Cardiac Involvement in Childhood-Onset Systemic Lupus Erythematosus: A Literature Review of Pathophysiology, Diagnosis, and Management

Marvelle Jocellyne Aubrey Lee¹, Azwin Mengindra Putera^2*

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disorder that arises from a loss of immune tolerance and damages many organs through autoantibodies, immune-complex deposition, and inflammation. Childhood-onset SLE (cSLE) accounts for about 15–20% of cases and follows a more aggressive course than adult-onset disease. The heart is a frequent target: pericarditis, pericardial effusion, myocarditis, Libman–Sacks endocarditis, and conduction abnormalities can involve every cardiac layer, and many of these lesions produce no symptoms. This narrative review summarizes immunopathology, clinical spectrum, detection, and management of cardiac involvement in SLE, with emphasis on children. Endothelial activation, neutrophil extracellular traps, excess type I interferon, dysfunctional T- and B-cell subsets, and antiphospholipid antibodies drive vascular injury, valve damage, and thrombosis. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) and the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria classify the disease, and the SLE Disease Activity Index 2000 (SLEDAI-2K) tracks its activity. Electrocardiography and echocardiography uncover subclinical involvement; in one pediatric cohort, 29.3% of children had electrocardiographic abnormalities, and European pediatric recommendations advise both tests at diagnosis. Management combines hydroxychloroquine for every patient, glucocorticoids reduced to the smallest dose that controls the disease, immunosuppressive agents for organ-threatening disease, anticoagulation for thrombosis, supportive cardiovascular and renal care, and tailored exercise. Routine cardiac screening of children with SLE allows earlier treatment of silent disease.

Keywords

childhood-onset systemic lupus erythematosus; cardiac involvement; pericardial effusion; Libman–Sacks endocarditis; echocardiography; hydroxychloroquine

Cite This Article

Lee, M. J. A., Putera, A. M. (2026). Cardiac Involvement in Childhood-Onset Systemic Lupus Erythematosus: A Literature Review of Pathophysiology, Diagnosis, and Management. International Journal of Scientific Advances (IJSCIA), Volume 7| Issue 6: Nov – Dec 2026, Pages 1041-1047. URL: https://www.ijscia.com/wp-content/uploads/2026/11/Volume7-Issue6-Nov-Dec-No.1127-1041-1047.pdf

Volume 7 | Issue 6: Nov – Dec 2026