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

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 years) with incident SLE (≥3 International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9 CM) code 710.0, > 30 days apart) using Clinformatics® DataMart (OptumInsight, Eden Prairie, MN) deidentified United States 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 vs adulthood was independently associated with cardiac involvement. RESULTS: There were 297 children and 6927 adults with new-onset SLE. A total of 17.8% of children had ICD-9 CM 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% confidence interval (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 adult-onset 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.

Use of echocardiography at diagnosis and detection of acute cardiac disease in youth with systemic lupus erythematosus.

Objectives There are no guidelines on the use of echocardiography to detect cardiac manifestations of childhood-onset systemic lupus erythematosus (SLE). We quantify the prevalence of acute cardiac disease in youth with SLE, describe echocardiogram utilization at SLE diagnosis, and compare regional echocardiogram use with incident cardiac diagnoses. Methods Using the Clinformatics® DataMart (OptumInsight, Eden Prairie, MN) de-identified United States administrative database from 2000 to 2013, we identified youth ages 5-24 years with new-onset SLE (≥3 ICD-9 SLE codes 710.0, > 30 days apart) and determined the prevalence of diagnostic codes for pericardial disease, myocarditis, endocarditis, and valvular insufficiency. Multiple logistic regression was used to identify factors associated with echocardiography during the baseline period, up to one year before or six months after SLE diagnosis. We calculated a regional echocardiogram utilization index, which is the ratio of observed use over the mean predicted probability based on all available baseline characteristics. Spearman's rank correlation coefficient was used to evaluate the association between regional echocardiogram utilization indices and percentage of imaged youth diagnosed with their first cardiac manifestation following echocardiography. Results Among 699 youth with new-onset SLE, 18% had ≥ 1 diagnosis code for acute cardiac disease, of which valvular insufficiency and pericarditis were most common. Twenty-five percent of all youth underwent echocardiogram during the baseline period. Regional echocardiogram use was positively correlated with the percentage of imaged youth found to have cardiac disease (ρ = 0.71, p = 0.05). There was up to a five-fold difference in adjusted odds of baseline echocardiography between low- and high-utilizing regions (OR = 0.19, p = 0.007). Conclusion Nearly one-fifth of youth with new-onset SLE have acute cardiac manifestations; however, use of echocardiograms at SLE diagnosis is highly variable. There may be incremental diagnostic value to early use of echocardiography, but prospective studies are needed to determine whether greater use of echocardiograms modifies outcomes.