Endocardial involvement as the onset of systemic lupus erythematosus: own observation and literature review
Keywords:systemic lupus erythematosus, Libman-Sacks endocarditis, nonbacterial thrombotic endocarditis, diagnosis, treatment, review
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that is characterized by inflammation and damage to many systems. Cardiac involvement in SLE is one of the many possible manifestations of this polymorphic disease and occurs in more than 50 % of these patients, affecting all structural components of the heart. The endocardium involvement in SLE is a rather rare, but at the same time it is difficult clinical manifestation for the correct interpretation of nosological affiliation. This is due, on the one hand, to the atypical nature of this lesion in SLE, when patients visit cardiologists, therapists, cardiac surgeons, receiving only symptomatic help, as in our clinical case. On the other hand, using the new classification criteria for SLE, it is possible to detect this pathology with a high degree of certainty and begin an adequate basic therapy that can lead to persistent clinical and laboratory remission and prevent the involvement of other organs and systems in the process. This paper presents a clinical observation of the SLE onset from valve lesions, late diagnosis and nosological verification of lupus endocarditis (Libman-Sacks endocarditis). A review of the literature on the management of patients with nonbacterial thrombotic endocarditis (NBTE) caused by the formation of sterile vegetations on the heart valves without any signs of infection due to endothelial damage in hypercoagulable conditions was carried out. The most common causes of NBTE are SLE, antiphospholipid syndrome, and malignant neoplasms. NBTE is usually asymptomatic and is often an incidental finding during examination or autopsy. Vegetations are more often localized on the valves of the left heart (mitral and less often aortic), but other valves can also be affected. Typical clinical manifestations are due to embolism. Treatment of these patients is based on the use of anticoagulant therapy and treatment of the underlying disease, although prospective studies have not been conducted.
Patino Giraldo S, González Naranjo LA, Vasquez Duque GM, Restrepo Escobar M. Heart disease characteristics in patients with systemic lupus erythematosus. Iatreia. 2013;26(4):447-56.
Ishimori ML, Martin R, Berman DS, Goykhman P, Shaw LJ, Shufelt CM, et al. Myocardial ischemia in the absence of obstructive coronary artery disease in systemic lupus erythematosus. JACC Cardiovasc Imaging. 2011;4(1):27-3. https://doi.org/10.1016/j.jcmg.2010.09.019
Clarke AE, Urowitz MB, Monga N, Hanly JG. Costs associated with severe and non-severe SLE in Canada. Arthritis Care Res (Hoboken). 2014; 67(3):431-6. doi: 10.1002/acr.22452.
Aringer M, Costenbader KH, Daikh DI, et al. 2019 EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus Arthritis Rheumatol. 2019; 71(9): 1400–1412. doi: 10.1002/art.40930
Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus Annals of the Rheumatic Diseases 2019; 78(6):736-745. doi: 10.1136/annrheumdis-2019-215089.
Yoo BW, Lee SW, Song JJ, Park YB, Jung SM. Clinical characteristics and long-term outcomes of Libman-Sacks endocarditis in patients with systemic lupus erythematosus. Lupus. 2020;29(9):1115-1120. doi: 10.1177/0961203320930097.
Kousa O, Baskaran J, Ahmad A, Awad DH, Aboeata A. A Transesophageal Echocardiogram Finding: From Infection to Malignancy. Cureus. 2020 Feb 05;12(2):e6886. DOI: 10.7759/cureus.6886.
Ibrahim AM, Siddique MS. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Sep 10, 2020. Libman Sacks Endocarditis.
Ginanjar E, Yulianto Y. Autoimmune Disease with Cardiac Valves Involvement: Libman-Sacks Endocarditis. Acta Med Indones. 2017 Apr;49(2):148-150.
Lenz CJ, Mankad R, Klarich K, Kurmann R, McBane RD. Antiphospholipid syndrome and the relationship between laboratory assay positivity and prevalence of non-bacterial thrombotic endocarditis: A retrospective cohort study. J Thromb Haemost. 2020 Jun;18(6):1408-1414. doi: 10.1111/jth. 14798.
Ruiz D, Oates JC, Kamen DL Antiphospholipid Antibodies and Heart Valve Disease in Systemic Lupus Erythematosus. Am J Med Sci. 2018;355(3):293. doi: 10.1016/j.amjms.2017.07.007.
Zuily S, Regnault V, Selton-Suty C, et al. Increased risk for heart valve disease associated with antiphospholipid antibodies in patients with systemic lupus erythematosus: meta-analysis of echocardiographic studies. Circulation. 2011;124(2):215. doi.org/10.1161/CIRCULATIONAHA.111.028522.
Hussain K, Gauto-Mariotti E, Cattoni HM, et al. A Meta-analysis and Systematic Review of Valvular Heart Disease in Systemic Lupus Erythematosus and Its Association With Antiphospholipid Antibodies. J Clin Rheumatol. 2020; doi: 10.1097/RHU.0000000000001464. Online ahead of print.
Roldan CA, Sibbitt WL, Qualls CR, et al. Libman-Sacks endocarditis and embolic cerebrovascular disease. JACC Cardiovasc Imaging. 2013 Sep;6(9):973-83. doi: 10.1016/j.jcmg.2013.04.012.
Roldan CA, Qualls CR, Sopko KS, Sibbitt WL. Transthoracic versus transesophageal echocardiography for detection of Libman-Sacks endocarditis: a randomized controlled study. J Rheumatol. 2008;35(2):224. Epub 2007 Dec 15.
Whitlock RP, Sun JC, Fremes SE, Rubens FD, Teoh KH, American College of Chest Physicians Antithrombotic and thrombolytic therapy for valvular disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012;141(2 Suppl):e576S. doi: 10.1378/chest.11-2305.
Sonsöz MR, Tekin RD, Gül A, Buğra Z, Atılgan D. Treatment of Libman-Sacks endocarditis by combination of warfarin and immunosuppressive therapy. Turk Kardiyol Dern Ars. 2019 Dec;47(8):687-690. doi: 10.5543/tkda.2019.29213.
Fujimoto D, Mochizuki Y, Nakagiri K. Unusual rapid progression of non-bacterial thrombotic endocarditis in a patient with bladder cancer despite undergoing intensification treatment with rivaroxaban for acute venous thromboembolism. J Eur Heart J. 2018;39(43):3907. doi: 10.1093/eurheartj/ ehy569.
Golovach IYu, Yehudina YeD. Cardiovascular Manifestations in Patients with Antiphospholipid Syndrome: Diagnostics and Management. Arterial Hypertension. 2019;5-6:67-68. doi 10.22141/2224-1485.5-6.66-67.2019.186046.
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