Background Cardiac manifestations of neonatal lupus (cardiac-NL) include conduction disease and rarely an isolated cardiomyopathy. This study was initiated to determine the mortality and morbidity of cardiac-NL and associated risk factors in a multi-racial/ethnic US-based registry to provide insights into the pathogenesis of antibody mediated injury and data for counseling. Methods and Results Three hundred and twenty-five offspring exposed to maternal anti-SSA/Ro antibodies with cardiac-NL met entry criteria. Maternal, fetal echocardiographic, and neonatal risk factors were assessed for association with mortality. Fifty-seven (17.5%) died; 30% in utero. The probability of in utero death was 6%. The cumulative probability of survival at 10 years for a child born alive was 86%. Fetal echocardiographic risk factors associated with increased mortality in a multivariable analysis of all cases included hydrops and endocardial fibroelastosis (EFE). Significant predictors of in utero death were hydrops and earlier diagnosis, and for postnatal death, hydrops, EFE, and lower ventricular rate. Isolated heart block was associated with a 7.8% case fatality rate whereas the concomitant presence of dilated cardiomyopathy or EFE quadrupled the case fatality rate. There was a significantly higher case fatality rate in minorities compared to Caucasians, who were at a lower risk of hydrops and EFE. Pacing was required in 70% and cardiac transplantation in four children. Conclusion Nearly one-fifth of fetuses who develop cardiac-NL die from complications which are predicted by echocardiographic abnormalities consistent with antibody associated disease beyond the AV node. The disparity in outcomes observed between minorities and Caucasians warrants further investigation.

Objective. The Manhattan Lupus Surveillance Program (MLSP) is a population-based registry designed to determine the prevalence of systemic lupus erythematosus (SLE) in 2007 and the incidence from 2007 to 2009 among residents of New York County (Manhattan), New York, and to characterize cases by race/ethnicity, including Asians and Hispanics, for whom data are lacking.Methods. We identified possible SLE cases from hospital records, rheumatologist records, and administrative databases. Cases were defined according to the American College of Rheumatology (ACR) classification criteria, the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria, or the treating rheumatologist's diagnosis. Rates among Manhattan residents were age-standardized, and capture-recapture analyses were conducted to assess case underascertainment.Results. By the ACR definition, the agestandardized prevalence and incidence rates of SLE were 62.2 and 4.6 per 100,000 person-years, respectively. Rates were~9 times higher in women than in men for prevalence (107.4 versus 12.5) and incidence (7.9 versus 1.0). Compared with non-Hispanic white women (64.3), prevalence was higher among non-Hispanic black (210.9), Hispanic (138.3), and non-Hispanic Asian (91.2) women. Incidence rates were higher among non-Hispanic black women (15.7) compared with non-Hispanic Asian (6.6), Hispanic (6.5), and non-Hispanic white (6.5) women. Capture-recapture adjustment increased the prevalence and incidence rates (75.9 and 6.0, respectively). Alternate SLE definitions without capture-recapture adjustment revealed higher age-standardized prevalence and incidence rates (73.8 and 6.2, respectively, by the SLICC definition and 72.6 and 5.0 by the rheumatologist definition) than the ACR definition, with similar patterns by sex and race/ethnicity.Conclusion. The MLSP confirms findings from other registries on disparities by sex and race/ethnicity, provides new estimates among Asians and Hispanics, and provides estimates using the SLICC criteria.Systemic lupus erythematosus (SLE) is a potentially fatal, heterogeneous, chronic, systemic autoimmune disease of unknown etiology (1). Given widely The views expressed herein are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.