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Management of Myocarditis in Myositis: Diagnosis and Treatment

  • Inflammatory Muscle Disease (L Diederichsen and H Chinoy, Section Editors)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

To provide a summary of the current knowledge on myocarditis in idiopathic inflammatory myopathies (IIMs).

Recent Findings

There is increasing epidemiological knowledge about heart involvement generally and myocarditis specifically in IIMs. Cardiac magnetic resonance (CMR) plays an important role in this regard.

Summary

Myocarditis occupies an important place in the spectrum of pathologies that involve the myocardium in patients with IIMs. Nevertheless, its full impact still remains to be elucidated. A larger cooperation between rheumatologists and cardiologists in the clinical, as well as in the research field, is necessary to expand our knowledge in the area.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Zhang L, Wang GC, Ma L, Zu N. Cardiac involvement in adult polymyositis or dermatomyositis: a systematic review. Clin Cardiol. 2012;35(11):686–91.

    CAS  PubMed  Google Scholar 

  2. Schwartz T, Diederichsen LP, Lundberg IE, Sjaastad I, Sanner H. Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies. RMD Open. 2016;2(2):e000291.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Caforio AL, Marcolongo R, Basso C, Iliceto S. Clinical presentation and diagnosis of myocarditis. Heart. 2015;101(16):1332–44.

    CAS  PubMed  Google Scholar 

  4. • Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J, et al. The EuroMyositis registry: an international collaborative tool to facilitate myositis research. Ann Rheum Dis. 2018;77(1):30–9 Due to its large number of patients and the multicenter cooperation, this study provides important epidemiological insights in IIMs and associated pathologies.

    PubMed  Google Scholar 

  5. Caforio AL, Pankuweit S, Arbustini E, Basso C, Gimeno-Blanes J, Felix SB, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2013;34(33):2636–48 48a-48d.

    PubMed  Google Scholar 

  6. Tschope C, Cooper LT, Torre-Amione G, Van Linthout S. Management of myocarditis-related cardiomyopathy in adults. Circ Res. 2019;124(11):1568–83.

    PubMed  Google Scholar 

  7. Baughman KL. Diagnosis of myocarditis: death of Dallas criteria. Circulation. 2006;113(4):593–5.

    PubMed  Google Scholar 

  8. Chow LH, Radio SJ, Sears TD, McManus BM. Insensitivity of right ventricular endomyocardial biopsy in the diagnosis of myocarditis. J Am Coll Cardiol. 1989;14(4):915–20.

    CAS  PubMed  Google Scholar 

  9. Shah Z, Vuddanda V, Rali AS, Pamulapati H, Masoomi R, Gupta K. National trends and procedural complications from endomyocardial biopsy: results from the National Inpatient Sample, 2007-2014. Cardiology. 2018;141(3):125–31.

    PubMed  Google Scholar 

  10. Gupta R, Wayangankar SA, Targoff IN, Hennebry TA. Clinical cardiac involvement in idiopathic inflammatory myopathies: a systematic review. Int J Cardiol. 2011;148(3):261–70.

    PubMed  Google Scholar 

  11. Rosenbohm A, Buckert D, Gerischer N, Walcher T, Kassubek J, Rottbauer W, et al. Early diagnosis of cardiac involvement in idiopathic inflammatory myopathy by cardiac magnetic resonance tomography. J Neurol. 2015;262(4):949–56.

    PubMed  Google Scholar 

  12. Mavrogeni S, Douskou M, Manoussakis MN. Contrast-enhanced CMR imaging reveals myocardial involvement in idiopathic inflammatory myopathy without cardiac manifestations. JACC Cardiovasc Imaging. 2011;4(12):1324–5.

    PubMed  Google Scholar 

  13. Van Gelder H, Charles-Schoeman C. The heart in inflammatory myopathies. Rheum Dis Clin N Am. 2014;40(1):1–10.

    Google Scholar 

  14. Dobloug GC, Svensson J, Lundberg IE, Holmqvist M. Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study. Ann Rheum Dis. 2018;77(1):40–7.

    PubMed  Google Scholar 

  15. Danko 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(1):35–42.

    Google Scholar 

  16. Ammirati E, Cipriani M, Moro C, Raineri C, Pini D, Sormani P, et al. Clinical presentation and outcome in a contemporary cohort of patients with acute myocarditis: multicenter Lombardy registry. Circulation. 2018;138(11):1088–99.

    PubMed  Google Scholar 

  17. Chang JJ, Lin MS, Chen TH, Chen DY, Chen SW, Hsu JT, et al. Heart failure and mortality of adult survivors from acute myocarditis requiring intensive care treatment - a nationwide cohort study. Int J Med Sci. 2017;14(12):1241–50.

    CAS  PubMed  PubMed Central  Google Scholar 

  18. Kindermann I, Kindermann M, Kandolf R, Klingel K, Bultmann B, Muller T, et al. Predictors of outcome in patients with suspected myocarditis. Circulation. 2008;118(6):639–48.

    PubMed  Google Scholar 

  19. Cho SK, Kim H, Myung J, Nam E, Jung SY, Jang EJ, et al. Incidence and prevalence of idiopathic inflammatory myopathies in Korea: a Nationwide population-based study. J Korean Med Sci. 2019;34(8):e55.

    PubMed  PubMed Central  Google Scholar 

  20. Yukiiri K, Mizushige K, Ueda T, Nanba T, Tanimoto K, Wada Y, et al. Fulminant myocarditis in polymyositis. Jpn Circ J. 2001;65(11):991–3.

    CAS  PubMed  Google Scholar 

  21. Ibrahim A, Meagher E, Fraser A, Kiernan TJ. A young male with severe myocarditis and skeletal muscle myositis. Case Rep Cardiol. 2018;2018:5698739.

    PubMed  PubMed Central  Google Scholar 

  22. Maron BJ. Sudden death in young athletes. N Engl J Med. 2003;349(11):1064–75.

    CAS  PubMed  Google Scholar 

  23. Diederichsen LP, Simonsen JA, Diederichsen AC, Kim WY, Hvidsten S, Hougaard M, et al. Cardiac abnormalities assessed by non-invasive techniques in patients with newly diagnosed idiopathic inflammatory myopathies. Clin Exp Rheumatol. 2015;33(5):706–14.

    PubMed  Google Scholar 

  24. Lam L, Sathirareuangchai S, Oyama J, Devendra G, Hong R. Lymphocytic myocarditis and cardiogenic shock in Hawai’i: a case series. Hawaii J Health Soc Welf. 2019;78(7):223–9.

    PubMed  PubMed Central  Google Scholar 

  25. Morgera T, Di Lenarda A, Dreas L, Pinamonti B, Humar F, Bussani R, et al. Electrocardiography of myocarditis revisited: clinical and prognostic significance of electrocardiographic changes. Am Heart J. 1992;124(2):455–67.

    CAS  PubMed  Google Scholar 

  26. Butta C, Zappia L, Laterra G, Roberto M. Diagnostic and prognostic role of electrocardiogram in acute myocarditis: a comprehensive review. Ann Noninvasive Electrocardiol. 2020;25(3). https://doi.org/10.1111/anec.12726.

  27. Schultz JC, Hilliard AA, Cooper LT Jr, Rihal CS. Diagnosis and treatment of viral myocarditis. Mayo Clin Proc. 2009;84(11):1001–9.

    PubMed  PubMed Central  Google Scholar 

  28. Nakashima H, Honda Y, Katayama T. Serial electrocardiographic findings in acute myocarditis. Intern Med. 1994;33(11):659–66.

    CAS  PubMed  Google Scholar 

  29. Lundberg IE. The heart in dermatomyositis and polymyositis. Rheumatology (Oxford). 2006;45 Suppl 4:iv18–21.

    CAS  Google Scholar 

  30. Jacob R, Khan M. Cardiac biomarkers: what is and what can be. Indian J Cardiovasc Dis Women WINCARS. 2018;3(4):240–4.

    PubMed  PubMed Central  Google Scholar 

  31. Thygesen K. 'Ten Commandments' for the Fourth Universal Definition of Myocardial Infarction 2018. Eur Heart J. 2019;40(3):226.

    PubMed  Google Scholar 

  32. Jaffe AS, Vasile VC, Milone M, Saenger AK, Olson KN, Apple FS. Diseased skeletal muscle: a noncardiac source of increased circulating concentrations of cardiac troponin T. J Am Coll Cardiol. 2011;58(17):1819–24.

    PubMed  Google Scholar 

  33. Erlacher P, Lercher A, Falkensammer J, Nassonov EL, Samsonov MI, Shtutman VZ, et al. Cardiac troponin and beta-type myosin heavy chain concentrations in patients with polymyositis or dermatomyositis. Clin Chim Acta. 2001;306(1–2):27–33.

    CAS  PubMed  Google Scholar 

  34. Rittoo D, Jones A, Lecky B, Neithercut D. Elevation of cardiac troponin T, but not cardiac troponin I, in patients with neuromuscular diseases: implications for the diagnosis of myocardial infarction. J Am Coll Cardiol. 2014;63(22):2411–20.

    CAS  PubMed  Google Scholar 

  35. Lilleker JB, Diederichsen ACP, Jacobsen S, Guy M, Roberts ME, Sergeant JC, et al. Using serum troponins to screen for cardiac involvement and assess disease activity in the idiopathic inflammatory myopathies. Rheumatology (Oxford). 2018;57(6):1041–6.

    CAS  Google Scholar 

  36. Kobayashi D, Aggarwal S, Kheiwa A, Shah N. Myopericarditis in children: elevated troponin I level does not predict outcome. Pediatr Cardiol. 2012;33(7):1040–5.

    PubMed  Google Scholar 

  37. Pinamonti B, Alberti E, Cigalotto A, Dreas L, Salvi A, Silvestri F, et al. Echocardiographic findings in myocarditis. Am J Cardiol. 1988;62(4):285–91.

    CAS  PubMed  Google Scholar 

  38. Mendes LA, Dec GW, Picard MH, Palacios IF, Newell J, Davidoff R. Right ventricular dysfunction: an independent predictor of adverse outcome in patients with myocarditis. Am Heart J. 1994;128(2):301–7.

    CAS  PubMed  Google Scholar 

  39. Schwartz T, Sanner H, Gjesdal O, Flato B, Sjaastad I. In juvenile dermatomyositis, cardiac systolic dysfunction is present after long-term follow-up and is predicted by sustained early skin activity. Ann Rheum Dis. 2014;73(10):1805–10.

    PubMed  Google Scholar 

  40. Schwartz T, Sanner H, Husebye T, Flato B, Sjaastad I. Cardiac dysfunction in juvenile dermatomyositis: a case-control study. Ann Rheum Dis. 2011;70(5):766–71.

    PubMed  Google Scholar 

  41. Lu Z, Wei Q, Ning Z, Qian-Zi Z, Xiao-Ming S, Guo-Chun W. Left ventricular diastolic dysfunction -- early cardiac impairment in patients with polymyositis/dermatomyositis: a tissue Doppler imaging study. J Rheumatol. 2013;40(9):1572–7.

    PubMed  Google Scholar 

  42. Wang H, Liu HX, Wang YL, Yu XQ, Chen XX, Cai L. Left ventricular diastolic dysfunction in patients with dermatomyositis without clinically evident cardiovascular disease. J Rheumatol. 2014;41(3):495–500.

    PubMed  Google Scholar 

  43. Ponikowski P, Voors AA, Anker SD, Bueno H, Cleland JGF, Coats AJS, et al. 2016 ESC guidelines for the diagnosis and treatment of acute and chronic heart failure: the task force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) developed with the special contribution of the Heart Failure Association (HFA) of the ESC. Eur Heart J. 2016;37(27):2129–200.

    PubMed  Google Scholar 

  44. Friedrich MG, Sechtem U, Schulz-Menger J, Holmvang G, Alakija P, Cooper LT, et al. Cardiovascular magnetic resonance in myocarditis: a JACC white paper. J Am Coll Cardiol. 2009;53(17):1475–87.

    PubMed  PubMed Central  Google Scholar 

  45. Khoo T, Stokes MB, Teo K, Proudman S, Basnayake S, Sanders P, et al. Cardiac involvement in idiopathic inflammatory myopathies detected by cardiac magnetic resonance imaging. Clin Rheumatol. 2019;38(12):3471–6.

    PubMed  Google Scholar 

  46. Messroghli DR, Moon JC, Ferreira VM, Grosse-Wortmann L, He T, Kellman P, et al. Clinical recommendations for cardiovascular magnetic resonance mapping of T1, T2, T2* and extracellular volume: a consensus statement by the Society for Cardiovascular Magnetic Resonance (SCMR) endorsed by the European Association for Cardiovascular Imaging (EACVI). J Cardiovasc Magn Reson. 2017;19(1):75.

    PubMed  PubMed Central  Google Scholar 

  47. Hinojar R, Foote L, Arroyo Ucar E, Jackson T, Jabbour A, Yu CY, et al. Native T1 in discrimination of acute and convalescent stages in patients with clinical diagnosis of myocarditis: a proposed diagnostic algorithm using CMR. JACC Cardiovasc Imaging. 2015;8(1):37–46.

    PubMed  Google Scholar 

  48. Bohnen S, Radunski UK, Lund GK, Kandolf R, Stehning C, Schnackenburg B, et al. Performance of t1 and t2 mapping cardiovascular magnetic resonance to detect active myocarditis in patients with recent-onset heart failure. Circ Cardiovasc Imaging. 2015;8(6):e003073.

    PubMed  Google Scholar 

  49. Huber AT, Bravetti M, Lamy J, Bacoyannis T, Roux C, de Cesare A, et al. Non-invasive differentiation of idiopathic inflammatory myopathy with cardiac involvement from acute viral myocarditis using cardiovascular magnetic resonance imaging T1 and T2 mapping. J Cardiovasc Magn Reson. 2018;20(1):11.

    PubMed  PubMed Central  Google Scholar 

  50. • Xu Y, Sun J, Wan K, Yu L, Wang J, Li W, et al. Multiparametric cardiovascular magnetic resonance characteristics and dynamic changes in myocardial and skeletal muscles in idiopathic inflammatory cardiomyopathy. J Cardiovasc Magn Reson. 2020;22(1):22 According to this study, CMR is not only sensitive for detecting cardiac involvement, but also can detect changes over time, and as such has the potential to be used as a screening tool for treatment response.

    CAS  PubMed  PubMed Central  Google Scholar 

  51. Buchpiguel CA, Roizemblatt S, Pastor EH, Hironaka FH, Cossermelli W. Cardiac and skeletal muscle scintigraphy in dermato- and polymyositis: clinical implications. Eur J Nucl Med. 1996;23(2):199–203.

    CAS  PubMed  Google Scholar 

  52. Diederichsen LP, Simonsen JA, Diederichsen AC, Hvidsten S, Hougaard M, Junker P, et al. Cardiac abnormalities in adult patients with polymyositis or dermatomyositis as assessed by noninvasive modalities. Arthritis Care Res (Hoboken). 2016;68(7):1012–20.

    CAS  Google Scholar 

  53. Denbow CE, Lie JT, Tancredi RG, Bunch TW. Cardiac involvement in polymyositis: a clinicopathologic study of 20 autopsied patients. Arthritis Rheum. 1979;22(10):1088–92.

    CAS  PubMed  Google Scholar 

  54. Haupt HM, Hutchins GM. The heart and cardiac conduction system in polymyositis-dermatomyositis: a clinicopathologic study of 16 autopsied patients. Am J Cardiol. 1982;50(5):998–1006.

    CAS  PubMed  Google Scholar 

  55. Vartanian RK, Finkbeiner WE. Endomyocardial biopsy findings in a patient with polymyositis-dermatomyositis. Cardiovasc Pathol. 1993;2(1):1–5.

    CAS  PubMed  Google Scholar 

  56. Sharma K, Orbai AM, Desai D, Cingolani OH, Halushka MK, Christopher-Stine L, et al. Brief report: antisynthetase syndrome-associated myocarditis. J Card Fail. 2014;20(12):939–45.

    PubMed  PubMed Central  Google Scholar 

  57. Dieval C, Deligny C, Meyer A, Cluzel P, Champtiaux N, Lefevre G, et al. Myocarditis in patients with antisynthetase syndrome: prevalence, presentation, and outcomes. Medicine (Baltimore). 2015;94(26):e798.

    CAS  Google Scholar 

  58. Cooper LT, Baughman KL, Feldman AM, Frustaci A, Jessup M, Kuhl U, et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology. Eur Heart J. 2007;28(24):3076–93.

    PubMed  Google Scholar 

  59. Allanore Y, Vignaux O, Arnaud L, Puechal X, Pavy S, Duboc D, et al. Effects of corticosteroids and immunosuppressors on idiopathic inflammatory myopathy related myocarditis evaluated by magnetic resonance imaging. Ann Rheum Dis. 2006;65(2):249–52.

    CAS  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark

    Katrine Aagaard Myhr

  2. Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark

    Redi Pecini

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  1. Katrine Aagaard Myhr
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Myhr, K.A., Pecini, R. Management of Myocarditis in Myositis: Diagnosis and Treatment. Curr Rheumatol Rep 22, 49 (2020). https://doi.org/10.1007/s11926-020-00925-4

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