• Lupus Clinical Trials

According to long-term follow-up of patients from the Research Registry for Neonatal Lupus (RRNL), patients with cardiac manifestations of neonatal lupus (NL) are more likely to develop an enlarged heart and the need for pacemaker replacement as they age. The findings emphasize the need for continued long-term cardiac evaluation of patients with cardiac-NL. They were presented by Amit Saxena, MD, New York University School of Medicine, New York, New York, USA, the lead investigator of the analysis [Saxena A et al. ACR 2012 Poster 290].

NL is a model of passively acquired autoimmunity. It occurs when anti-SSA/Ro and/or SSB/La antibodies cross the placenta and make their way into the fetal circulation. The greatest morbidity and mortality occurs from irreversible damage to the heart, for example due to congenital heart block or cardiomyopathy, said Dr. Saxena. Long-term cardiac and rheumatologic outcomes as well as those related to neurodevelopment have not been formally assessed.

Family members of 75 children from the RRNL with cardiac-NL completed questionnaires, as did 35 families of children with cutaneous NL and 74 unaffected siblings. Twenty-four percent of the children were 0 to 5 years old at latest follow-up, 27% were 5 to 10 years old, 11% were 10 to 15 years old, 21% were 15 to 20 years old, and 17% were >20 years old. Eighty-five percent of the children had third-degree heart block.

Questionnaire responses revealed that 74% of the children had a pacemaker implanted and 65% needed a replacement pacemaker. Forty-one percent of family members had ever been told that their child had a dilated heart and 20% had ever been told that their child had congestive heart failure. One child had undergone heart transplant.

When cardiac outcomes were examined by age group, older age was associated with a reported history of an enlarged heart (p=0.016), a greater need for pacing (p=0.001), and a greater likelihood of having a pacemaker replaced (p=0.003; Table 1). Of the 37 children with cardiac-NL who were aged >10 years, 19% required cardiac medications (ie, digoxin, angiotensin-converting enzyme inhibitors, or β-blockers) compared with 5% of those aged <10 years (p=0.086).

Children who had dilated cardiomyopathy or valvular disease on their fetal echocardiogram had significantly more pacemakers replaced as they got older compared with those without these features on their fetal echocardiogram (p<0.05).

Twelve percent of children with cardiac-NL had delayed motor milestones compared with only 3% of unaffected siblings (p=0.027), but there was no difference in intellectual delay between these groups (19% vs 15%, respectively).

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