lead to congestive heart failure or conduction disturbances. In conclusion, this case illustrates the specific and severe cardiomyopathy that can be observed in patients with anti-SRP necrotizing myopathy, characterized by severe global hypokinesia and transmural myocardial infiltration, and the importance of considering this disorder in the differential diagnosis of myocardial pathology with transmural infiltration and necrosis.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.112.118877/-/DC1.Correspondence to Luc Mouthon, MD, PhD, Service de Médecine Interne, hôpital Cochin, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France. E-mail [email protected]References1. Dankó K, Ponyi A, Constantin T, Borgulya G, Szegedi G. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases.Medicine (Baltimore). 2004; 83:35–42.CrossrefMedlineGoogle Scholar2. Gupta R, Wayangankar SA, Targoff IN, Hennebry TA. Clinical cardiac involvement in idiopathic inflammatory myopathies: a systematic review.Int J Cardiol. 2011; 148:261–270.CrossrefMedlineGoogle Scholar3. Targoff IN, Johnson AE, Miller FW. Antibody to signal recognition particle in polymyositis.Arthritis Rheum. 1990; 33:1361–1370.CrossrefMedlineGoogle Scholar4. Hengstman GJ, ter Laak HJ, Vree Egberts WT, Lundberg IE, Moutsopoulos HM, Vencovsky J, Doria A, Mosca M, van Venrooij WJ, van Engelen BG. Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy.Ann Rheum Dis. 2006; 65:1635–1638.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Ma X, Xu L, Li Y and Bu B (2021) Immunotherapy reversed myopathy but not cardiomyopathy in a necrotizing autoimmune myopathy patient with positive anti-SRP and MDA-5 autoantibodies, BMC Cardiovascular Disorders, 10.1186/s12872-021-01900-2, 21:1, Online publication date: 1-Dec-2021. Gómez G, Pérez N, Braillard Poccard A, Gómez R, Costi A, García M, Viola M, Benitez A, Aciar M, Crespo Espíndola M, Yucra D, Cosatti M, Pisoni C, Capelusnik D, Lojo M, Barrios B, Rivero M, Kisluk B and Granel A (2021) Myositis-specific antibodies and clinical characteristics in patients with autoimmune inflammatory myopathies: reported by the Argentine Registry of Inflammatory Myopathies of the Argentine Society of Rheumatology, Clinical Rheumatology, 10.1007/s10067-021-05797-2, 40:11, (4473-4483), Online publication date: 1-Nov-2021. Tamura T, Miyajima K, Watanabe K, Ito K, Kin F, Okazaki A, Takashima Y, Watanabe T, Kawaguchi Y, Wakabayashi Y, Miyajima H and Maekawa Y (2021) An Autopsy Case of Antibody-negative Immune-mediated Necrotizing Myopathy with Severe Cardiac Involvement, Internal Medicine, 10.2169/internalmedicine.6355-20, 60:19, (3113-3119), Online publication date: 1-Oct-2021. Allenbach Y, Benveniste O, Stenzel W and Boyer O (2020) Immune-mediated necrotizing myopathy: clinical features and pathogenesis, Nature Reviews Rheumatology, 10.1038/s41584-020-00515-9, 16:12, (689-701), Online publication date: 1-Dec-2020. Hara A, Amano R, Yokote H, Ijima M, Zeniya S, Uchihara T, Yada S, Masumura M, Takei H, Nishino I and Toru S (2020) Secondary cardiac involvement in anti-SRP-antibody-positive myopathy: an 87-year-old woman with heart failure symptoms as the first clinical presentation, BMC Neurology, 10.1186/s12883-020-1599-5, 20:1, Online publication date: 1-Dec-2020. Martínez León A, Rugeles Niño J, Brandy García A, Morís de la Tassa C, Caminal Montero L and Rozado Castaño J (2019) Miocarditis como forma de presentación de una miopatía inflamatoria autoinmunitaria asociada con anticuerpos antipartícula de reconocimiento de señal, Revista Española de Cardiología, 10.1016/j.recesp.2018.04.021, 72:5, (423-424), Online publication date: 1-May-2019. Martínez León A, Rugeles Niño J, Brandy García A, Morís de la Tassa C, Caminal Montero L and Rozado Castaño J (2019) Myocarditis as a Form of Presentation of an Inflammatory Autoimmune Myopathy Associated With Anti-signal Recognition Particle Antibodies, Revista Española de Cardiología (English Edition), 10.1016/j.rec.2018.04.024, 72:5, (422-424), Online publication date: 1-May-2019. Takeguchi-Kikuchi S, Hayasaka T, Katayama T, Kano K, Takahashi K, Saito T, Sawada J, Minoshima A, Sakamoto N, Akasaka K, Miyokawa N, Nishino I, Ishibashi-Ueda H and Hasebe N (2019) Anti-signal Recognition Particle Antibody-positive Necrotizing Myopathy with Secondary Cardiomyopathy: The First Myocardial Biopsy- and Multimodal Imaging-proven Case, Internal Medicine, 10.2169/internalmedicine.2564-18, 58:21, (3189-3194), Online publication date: 1-Nov-2019. February 5, 2013Vol 127, Issue 5 Advertisement Article InformationMetrics © 2013 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.112.118877PMID: 23381964 Originally publishedFebruary 5, 2013 PDF download Advertisement SubjectsChronic Ischemic Heart DiseaseImaging
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