Abstract
BackgroundPediatric onset of systemic lupus erythematosus (SLE) is associated with major organ involvement, and African patients tend to develop more aggressive disease than patients of European descent. Although cardiovascular involvement is common in pediatric SLE, there are few published reports on the subject. This study describes the frequency and characteristics of cardiac and vascular manifestations of pediatric SLE in a multi-ethnic South African cohort.MethodsDemographic, clinical, and echocardiographic data were collected from pediatric SLE patients at two centers in Cape Town, South Africa. At the time of investigation, this cohort consisted of 93 participants diagnosed with SLE according to international classification criteria prior to the age of 19. Individuals with cardiac and/or vascular involvement were identified by retrospective chart review. Cardiac manifestations were defined as presence of pericardial effusion, myocarditis, cardiomyopathy, cardiac failure, Libman-Sacks endocarditis, myocardial infarction, and arrhythmia. Vascular manifestations included deep vein thrombosis, pulmonary embolism, sinus thrombosis, stroke, critical limb ischemia, cerebral vasculitis and systemic vasculitis. Statistical analysis was performed using R (v3.4.1).ResultsCardiac and vascular involvement was present in 47% of the cohort. Previous studies have reported prevalence of 5%—50%. Demographic features of those with cardiac/vascular involvement did not differ from the overall cohort. Echocardiographic data were available for 23 participants. The most common cardiac manifestations were pericardial effusion (n = 24) and cardiac failure (n = 8), while the most common vascular manifestations were cerebral vasculitis (n = 9), stroke (n = 7), and pulmonary embolism (n = 7). Cardiovascular manifestations were frequently severe; one third of pericardial effusion cases required intervention, including three cases of cardiac tamponade. Cardiac and vascular involvement conferred an increased risk of mortality (31.1% versus 10.4%).ConclusionsCardiac and vascular involvement were highly prevalent in this South African cohort. The mortality rate was high, and severe manifestations were frequent. Prospective research is needed to improve knowledge of pediatric SLE in Africa and to improve outcomes for this high-risk population.
Highlights
Pediatric onset of systemic lupus erythematosus (SLE) is associated with major organ involvement, and African patients tend to develop more aggressive disease than patients of European descent
A study of 33 Pediatric-onset SLE (pSLE) patients in Trinidad reported that 24% had pericarditis at presentation and 6% had a diagnosis of stroke [19]
We evaluated enrollment SLEDAI-2 K scores of the cardiac/vascular cohort compared to the entire South African cohort
Summary
Pediatric onset of systemic lupus erythematosus (SLE) is associated with major organ involvement, and African patients tend to develop more aggressive disease than patients of European descent. This study describes the frequency and characteristics of cardiac and vascular manifestations of pediatric SLE in a multi-ethnic South African cohort. Pediatric-onset SLE (pSLE) is associated with greater mortality and a higher rate of major organ involvement in comparison to SLE in adults [4,5,6]. Cardiovascular manifestations are more frequently reported in patients of African origin, in both adult and pediatric populations [7, 8]. Few published reports on cardiovascular complications of SLE in African populations exist [21,22,23], a recent study described high severity of lupus myocarditis in adult SLE patients in Cape Town [24]. A similar study of 50 adult patients in Senegal reported 3 cases of cardiomyopathy and 19 cases of pericarditis, 2 with tamponade physiology [25]
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