Rheumatology International

, Volume 39, Issue 10, pp 1821–1827 | Cite as

Spontaneous coronary artery dissection in systemic lupus erythematosus: case-based review

  • Bonifacio Álvarez-LarioEmail author
  • Laura Álvarez-Roy
  • Sandra Mayordomo-Gómez
  • Juan Marcos García-García
Case Based Review


Patients with systemic lupus erythematosus (SLE) present an increased prevalence of coronary heart disease. The majority of cases of acute coronary syndrome (ACS) in patients with SLE are due to atherosclerosis. Less common causes include thrombosis of an angiographically normal coronary artery and coronary vasculitis. Spontaneous coronary artery dissection (SCAD) is a rare cause of ACS in these patients. We report the case of a 53-year-old female diagnosed of SLE presenting with an ACS caused by SCAD. She was treated medically and her clinical course was favorable. A literature search identified seven additional cases of SCAD associated with SLE. The main clinical features found in these reports are revised. ACS caused by SCAD in SLE patients is a condition likely under-reported in literature. SCAD should be suspected in patients with SLE and ACS, especially in younger women without evident cardiovascular risk factors. An early accurate diagnosis of SCAD is key to provide specific treatment, which differs from that of usual atherosclerotic ACS.


Systemic lupus erythematosus Coronary artery disease Myocardial infarction Spontaneous coronary artery dissection 



The authors wish to thank the Research Unit of Hospital Universitario de Burgos (HUBU) for their help in the preparation of the English version of the manuscript.

Author contributions

The case was diagnosed and followed up by SMG, BAL and JMGG. BAL and LAR conceived and planned the study. BAL and LAR wrote the manuscript. Literature data were searched and analyzed by all the authors. The final version was read, corrected and approved by all the authors.


The authors received no financial support for the research, authorship, and/or publication of this article.

Compliance with ethical standards

Conflict of interest

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Ethical approval

All the procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. All the authors fulfilled the ICMJE authorship criteria.

Informed consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


  1. 1.
    Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE et al (2018) Spontaneous coronary artery dissection: current state of the science: a scientific statement from the American Heart Association. Circulation 137(19):e523–e557. CrossRefGoogle Scholar
  2. 2.
    Alfonso F, García-Guimaraes M, Bastante T, de la Cuerda F, Antuña P, Cuesta J, Rivero F (2018) Spontaneous coronary artery dissection: from expert consensus statements to evidence-based medicine. J Thorac Dis 10(7):4602–4608. CrossRefGoogle Scholar
  3. 3.
    Adlam D, Alfonso F, Maas A, Vrints C (2018) European Society of Cardiology, acute cardiovascular care association, SCAD study group: a position paper on spontaneous coronary artery dissection. Eur Heart J 39(36):3353–3368. CrossRefGoogle Scholar
  4. 4.
    Krittanawong C, Kumar A, Johnson KW, Luo Y, Yue B, Wang Z, Bhatt DL (2019) Conditions and factors associated with spontaneous coronary artery dissection (from a national population-based cohort study). Am J Cardiol 123(2):249–253. CrossRefGoogle Scholar
  5. 5.
    Aldoboni AH, Hamza EA, Majdi K, Ngibzadhe M, Palasaidi S, Moayed DA (2002) Spontaneous dissection of coronary artery treated by primary stenting as the first presentation of systemic lupus erythematosus. J Invasive Cardiol 14(11):694–696Google Scholar
  6. 6.
    Sharma AK, Farb A, Maniar P, Ajani AE, Castagna M, Virmani R, Suddath W, Lindsay J (2003) Spontaneous coronary artery dissection in a patient with systemic lupus erythematosus. Hawaii Med J 62(11):248–253Google Scholar
  7. 7.
    Kothari D, Ruygrok P, Gentles T, Occleshaw C (2007) Spontaneous coronary artery dissection in an adolescent man with systemic lupus erythematosus. Intern Med J 37(5):342–343. CrossRefGoogle Scholar
  8. 8.
    Nisar MK, Mya T (2011) Spontaneous coronary artery dissection in the context of positive anticardiolipin antibodies and clinically undiagnosed systemic lupus erythematosus. Lupus 20(13):1436–1438. CrossRefGoogle Scholar
  9. 9.
    Besinger BR, Gardner S (2013) Spontaneous coronary artery dissection in a 27-year-old woman. J Emerg Med 44(2):e239–e242. CrossRefGoogle Scholar
  10. 10.
    Rekik S, Lanfranchi P, Jacq L, Bernasconi F (2013) Spontaneous coronary artery dissection in a 35 year-old woman with systemic lupus erythematosus successfully treated by angioplasty. Heart Lung Circ 22(11):955–958. CrossRefGoogle Scholar
  11. 11.
    Reddy S, Vaid T, Ganiga Sanjeeva NC, Shetty RK (2016) Spontaneous coronary artery dissection as the first presentation of systemic lupus erythematosus. BMJ Case Rep. (pii: bcr2016216344) Google Scholar
  12. 12.
    Kavandi H, Golmohammadi A, Sadeghi R, Hajizadeh R (2018) Coronary artery dissection in a woman with previous systemic lupus erythematosus using oral contraceptive pills. Acta Med Iran 56(12):815–817Google Scholar
  13. 13.
    Manzi S, Meilahn EN, Rairie JE, Conte CG, Medsger TA Jr, Jansen-McWilliams L, D’Agostino RB, Kuller LH (1997) Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. Am J Epidemiol 145(5):408–415CrossRefGoogle Scholar
  14. 14.
    Karrar A, Sequeira W, Block JA (2001) Coronary artery disease in systemic lupus erythematosus: a review of the literature. Semin Arthritis Rheum 30(6):436–443. CrossRefGoogle Scholar
  15. 15.
    Li H, Tong Q, Guo L, Yu S, Li Y, Cao Q, Li J, Li F (2018) Risk of coronary artery disease in patients with systemic lupus erythematosus: a systematic review and meta-analysis. Am J Med Sci 356(5):451–463. CrossRefGoogle Scholar
  16. 16.
    Kaul MS, Rao SV, Shaw LK, Honeycutt E, Ardoin SP, St Clair EW (2013) Association of systemic lupus erythematosus with angiographically defined coronary artery disease: a retrospective cohort study. Arthritis Care Res (Hoboken) 65(2):266–273. CrossRefGoogle Scholar
  17. 17.
    Ando T, Adegbala O, Akintoye E, Ashraf S, Briasoulis A, Takagi H, Afonso L (2019) Acute myocardial infarction outcomes in systemic lupus erythematosus (from the nationwide inpatient sample). Am J Cardiol 123(2):227–232. CrossRefGoogle Scholar
  18. 18.
    Kiani AN, Magder LS, Petri M (2012) Mycophenolate mofetil (MMF) does not slow the progression of subclinical atherosclerosis in SLE over 2 years. Rheumatol Int 32(9):2701–2705. CrossRefGoogle Scholar
  19. 19.
    Frostegård J (2008) Systemic lupus erythematosus and cardiovascular disease. Lupus 17(5):364–367. CrossRefGoogle Scholar
  20. 20.
    Haque S, Gordon C, Isenberg D, Rahman A, Lanyon P, Bell A, Emery P, McHugh N, Teh LS, Scott DG, Akil M, Naz S, Andrews J, Griffiths B, Harris H, Youssef H, McLaren J, Toescu V, Devakumar V, Teir J, Bruce IN (2010) Risk factors for clinical coronary heart disease in systemic lupus erythematosus: the lupus and atherosclerosis evaluation of risk (LASER) study. J Rheumatol 37(2):322–329. CrossRefGoogle Scholar
  21. 21.
    Reiss AB, Anwar K, Merrill JT, Chan ES, Awadallah NW, Cronstein BN, Michael Belmont H, Belilos E, Rosenblum G, Belostocki K, Bonetti L, Hasneen K, Carsons SE (2010) Plasma from systemic lupus patients compromises cholesterol homeostasis: a potential mechanism linking autoimmunity to atherosclerotic cardiovascular disease. Rheumatol Int 30(5):591–598. CrossRefGoogle Scholar
  22. 22.
    Fang CT, Fang YP, Huang YB, Kuo CC, Chen CY (2017) Epidemiology and risk factors of coronary artery aneurysm in Taiwan: a population based case control study. BMJ Open 7(6):e014424. CrossRefGoogle Scholar
  23. 23.
    Nobrega TP, Klodas E, Breen JF, Liggett SP, Higano ST, Reeder GS (1996) Giant coronary artery aneurysms and myocardial infarction in a patient with systemic lupus erythematosus. Cathet Cardiovasc Diagn 39(1):75–79CrossRefGoogle Scholar
  24. 24.
    Robinowitz M, Virmani R, JrU McAllister HA (1982) Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship? Am J Med 72(6):923–928CrossRefGoogle Scholar
  25. 25.
    Pitliya A, Datta S, Kalayci A, Kahe F, Sharfaei S, Jafarizade M, Goudarzi S, Chi G (2018) Eosinophilic inflammation in spontaneous coronary artery dissection: A potential therapeutic target? Med Hypotheses 121:91–94. CrossRefGoogle Scholar
  26. 26.
    Kounis NG, Koniari I, Velissaris D, Soufras G, Hahalis G (2018) Aortic aneurysm and dissection in systemic lupus erythematosus -pathophysiologic and therapeutic considerations-. Eur J Rheumatol 5(3):209–211. CrossRefGoogle Scholar
  27. 27.
    Chongprasertpon N, Ibrahim A, Goggins M, Kiernan T (2019) Chronic spontaneous coronary artery dissection in association with antiphospholipid syndrome presenting as stable angina. BMJ Case Rep 12(3):e227674. (pii: e227674) CrossRefGoogle Scholar
  28. 28.
    Peng X, Zhu Z, Tang J, Zhou S (2018) Spontaneous coronary artery dissection accompanied with antiphospholipid syndrome and leukemia. Int Heart J 59(4):891–894. CrossRefGoogle Scholar
  29. 29.
    Krishnamurthy M, Desai R, Patel H (2004) Spontaneous coronary artery dissection in the postpartum period: association with antiphospholipid antibody. Heart 90(9):e53. CrossRefGoogle Scholar
  30. 30.
    Tweet MS, Hayes SN, Pitta SR, Simari RD, Lerman A, Lennon RJ, Gersh BJ, Khambatta S, Best PJ, Rihal CS, Gulati R (2012) Clinical features, management, and prognosis of spontaneous coronary artery dissection. Circulation 126(5):579–588. CrossRefGoogle Scholar
  31. 31.
    Tweet MS, Gulati R, Williamson EE, Vrtiska TJ, Hayes SN (2016) Multimodality imaging for spontaneous coronary artery dissection in women. JACC Cardiovasc Imaging 9(4):436–450. CrossRefGoogle Scholar
  32. 32.
    Roura G, Ariza-Solé A, Rodriguez-Caballero IF, Gomez-Lara J, Ferreiro JL, Romaguera R, Teruel L, de Albert M, Gomez-Hospital JA, Cequier A (2016) Noninvasive follow-up of patients with spontaneous coronary artery dissection with CT angiography. JACC Cardiovasc Imaging 9(7):896–897. CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Sección de ReumatologíaHospital Universitario de BurgosBurgosSpain
  2. 2.Cardiology DepartmentComplejo Hospitalario San Millán-San PedroLogroñoSpain
  3. 3.Cardiology DepartmentHospital Universitario de BurgosBurgosSpain

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