Cardiac Tamponade as The Initial Presentation of Childhood Systemic Lupus Erythematosus: A Case Report

Main Article Content

Kevin Bassey
Frances SamOkpokowuruk
Ifunanya Ularinma Ebiekpi
Idorenyin Diana Etebong

Keywords

Cardiac Tamponade, Pericardial Effusion, Systemic Lupus Erythematosus

Abstract

Systemic Lupus erythematosus (SLE) is an autoimmune disorder characterized by the proliferation of autoantibodies and immune dysregulation resulting in damage to many body organs. Pediatric SLE usually presents with fever, joint pain, rashes, and lupus nephritis. It is uncommon to have large pericardial effusions in children with SLE and cardiac tamponade as the initial presentation of SLE is even rarer.


An 11-year-old female presented to our Children Emergency Unit with fever and fast breathing for two weeks, bilateral leg swelling of four days, and cough of two days duration. She was acutely ill, tachypneic, and dyspneic with marked orthopnea, bilateral leg edema, and raised JVP. She was tachycardic with a diffuse apex beat. Chest X-ray showed a large globular heart. 2D-Echocardiography showed a large circumferential pericardial effusion with a dilated non-collapsing IVC and diastolic collapse of the right ventricle. She had a pericardiotomy done and 650mls of serous pericardial fluid was drained. The inner pericardium had a fibrinoid exudate with a “bread-and-butter” appearance. Pericardial fluid cytology showed no malignant cells while pericardial biopsy showed suppurative granulomatous inflammation. Antinuclear antibody (ANA) was strongly positive. The patient was managed with corticosteroids, colchicine, and hydroxychloroquine, and has remained stable on follow-up.


While cardiac tamponade as an initial presenting complaint in SLE is rare, it is important that children presenting with large pericardial effusions and tamponade be evaluated for rheumatologic disorders. This can be crucial to revealing the correct diagnosis and instituting appropriate care.

Abstract 31 | PDF Downloads 25 EPUB Downloads 9 HTML Downloads 7

References

1. Klein-Gitelman MS. Pediatric systemic lupus erythematosus. Medscape. Available online at https://emedicine.medscape.com/article/1008066-overview#a5. Accessed 22/09/22

2. Briggs DC, Aitafo JE. Childhood-onset systemic lupus erythematosus with lupus nephritis in a 38-month-old Nigerian Child–a case report. African Journal of Paediatric Nephrology. 2021;8(2):53-8.

3. Levy DM. Childhood-onset systemic lupus erythematosus: clinical manifestations and diagnosis. UpToDate. Available online at https://www.uptodate.com/contents/childhood-onset-systemic-lupus-erythematosus-sle-clinical-manifestations-and-diagnosis. Accessed 22/09/22

4. Peñataro JS, De Mingo A, Del Río A, Martínez JA, Heras M, Regueiro A. Myopericarditis and severe myocardial dysfunction as the initial manifestation of systemic lupus erythematosus. European Heart Journal: Acute Cardiovascular Care. 2012;1(3):253-5.

5. Maharaj SS, Chang SM. Cardiac tamponade as the initial presentation of systemic lupus erythematosus: a case report and review of the literature. Pediatric Rheumatology. 2015;13(1):1-7.

6. Bezwada P, Quadri A, Shaikh A, Ayala-Rodriguez C, Green S. Myopericarditis and pericardial effusion as the initial presentation of systemic lupus erythematosus. Case Reports in Medicine. 2017;2017.

7. Cheo SW, Low QJ. Massive pericardial effusion - An uncommon initial presentation of systemic lupus erythematosus (SLE). Med J Malaysia. 2019;74(5):439-440. PMID: 31649224.

8. Harrison MJ, Zühlke LJ, Lewandowski LB, Scott C. Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations. Pediatr Rheumatol Online J. 2019;17(1):76. doi: 10.1186/s12969-019-0382-x

9. Weich HS, Burgess LJ, Reuter H, Brice EA, Doubell AF. Large pericardial effusions due to systemic lupus erythematosus: a report of eight cases. Lupus. 2005;14(6):450-7. doi: 10.1191/0961203305lu2131oa.

10. Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G. Derivation, and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis & Rheumatism. 2012;64(8):2677-86.

11. Goswami RP, Sircar G, Ghosh A, Ghosh P. Cardiac tamponade in systemic lupus erythematosus. QJM: An International Journal of Medicine. 2018;111(2):83-7.

12. Roy CL, Minor MA, Brookhart MA, Choudhry NK. Does This Patient with a Pericardial Effusion Have Cardiac Tamponade? JAMA. 2007;297(16):1810–1818. doi:10.1001/jama.297.16.1810

13. Ristić AD, Imazio M, Adler Y, Anastasakis A, Badano LP, Brucato A, Caforio AL, Dubourg O, Elliott P, Gimeno J, Helio T. Triage strategy for urgent management of cardiac tamponade: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. European Heart Journal. 2014;35(34):2279-84.

14. Kumar MA, Sathyamurthy I, Jayanthi K. Systemic lupus erythematosus presenting as cardiac tamponade—a case report. Indian Heart Journal. 2012;64(1):106-7.

15. Noubiap JJ, Agbor VN, Ndoadoumgue AL, Nkeck JR, Kamguia A, Nyaga UF, Ntsekhe M. Epidemiology of pericardial diseases in Africa: a systematic scoping review. Heart. 2019;105(3):180-8.

16. Malgope R, Basu S, Sinha MK. Clinico-Etiological Profile of Children with Pericardial Effusion in a Tertiary Care Hospital in Eastern India. Journal of Tropical Pediatrics. 2021;67(1): fmaa118. 

17. Maharaj S, Chang S. Consideration of C-reactive protein and polyserositis in systemic lupus erythematosus presenting with cardiac symptoms. Am J Emerg Med. 2015;33(1):115–6.

18. Samohvalov E, Samohvalov S. The pattern of anemia in lupus. Current Topics in Anemia. 2018;165.

19. Bae S-C, Fraser P, Liang MH. The epidemiology of systemic lupus erythematosus in populations of African ancestry: a critical review of the “prevalence gradient hypothesis”. Arthritis Rheum. 1998; 41:2091–99.

20. Vento S, Cainelli F. Commentary: systemic lupus erythematosus in native sub-Saharan Africans: a systematic review and meta-analysis. Frontiers in Medicine. 2020; 7:202.

21. Aregbeshola BS, Khan SM. Out-of-pocket health-care spending and its determinants among households in Nigeria: a national study. Journal of Public Health. 2021; 29:931-42.

22. Sag E, Tartaglione A, Batu ED, Ravelli A, Khalil SMA, Marks SD, et al. Performance of the new SLICC classification criteria in childhood systemic lupus erythematosus: a multicentre study. Clin Exp Rheumatol. 2014;32(3):440–4.

Most read articles by the same author(s)