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

Abstract

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.

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

“…SLE nephritis was identified using a validated algorithm, 11,12 and we have previously described methods for categorization of demographic characteristics and identification of other disease characteristics in this database. 13,18…”

“…Since regional differences in echocardiography use influence cardiac disease detection rates, clustering by U.S. census region was accounted for. 18…”

“…SLE nephritis was identified using a validated algorithm, 11,12 and we have previously described methods for categorization of demographic characteristics and identification of other disease characteristics in this database. 13,18 Statistical analysis Demographic and disease characteristics among children and adults with SLE were assessed using standard descriptive statistics and compared using Pearson's chi-squared tests. Prevalence rates for each cardiac manifestation were calculated using Poisson regression, with the outcome being at least one cardiac diagnosis code between 12 months before SLE diagnosis and the end of enrollment, and total SLE cases as the offset.…”

“…Since regional differences in echocardiography use influence cardiac disease detection rates, clustering by US census region was accounted for. 18 Comparisons of unadjusted incidence rates were performed using mid-p exact tests. Separate negative binomial regression models adjusted for race/ ethnicity, sex, noncardiac organ involvement, and antiphospholipid antibodies/venous thromboembolism were used to determine whether age of SLE onset is independently associated with myopericardial involvement or valvular disease.…”

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

“…Pediatric SLE patients have been reported to have lower LVEF than healthy age-matched controls that represents cardiac dysfunction even when LVEF is within normal range; therefore, an abnormal LVEF is a concerning finding in our cohort [47]. Studies in both adults and children with SLE demonstrate a high prevalence of subclinical cardiac disease, leading many to motivate for routine use of echocardiography [2, 48–50]. As children with SLE have a higher cumulative risk of damage accrual, and risk factors for cardiac involvement in these patients remain unknown, routine echocardiogram may be of greater benefit in the pediatric population, although formal guidelines regarding the frequency of monitoring are lacking.…”