A case of neonatal lupus presenting with severe systemic hypertension and multiorgan failure in absence of congenital heart block (CHB): clinical management and recent literature review of neonatal cardiac lupus

Abstract

Neonatal Lupus (NLE) is a rare acquired autoimmune disorder caused by transplacental passage of maternal autoantibodies to Sjogren’s Syndrome A or B (SSA-SSB) autoantigens1 which target fetal and neonatal tissues for immune destruction. The cardiac trademark of NLE is autoimmune heart block, which accounts for more than 80% of cases of complete atrioventricular heart-block (AVB) in newborns with a structurally normal heart2. NLE presenting with cardiac alterations not involving rhythm disturbances are described in the literature, but they are rare. Here, we report a case of a neonate with high anti-SSA antibodies who developed severe systemic hypertension and transient ventricular dysfunction in absence of the most common cardiac presentations of NLE: rhythm abnormalities, endocardial fibroelastosis, and dilated cardiomyopathy3. The patient developed severe multiorgan dysfunction syndrome that required prolonged critical care support but fully recovered and was discharged home. We highlight the unusual clinical features of this NLE case and the importance of timely treatment of NLE allowing complete recovery of a critically ill neonate.