A misdiagnosed case of antisynthetase syndrome complicated by myocarditis: when the cardiologist deals with rheumatology

  • Paolo Diego L’AngiocolaEmail author
  • Luisa Mattei
  • Gerardina Lardieri
Case Based Review


We report a case of a 56-year-old woman with an alleged diagnosis of an acute coronary syndrome that was later correctly identified in our Cardiology Unit as antisynthetase syndrome (AS) with inflammatory cardiac involvement. In this case report, we focus on clinical features of this rare autoimmune disease aiming to provide useful tips to achieve correct differential diagnosis according to updated international guidelines and recommendations, especially in cases of concurrent disease-related myocarditis.


Acute coronary syndrome Antisynthetase syndrome Myocarditis 



  1. 1.
    Yoshida S, Akizuki M, Mimori T, Yamagata H, Inada S, Homma M (1983) The precipitating antibody to an acidic nuclear protein antigen, the Jo-1, in connective tissue diseases. A marker for a subset of poly-myositis with interstitial pulmonary fibrosis. Arthritis Rheum 26:604–611CrossRefGoogle Scholar
  2. 2.
    Brouwer R, Hengstman GJ, Vree Egberts W, Ehrfeld H, Bozic B, Ghirardello A et al (2001) Autoantibody profiles in the sera of European patients with myositis. Ann Rheum Dis 60:116–123CrossRefGoogle Scholar
  3. 3.
    Sharma K, Orbai A-M, Desai D, Cingolani OH, Halushka MK, Christopher-Stine L, Mammen AL, Wu KC, Zakaria S (2014) Brief report: antisynthetase syndrome-associated myocarditis. J Card Fail 20:939–945CrossRefGoogle Scholar
  4. 4.
    Cooper LT Jr (2009) Myocarditis. N Engl J Med 360:1526–1538CrossRefGoogle Scholar
  5. 5.
    Sagar S, Liu PP, Cooper LT Jr (2012) Myocarditis. Lancet 379:738–747CrossRefGoogle Scholar
  6. 6.
    Elamm C, Fairweather D, Cooper LT (2012) Pathogenesis and diagnosis of myocarditis. Heart 98:835–840CrossRefGoogle Scholar
  7. 7.
    Sinagra G, Anzini M, Pereira NL, Bussani R, Finocchiaro G, Bar-tunek J et al (2016) Myocarditis in clinical practice. Mayo Clin Proc 91:1256–1266CrossRefGoogle Scholar
  8. 8.
    Ammirati E, Cipriani M, Lilliu M, Sormani P, Varrenti M, Raineri C, Petrella D, Garascia A, Pedrotti P, Roghi A, Bonacina E, Moreo A, Bottiroli M, Gagliardone MP, Mondino M, Ghio S, Totaro R, Turazza FM, Russo CF, Oliva F, Camici PG, Frigerio M (2017) Survival and left ventricular function changes in fulminant versus nonfulminant acute myocarditis. Circulation 136:529–545CrossRefGoogle Scholar
  9. 9.
    Dieval C, Deligny C, Meyer A, Cluzel P, Champtiaux N, Lefevre G, Saadoun D, Sibilia J, Pellegrin JL, Hachulla E, Benveniste O, Hervier B (2015) Myocarditis in patients with antisynthetase syndrome: prevalence, presentation, and outcomes. Medicine (Baltimore) 94:e798CrossRefGoogle Scholar
  10. 10.
    Meudec L, Jelin G, Forien M, Palazzo E, Dieudé P, Ottaviani S (2018) Antisynthetase syndrome and cardiac involvement: a rare association. Joint Bone Spine.
  11. 11.
    Kulkarni HS, Gutierrez FR, Despotovic V, Russell TD (2015) A 43-year-old man with antisynthetase syndrome presenting with acute worsening of dyspnea. Chest 147(6):e215–e219. CrossRefGoogle Scholar
  12. 12.
    Mahfoudhi M, Battikh AG (2015) Antisynthetase syndrome complicated with severe myocarditis. Pan Afr Med J 21:4. Google Scholar
  13. 13.
    Allanore Y, Vignaux O, Arnaud L, Puéchal X, Pavy S, Duboc D, Legmann P, Kahan A (2006) Effects of corticosteroids and immunosuppressors on idiopathic inflammatory myopathy related myocarditis evaluated by magnetic resonance imaging. Ann Rheum Dis 65(2):249–252CrossRefGoogle Scholar
  14. 14.
    Kramer CM, Barkhausen J, Flamm SD, Kim RJ, Nagel E (2013) Society for Cardiovascular Magnetic Resonance Board of Trustees Task Force on Standardized Protocols. Standardized cardiovascular magnetic resonance (CMR) protocols 2013 update. J Cardiovasc Magn Reson 15(91):2013Google Scholar
  15. 15.
    Lurz P, Luecke C, Eitel I, Föhrenbach F, Frank C, Grothoff M, de Waha S, Rommel KP, Lurz JA, Klingel K, Kandolf R, Schuler G, Thiele H, Gutberlet M (2016) Comprehensive cardiac magnetic resonance imaging in patients with suspected myocarditis: the MyoRacer-trial. J Am Coll Cardiol 67:1800–1811CrossRefGoogle Scholar
  16. 16.
    Veronese G, Ammirati E, Cipriani M, Frigerio M (2018) Fulminant myocarditis: characteristics, treatment, and outcomes. Anatol J Cardiol 19(4):279–286. Google Scholar
  17. 17.
    Caforio AL, Pankuweit S, Arbustini E et al (2013) 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 34(33):2636–2648, 2648a-2648d. CrossRefGoogle Scholar
  18. 18.
    Schultheiss HP, Kuhl U, Cooper LT (2011) The management of myocarditis. Eur Heart J 32:2616–2625CrossRefGoogle Scholar
  19. 19.
    Brambatti M, Matassini MV, Adler ED, Klingel K, Camici PG, Ammirati E (2017) Eosinophilic myocarditis: characteristics, treatment, and outcomes. J Am Coll Cardiol 70:2363–2375CrossRefGoogle Scholar
  20. 20.
    Cooper LT Jr, Hare JM, Tazelaar HD, Edwards WD, Starling RC, Deng MC, Menon S, Mullen GM, Jaski B, Bailey KR, Cunningham MW, Dec GW (2008) Usefulness of immunosuppression for giant cell myocarditis. Am J Cardiol 102:1535–1539CrossRefGoogle Scholar
  21. 21.
    Ammirati E, Oliva F, Belli O, Bonacina E, Pedrotti P, Turazza FM, Roghi A, Paino R, Martinelli L, Frigerio M (2016) Giant cell myocarditis successfully treated with antithymocyte globuline and extracorporeal membrane oxygenation for 21 days. J Cardiovasc Med (Hagerstown) 17(Suppl 2):e151–e153CrossRefGoogle Scholar
  22. 22.
    Barba T, Fort R, Cottin V, Provencher S, Durieu I, Jardel S, Hot A, Reynaud Q, Lega JC (2019) Treatment of idiopathic inflammatory myositis associated interstitial lung disease: a systematic review and meta-analysis. Autoimmun Rev 18(2):113–122. CrossRefGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Department of CardiologySan Giovanni di Dio HospitalGoriziaItaly
  2. 2.Department of Cardiology, San Giovanni di Dio HospitalGorizia - San Polo HospitalMonfalconeItaly

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