Sudden Death in a Young Male Patient: When Lupus and Cardiovascular Disease Intersect

Main Article Content

Guilherme Guilherme Trindade Martins Moreira Da Silva Álvaro Luís Steiner Fernandes de Souza Anna Carolina Alves Guimarães Aragão Vitor Guedes Bonisson Valério Fuks Álvaro Luís Steiner Fernandes de Souza Ludmilla da Rocha Freitas Vieitas Thiago Sant'Anna Coutinho

Abstract

Herein we present the case of a 27-year-old male patient who was recently diagnosed with Systemic Lupus Erythematosus (SLE) associated with nephritis with atypical findings on kidney biopsy and premature cardiovascular disease despite scarce traditional risk factors and negative laboratory findings for antiphospholipid syndrome. A literature review on the epidemiology and pathogenesis of coronary disease in SLE was conducted. The patient was admitted for pulse therapy after refractory disease on outpatient care but evolved with ventricular fibrillation secondary to acute thrombosis in the proximal segment of the anterior left descending artery, with no evidence of atherosclerotic disease. Laboratory findings were also negative for antiphospholipid syndrome both upon diagnosis and after the coronary event.  He underwent manual thrombus aspiration and angioplasty with a drug-eluting stent, successfully weaning off mechanical ventilation and vasopressors at the intensive care unit and showing no neurological deficits or left ventricular function impairment. Kidney biopsy later resulted positive for immunoglobulin A nephropathy. The patient was discharged asymptomatic on guideline-directed therapy for coronary disease, warfarin associated with clopidogrel, anti-hypertensive drugs and a combination of immunosuppressants, remaining well on three-month follow-up. SLE is a chronic inflammatory disease that has increasingly been recognized as a major cardiovascular risk factor due to a variety of mechanisms involving accelerated atherosclerosis and thrombosis. As patients survive the first years of disease onset from infection and disease-specific complications, cardiovascular outcomes become a great concern. However, early events such as in this case are possible, especially in the context of flaring disease. We emphasize the importance of an interdisciplinary approach to such patients for better outcomes.


 

Article Details

How to Cite
DA SILVA, Guilherme Guilherme Trindade Martins Moreira et al. Sudden Death in a Young Male Patient: When Lupus and Cardiovascular Disease Intersect. Medical Research Archives, [S.l.], v. 10, n. 8, aug. 2022. ISSN 2375-1924. Available at: <https://esmed.org/MRA/mra/article/view/2979>. Date accessed: 28 sep. 2022. doi: https://doi.org/10.18103/mra.v10i8.2979.
Section
Case Reports

References

1. Fortuna G, Brennan MT. Systemic lupus erythematosus. Epidemiology, pathophysiology, manifestations, and management. Dental Clinics of North America. 2013;57(4):631-655. doi:10.1016/j.cden.2013.06.003
2. Yazdany J, Pooley N, Langham J, et al. Systemic lupus erythematosus; stroke and myocardial infarction risk: a systematic review and meta-analysis. RMD Open. 2020;6(2). doi:10.1136/rmdopen-2020-001247
3. Stojan G, Petri M. Atherosclerosis in systemic lupus erythematosus. J Cardiovasc Pharmacol. 2013;62(3):255-262. doi:10.1097/FJC.0b013e31829dd857
4. McLaren JE, Ramji DP. Interferon gamma: a master regulator of atherosclerosis. Cytokine Growth Factor Rev. 2009;20(2):125-135. doi:10.1016/j.cytogfr.2008.11.003
5. Svenungsson E, Fei GZ, Jensen-Urstad K, de Faire U, Hamsten A, Frostegard J. TNF-alpha: a link between hypertriglyceridaemia and inflammation in SLE patients with cardiovascular disease. Lupus. 2003;12(6):454-461. doi:10.1191/0961203303lu412oa
6. López-Pedrera C, Aguirre MÁ, Barbarroja N, Cuadrado MJ. Accelerated atherosclerosis in systemic lupus erythematosus: role of proinflammatory cytokines and therapeutic approaches. J Biomed Biotechnol. 2010;2010. doi:10.1155/2010/607084
7. Hanley DA, Davison KS. Vitamin D Insufficiency in North America. The Journal of Nutrition. 2005;135(2):332-337. doi:10.1093/jn/135.2.332
8. Sager PT, Capece R, Lipka L, et al. Effects of ezetimibe coadministered with simvastatin on C-reactive protein in a large cohort of hypercholesterolemic patients. Atherosclerosis. 2005;179(2):361-367. doi:10.1016/j.atherosclerosis.2004.10.021
9. Vivekananthan DP, Penn MS, Sapp SK, Hsu A, Topol EJ. Use of antioxidant vitamins for the prevention of cardiovascular disease: meta-analysis of randomised trials. Lancet. 2003;361(9374):2017-2023. doi:10.1016/S0140-6736(03)13637-9
10. Reynolds JA, Haque S, Berry JL, et al. 25-Hydroxyvitamin D deficiency is associated with increased aortic stiffness in patients with systemic lupus erythematosus. Rheumatology (Oxford). 2012;51(3):544-551. doi:10.1093/rheumatology/ker352
11. Ridker PM. Testing the inflammatory hypothesis of atherothrombosis: scientific rationale for the cardiovascular inflammation reduction trial (CIRT). J Thromb Haemost. 2009;7 Suppl 1:332-339. doi:10.1111/j.1538-7836.2009.03404.x
12. Ridker PM, Thuren T, Zalewski A, Libby P. Interleukin-1β inhibition and the prevention of recurrent cardiovascular events: rationale and design of the Canakinumab Anti-inflammatory Thrombosis Outcomes Study (CANTOS). Am Heart J. 2011;162(4):597-605. doi:10.1016/j.ahj.2011.06.012
13. Gu MM, Wang XP, Cheng QY, et al. A Meta-Analysis of Cardiovascular Events in Systemic Lupus Erythematosus. Immunological Investigations. 2019;48(5):505-520. doi:10.1080/08820139.2019.1567534
14. Tselios K, Urowitz MB. Cardiovascular and Pulmonary Manifestations of Systemic Lupus Erythematosus. Current Rheumatology Reviews. 2017;13(3). doi:10.2174/1573397113666170704102444
15. Selzer F, Sutton-Tyrrell K, Fitzgerald SG, et al. Comparison of risk factors for vascular disease in the carotid artery and aorta in women with systemic lupus erythematosus. Arthritis Rheum. 2004;50(1):151-159. doi:10.1002/art.11418
16. Doria A, Shoenfeld Y, Wu R, et al. Risk factors for subclinical atherosclerosis in a prospective cohort of patients with systemic lupus erythematosus. Ann Rheum Dis. 2003;62(11):1071-1077. doi:10.1136/ard.62.11.1071
17. Haque S, Skeoch S, Rakieh C, et al. Progression of subclinical and clinical cardiovascular disease in a UK SLE cohort: the role of classic and SLE-related factors. Lupus Science & Medicine. 2018;5(1):e000267. doi:10.1136/lupus-2018-000267
18. McMahon M, Seto R, Skaggs BJ. Cardiovascular disease in systemic lupus erythematosus. Rheumatology and Immunology Research. 2021;2(3):157-172. doi:10.2478/rir-2021-0022
19. Vaarala O, Mänttäri M, Manninen V, et al. Anti-cardiolipin antibodies and risk of myocardial infarction in a prospective cohort of middle-aged men. Circulation. 1995;91(1):23-27. doi:10.1161/01.cir.91.1.23
20. Gustafsson JT, Simard JF, Gunnarsson I, et al. Risk factors for cardiovascular mortality in patients with systemic lupus erythematosus, a prospective cohort study. Arthritis Res Ther. 2012;14(2):R46. doi:10.1186/ar3759
21. Magder LS, Petri M. Incidence of and risk factors for adverse cardiovascular events among patients with systemic lupus erythematosus. Am J Epidemiol. 2012;176(8):708-719. doi:10.1093/aje/kws130
22. Sivakumaran J, Harvey P, Omar A, et al. Assessment of cardiovascular risk tools as predictors of cardiovascular disease events in systemic lupus erythematosus. Lupus Science & Medicine. 2021;8(1):e000448. doi:10.1136/lupus-2020-000448
23. Esdaile JM, Abrahamowicz M, Grodzicky T, et al. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Arthritis Rheum. 2001;44(10):2331-2337. doi:10.1002/1529-0131(200110)44:10<2331::aid-art395>3.0.co;2-i
24. Barile-Fabris L, Hernández-Cabrera MF, Barragan-Garfias JA. Vasculitis in systemic lupus erythematosus. Current Rheumatology Reports. 2014;16(9). doi:10.1007/s11926-014-0440-9
25. Doyle MK. Vasculitis associated with connective tissue disorders. Current Rheumatology Reports. 2006;8(4):312-316. doi:10.1007/s11926-006-0015-5
26. Leone P, Prete M, Malerba E, et al. Lupus vasculitis: An overview. Biomedicines. 2021;9(11). doi:10.3390/biomedicines9111626
27. Alarcon-Segovia D. Vasculitis and the antiphospholipid syndrome. Rheumatology. 2000;39(8):922-923. doi:10.1093/rheumatology/39.8.922
28. Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in Systemic Lupus Erythematosus: prevalence and clinical characteristics in 670 patients. Medicine. 2006;85(2):95-104. doi:10.1097/01.md.0000216817.35937.70
29. Caracciolo EA, Marcu CB, Ghantous A, Donohue TJ, Hutchinson G. Coronary Vasculitis With Acute Myocardial Infarction in a Young Woman With Systemic Lupus Erythematosus. JCR: Journal of Clinical Rheumatology. 2004;10(2):66-68. doi:10.1097/01.rhu.0000111317.80408.16
30. Ocampo-Piraquive V, Nieto-Aristizábal I, Cañas CA, Tobón GJ. Mortality in systemic lupus erythematosus: causes, predictors and interventions. Expert Review of Clinical Immunology. 2018;14(12):1043-1053. doi:10.1080/1744666X.2018.1538789
31. Moghaddam B, Marozoff S, Li L, Sayre EC, Antonio Aviña-Zubieta J. All-cause and Cause-specific Mortality in Systemic Lupus Erythematosus: A Population-based Study. doi:10.1093/rheumatology/keab362/6237935
32. Souza DCC, Santo AH, Sato EI. Mortality profile related to systemic lupus erythematosus: A multiple cause-of-death analysis. Journal of Rheumatology. 2012;39(3):496-503. doi:10.3899/jrheum.110241
33. Tornvall P, Göransson A, Ekman J, Järnbert-Pettersson H. Myocardial Infarction in Systemic Lupus Erythematosus: Incidence and Coronary Angiography Findings. Angiology. 2021;72(5):459-464. doi:10.1177/0003319720985337
34. Jha SB, Rivera AP, Flores Monar GV, et al. Systemic Lupus Erythematosus and Cardiovascular Disease. Cureus. Published online February 8, 2022. doi:10.7759/cureus.22027
35. Kolitz T, Shiber S, Sharabi I, Winder A, Zandman-Goddard G. Cardiac manifestations of antiphospholipid syndrome with focus on its primary form. Frontiers in Immunology. 2019;10(MAY). doi:10.3389/fimmu.2019.00941
36. Nazir S, Tachamo N, Lohani S, Hingorani R, Poudel DR, Donato A. Acute myocardial infarction and antiphospholipid antibody syndrome: A systematic review. Coronary Artery Disease. 2017;28(4):332-335. doi:10.1097/MCA.0000000000000476
37. Cervera R, Serrano R, Pons-Estel GJ, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: A multicentre prospective study of 1000 patients. Annals of the Rheumatic Diseases. 2015;74(6):1011-1018. doi:10.1136/annrheumdis-2013-204838
38. Roman MJ, Shanker BA, Davis A, et al. Prevalence and Correlates of Accelerated Atherosclerosis in Systemic Lupus Erythematosus. New England Journal of Medicine. 2003;349(25):2399-2406. doi:10.1056/NEJMoa035471
39. Denny MF, Thacker S, Mehta H, et al. Interferon-α promotes abnormal vasculogenesis in lupus: a potential pathway for premature atherosclerosis. Blood. 2007;110(8):2907-2915. doi:10.1182/blood-2007-05-089086
40. Rajagopalan S, Somers EC, Brook RD, et al. Endothelial cell apoptosis in systemic lupus erythematosus: a common pathway for abnormal vascular function and thrombosis propensity. Blood. 2004;103(10):3677-3683. doi:10.1182/blood-2003-09-3198
41. Moonen J, de Leeuw K, van Seijen X, et al. Reduced number and impaired function of circulating progenitor cells in patients with systemic lupus erythematosus. Arthritis Research & Therapy. 2007;9(4):R84. doi:10.1186/ar2283
42. Nhek S, Clancy R, Lee KA, et al. Activated Platelets Induce Endothelial Cell Activation via an Interleukin-1β Pathway in Systemic Lupus Erythematosus. Arteriosclerosis, Thrombosis, and Vascular Biology. 2017;37(4):707-716. doi:10.1161/ATVBAHA.116.308126
43. Taraborelli M, Sciatti E, Bonadei I, et al. Endothelial Dysfunction in Early Systemic Lupus Erythematosus Patients and Controls Without Previous Cardiovascular Events. Arthritis Care & Research. 2018;70(9):1277-1283. doi:10.1002/acr.23495
44. Ballocca F, D’Ascenzo F, Moretti C, et al. Predictors of cardiovascular events in patients with systemic lupus erythematosus (SLE): A systematic review and meta-analysis. European Journal of Preventive Cardiology. 2015;22(11):1435-1441. doi:10.1177/2047487314546826
45. Kaiser R, Cleveland CM, Criswell LA. Risk and protective factors for thrombosis in systemic lupus erythematosus: Results from a large, multi-ethnic cohort. Annals of the Rheumatic Diseases. 2009;68(2):238-241. doi:10.1136/ard.2008.093013
46. Chou CH, Lin CL, Chang SN, Lin MC, Kao CH, Huang YJ. A nationwide population-based retrospective cohort study: Increased risk of acute myocardial infarction in systemic lupus erythematous patients. International Journal of Cardiology. 2014;174(3):751-753. doi:10.1016/j.ijcard.2014.04.086
47. Petri M. Epidemiology of the Antiphospholipid Antibody Syndrome. Journal of Autoimmunity. 2000;15(2):145-151. doi:10.1006/jaut.2000.0409
48. Unlu O, Zuily S, Erkan D. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus. European Journal of Rheumatology. 2016;3(2):75-84. doi:10.5152/eurjrheum.2015.0085
49. Mani H. Interpretation of coagulation test results under direct oral anticoagulants. International Journal of Laboratory Hematology. 2014;36(3):261-268. doi:10.1111/ijlh.12235
50. Undas A, Góralczyk T. Direct Oral Anticoagulants in Patients with Thrombophilia: Challenges in Diagnostic Evaluation and Treatment. Advances in Clinical and Experimental Medicine. 2016;25(6):1321-1330. doi:10.17219/acem/65853
51. Karp I, Abrahamowicz M, Fortin PR, et al. Recent corticosteroid use and recent disease activity: Independent determinants of coronary heart disease risk factors in systemic lupus erythematosus? Arthritis & Rheumatism. 2008;59(2):169-175. doi:10.1002/art.23352
52. Patel AM, Karam LAR, Rojas SCF, Redfearn WE, Truong LD, Gonzalez JM. Rapidly Progressive Glomerulonephritis Secondary to IgA Nephropathy in a Patient with Systemic Lupus Erythematosus. Case Reports in Nephrology. 2019;2019. doi:10.1155/2019/8354823
53. Zhang Y miao, Zhou X jie, Wang YN, et al. Shared genetic study gives insights into the shared and distinct pathogenic immunity components of IgA nephropathy and SLE. Molecular Genetics and Genomics. 2021;296(4):1017-1026. doi:10.1007/s00438-021-01798-7