Neonatal lupus erythematosus with congenital heart block in twins

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Published Dec 8, 2016
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Vol. 94 No. 7 (2016): Issue Jul 2016

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Lamia Gargouri
Faiza Safi
Bayen Maalej
Souad Mallek
Fatma Turki
Imen Majdoub
Malek Akrout
Dorra Abid
Samir Kamoun
Abdelmajid Mahfoudh

Abstract

Summary
Background: Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. The most common clinical manifestations are skin rash, cardiac lesions, thrombocytopenia, anemia and hepatosplenomegaly. Complete congenital heart block is usually irreversible needing a pacemaker implantation in two-thirds of cases.
Cases report: We report neonatal lupus erythematosus with complete congenital heart block in twins. Newborns were delivered by caesarean section at week 38 of gestation with a heart rate regular at 70 beats per minute. Both twins and mother were positive for antinuclear, anti-SSA, and anti-SSB antibodies. Twins received single-chamber pacemaker implants at day 12 of life. The evolution was immediately favorable with a heart rate around 110 beats per minute.
The follow-up was 2 years. The twins are currently asymptomatic.
Conclusion: Complete congenital heart block is the most serious manifestation of the neonatal lupus erythematosus associated with significant morbidity and mortality.

Keywords:

lupus erythematosus; congenital heart block; maternal anti-SSA/Ro; newborn

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