Major Finding: Of 325 children enrolled in the Research Registry for Neonatal Lupus before October 2010, 57 (18%) died; 30% died in utero, 30% died during the neonatal period, 14% died between 1 and 6 months of age, and 26% died after 6 months of age.
Data Source: A retrospective analysis of data from a large U.S.-based cohort.
Disclosures: Dr. Izmirly had no disclosures.
ATLANTA — The overall case fatality rate in cardiac neonatal lupus is nearly 18%, according to a review of data from the Research Registry for Neonatal Lupus.
Of 325 children enrolled in the large U.S.-based registry before October 2010, 57 (18%) died; 30% died in utero, 30% died during the neonatal period, 14% died between 1 and 6 months of age, and 26% died after 6 months of age, Dr. Peter M. Izmirly reported at the meeting.
Of the deaths, 42 were cardiac related – most often a result of complications from cardiomyopathy, 6 were due to infectious complications, and 8 were a result of unknown causes. One pregnancy was terminated electively, said Dr. Izmirly of New York University, New York.
“There was a significantly higher case fatality rate in minorities, compared with Caucasians,” Dr. Izmirly said, noting that 14% of white children with cardiac neonatal lupus died, compared with 28% of children belonging to minority groups.
The study, which was conducted in an effort to update morality data on cardiac neonatal lupus and to thereby improve evidence-based counseling of anti-Ro/La positive mothers whose babies are at increased risk of cardiac neonatal lupus, identified fetal and maternal risk factors for death in affected babies.
Significant fetal risk factors for death were associated hematologic hepatic neonatal lupus (present in 27% vs. 7% of deceased vs. living babies), earlier gestational age at detection (detection occurred at 21.8 vs. 23.4 weeks in deceased vs. living babies), delivery prior to 37 weeks' gestation (delivery occurred prior to 37 weeks in 69% vs. 42% of deceased vs. living babies), and earlier gestational week of delivery (delivery occurred at 34.2 weeks vs. 36.9 weeks in deceased vs. living babies), Dr. Izmirly said.
Fetal risk factors not found to be associated with mortality were female sex, associated neonatal lupus rash, cesarean section delivery, and year of birth.
Fetal echocardiographic risk factors associated with mortality were lower ventricular rate nadir (rate was 50.2 vs. 53.6 in deceased vs. living babies), and the presence of endocardial fibroelastosis (which occurred in 30.25% vs. 4.3% of deceased vs. living babies), dilated cardiomyopathy (which occurred in 32.6% vs. 8.6% of deceased vs. living babies), hydrops (which occurred in 57.4% vs. 3.4% of deceased vs. living babies), and valvular disease (which occurred in 18.2% vs. 4.8% of deceased vs. living babies).
Fetal echocardiographic factors not associated with mortality were ventricular rate detection, atrial septal defect, ventricular septal defect, and patent ductus arteriosus.
Only one maternal risk factor – a maternal diagnosis of systemic lupus erythematosus or Sjögren's syndrome – showed a trend toward significance in terms of risk for fetal death. Diagnosis occurred in 56% of women whose babies died, vs. 43% of those whose babies were living.
Maternal age, maternal anti-La antibodies, maternal anti–52-kD Ro antibodies, and use of nonfluorinated steroids, fluorinated steroids, terbutaline, or hydroxychloroquine were not associated with increased risk of fetal mortality.
As for morbidity in affected children, 70% required pacing – including 1% in utero, 53% in the neonatal period, 12% between ages 1 and 6 months, and 32% after age 6 months. The timing was unknown in 3% of cases.
Also, four children required cardiac transplantation, including one child who required two transplants, Dr. Izmirly said.
“Cardiac neonatal lupus is associated with substantial mortality, which is predicted by slower heart rates and echocardiographic abnormalities consistent with antibody-associated disease beyond the AV node,” Dr. Izmirly concluded, adding that the disparity in outcomes between whites and minorities suggests that attention should be given to “an inherent difference in organ response to injury, and/or access to care.”