Child-onset systemic lupus erythematosus is associated with a higher incidence of myopericardial manifestations compared to adult-onset disease

Abstract

Objectives: There are no population-based estimates of the incidence or risk factors for acute cardiac manifestations in children with systemic lupus erythematosus (SLE) to guide screening and diagnostic imaging practices. We estimated the incidence and prevalence of acute cardiac manifestations of child-onset SLE compared to adult-onset SLE and identified factors associated with cardiac diagnoses. Methods: We identified children (5–17 years) and adults (18–64) with incident SLE (≥3 ICD-9 codes 710.0, >30 days apart) using Clinformatics® DataMart (OptumInsight, Eden Prairie, MN) de-identified U.S. administrative claims (2000–2013). We calculated incidence and prevalence of three outcomes: ≥1 diagnosis code for 1) pericarditis and/or myocarditis, 2) endocarditis or 3) valvular insufficiency. Negative binomial regression was used to identify characteristics associated with cardiac diagnoses in children and determine whether SLE onset in childhood versus adulthood was independently associated with cardiac involvement. Results: There were 297 children and 6927 adults with new-onset SLE. 17.8% of children had ICD-9 codes for acute cardiac diagnoses, the incidence of which were highest in the first year after SLE diagnosis (12.2 per 100 person-years). African American race (incidence rate ratio (IRR) 6.6, 95% CI [2.9, 15.0], p<0.01) and nephritis (IRR 7.0, 95% CI [2.6, 18.6], p<0.01) were associated with acute cardiac diagnoses in children. Child-onset disease was independently associated with a 4.4-fold higher rate of pericarditis or myocarditis compared to adult-onset SLE after adjustment for other disease and demographic characteristics (95% CI [2.4, 8.0], p<0.01). Conclusion: This study establishes baseline estimates of the incidence and prevalence of pericarditis and myocarditis in child-onset SLE, which is substantially higher than that of adultonset SLE. Prospective echocardiographic evaluations are needed to validate incidence measures and characterize the natural history of acute cardiac manifestations in child-onset SLE, as well as identify risk factors for poor cardiac outcomes to inform screening and management.

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

“…Cardiac and vascular findings were reported in 31.2 and 29.0% of patients in the PULSE cohort, respectively. This is consistent with the reported prevalence (12%—48%) of cardiovascular involvement in pediatric studies [1, 2, 4, 7]. Variation in the prevalence reported in the literature may be explained by single-center design, small sample size, variation in study definitions of cardiovascular involvement, and methodological differences with variable sensitivity for the detection of subclinical manifestations.…”

“…Data from a large, recently published, population-based study of cardiovascular disease in pSLE patients in the USA were included for comparison [7]. This study utilised a large administrative database to quantify cardiac involvement in a sample of children and adults with a new diagnosis of SLE between 2000 and 2013.…”

“…Pediatric-onset SLE (pSLE) is associated with greater mortality and a higher rate of major organ involvement in comparison to SLE in adults [4–6]. Although cardiovascular outcomes are not well described in this group, cardiovascular disease is emerging as an important cause of mortality in individuals with pSLE [6, 7]. Cardiovascular manifestations are more frequently reported in patients of African origin, in both adult and pediatric populations [7, 8].…”

“…Acute cardiac and vascular presentations of SLE, such as cardiac tamponade and stroke, are associated with significant mortality; however, a greater burden is likely exerted by the chronic cardiovascular effects of SLE in individuals surviving into adulthood [7, 9]. SLE patients develop atherosclerosis earlier and more rapidly than age- and sex-matched controls [10–12].…”

“…Pericarditis is the most commonly reported cardiac manifestation across the literature. However, its frequency appears to vary significantly across different settings, with individual studies reporting rates of 0.5–36.3%, depending on the setting [7, 16–19]. A large multicenter UK cohort reported only 20% of pSLE presented with serositis (but did not distinguish between pleuritis and pericarditis) and less than 0.01% of patients developed cardiomyopathy, while a population-based study of nearly 300 pSLE patients in the USA reported a diagnosis of pericarditis in 10% of patients [7, 16].…”