Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical features and variable clinical course. SLE tends to affect women during childbearing years and is characterized by multi-organ involvement. Cardiac complications in SLE, which have been described to occur in about 50% of the cases, contributes to significant morbidity and mortality in this population. We describe a patient with SLE and established lupus nephritis who subsequently developed cardiac manifestations including valvular abnormalities, arrythmia and end stage heart failure. The clinical features, work up and management will be discussed. Case presentation: A 35 year-old African American woman diagnosed with SLE in her twenties presented to our hospital for evaluation of shortness of breath. After SLE diagnosis, the patient had been prescribed hydrochloroquine and low dose steroids for joint and skin manifestations. Four years after initial presentation, she developed biopsy proven lupus nephritis for which standard induction therapy was administered. She was placed on maintenance immunosuppression with stable renal function. On admission, the patient’s symptoms included dyspnea on exertion, chest pain, palpitations, and a non-productive cough. Initial evaluation identified atrial fibrillation and new onset of heart failure given elevated brain natriuretic peptide (BNP) levels and left ventricular ejection fraction (EF) of 15% by echocardiogram. Cardiac catheterization revealed global hypokinesis and non-obstructive coronary artery disease (CAD). The patient was deemed not a suitable candidate for cardiac transplant and was offered a life vest as bridging to an implantable cardioverter (ICD). Twenty-four months after discharge, the patient continued to be managed medically and has not had any subsequent hospitalizations. Conclusion: Cardiac complications, reported in about 50% of SLE patients, are associated with high morbidity and mortality. Pericarditis is the most common, however conduction defects, valvular damage and heart failure are also observed among SLE patients. The pathogenesis of cardiac involvement seems to be multifactorial. The management of heart failure in SLE entails medical therapy and implantable device use. Further research is needed to explore new options to arrest the development and progression of cardiac disease among lupus patients.
| [1] | Jain, D., Halushka, M.K. Cardiac Pathology of Systemic Lupus Erythematosus. J Clin Pathol (2009). 62: 584-592.View Article PubMed |
| [2] | Kim C.H., Al-Kindi S.G., Jandali B., Askari A.D., Zacharias M., Oliveira G.H. Incidence and risk of heart failure in systemic lupus erythematosus. Heart. 2016; 103(3): 227-233.View Article PubMed |
| [3] | Jonsson, H., Nived O., Sturfelt G. Outcome in systemic lupus erythematosus: a prospective study of patients from a defined population. Medicine (Baltimore) 1989; 68: 141-50.View Article |
| [4] | Manzi S, Meilahn EN, Rairie JE, Conte CG, Medsger TA Jr, Jansen-McWilliams L, et al. Age-specific incidence rates of myo- cardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham study. Am J Epidemiol 1997; 145: 408-15.View Article PubMed |
| [5] | Esdaile JM, Abrahamowicz M, Grodzicky T, Li Y, Panaritis C, du Berge R, et al.Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Arthritis Rheum 2001; 44: 2331-7.View Article |
| [6] | Posadas-Romero C, Torres-Tamayo M, Zamora-Gonzalez J, Aguilar-Herrera BE, Posadas-Sanchez R, Cardosa-Saldana G, Ladron de Guevara G, Solis-Vallejo E, El Hafidi M. High insulin levels and increased low-density lipoprotein oxidizability in pediatric patients with systemic lupus erythematosus. Arthritis Rheum. 2004; 50: 160-165.View Article PubMed |
| [7] | El‐Magadmi M, Bodill H, Ahmad Y, Durrington PN, Mackness M, Walker M, et al.Systemic lupus erythematosus: an independent risk factor for endothelial dysfunction in women. Circulation 2004; 110: 399-404.View Article PubMed |
| [8] | Tincani, A., Rebaioli, C.B., Taglietti,M., Shoenfeld, Y., Heart Involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus, Rheumatology 2006, vol 45 (8-3).View Article PubMed |
| [9] | Atalay, F., Gulmez, O., and Ugurlu, A.O. Cardiotoxicity following cyclophosphamide therapy: a case report. Journal of Medical Case Reports 2014. 8: 252.View Article PubMed |
| [10] | Wu, H., Zeng, J., Yin, J., Peng, Q., Zhao, M., Lu, Q. Organ-specific biomarkers in lupus. Autoimmunity reviews. 2017. Vol 16, Issue 4. 391-397.View Article PubMed |
| [11] | Leonard, D., Svenungusson, E., Sandling, J.K, et al. Coronary heart disease in systemic lupus erythematosus is associated with interferon regulatory factor-8 gene variants. Circ Cardiovasc Genet. 2013 Jun; 6(3): 255-63.View Article PubMed |
| [12] | Ciccacci C, Perricone C, Ceccarelli F, et al. A Multilocus Genetic Study in a Cohort of Italian SLE Patients Confirms the Association with STAT4 Gene and Describes a New Association with HCP5 Gene. PLoS ONE. 2014; 9(11).View Article PubMed |
| [13] | Doria A, Iaccarino L, Sarzi-Puttini P, et al. Cardiovascular involvement in systemic lupus erythemathosus, Lupus , 2005, vol. 14 (pg. 683-6).View Article PubMed |
| [14] | Moyssakis I., Tektonidou M.G., Vasilliou V.A. Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution. Am J Med. 2007; 120: 636-642.View Article PubMed |
| [15] | Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman–Sacks endocarditis) in the antiphospholipid syndrome, Circulation , 1996, vol. 93 (pg. 1579-87).View Article PubMed |
| [16] | Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus erythematosus during a 5 year period: a multicenter prospective study of 1000 patients, Medicine, 1999, vol. 78 (pg. 167-75).View Article PubMed |
| [17] | Ashrafi, R., Garg, P., McKay, E., Gosney, J., Chuah, S., and Davis G. Aggressive cardiac involvement in systemic lupus erythematosus: a case report and a comprehensive literature review. Cardiol Res Pract. 2011; 2011: 578390.View Article PubMed |
| [18] | Raza K, Thambyrajah J, Townend JN, et al. Suppression of inflammation in primary systemic vasculitis restores vascular endothelial function: lessons for atherosclerotic disease? Circulation. 2000; 102: 1470-1472.View Article PubMed |
| [19] | Leonard D, Svenungsson E, Dahlqvist J, et al. Ann Rheum Dis 2018;77: 1063-1069.View Article PubMed |
| [20] | EJ, Dellsperger KC. Cardiorenal Syndrome: The Clinical Cardiologists’ Perspective. Cardiorenal Medicine. 2011; 1(1): 13-22.View Article PubMed |
| [21] | Ridker, PM, Everett, BM, Thuren, T, MacFadyen, JG, Chang, WH, Ballantyne, C., Fonseca, F., Nicolau, J., Koenig, W, Anker, SD et al. Antiinflammatory therapy with Canakinumab for atherosclerotic disease. N Engl J Med, 377 (2017), pp. 1119-1131.View Article PubMed |