A case of neonatal lupus erythematosus with multiorgan involvement and complete heart block

Abstract

Neonatal lupus erythematosus (NLE) is a rare autoimmune- mediated spectrum of disorders occurring in 1/20,000 live births and causing fetal tissue damage due to trans placental passage of anti-Sjögren’s-syndrome-related antigens A and B (anti Ro/SSA and anti La/SSB immunoglobulin G). Approximately 60% of the mothers are asymptomatic on diagnosis of NLE, while the remaining may have SLE, Sjögren syndrome, or other autoimmune disorders. Clinical presentation of NLE varies from dermatologic, cardiac, hepatic, splenic, hematologic, or neurogenic abnormalities. All except cardiac manifestations are reversible and benign. We report a case of NLE in a late preterm infant presenting with multi-organ involvement and congenital complete heart block. The mother was completely asymptomatic but had a significantly high anti-Ro/SSA antibody level. Antenatal fetal echocardiography revealed a structurally normal heart with significant bradycardia and complete heart block. After birth, the infant had multi-organ involvement and persistent bradycardia ranging from 45 to 65bpm with respiratory distress secondary to cardiac decompensation. A permanent epicardial pacemaker was implanted at the age of 2weeks with gradual improvement of respiratory and cardiac functions. Upon follow-up, the infant was thriving well and gaining weight with a stable general condition and reasonable pacemaker function at a rate of 100bpm.