[Neonatal lupus syndromes].
Children born from mothers positive for autoantibodies against SSA/Ro and/or anti-SSB/La ribonucleoproteins may develop heart conduction tissue damage resulting in atrioventricular block and/or transient skin rash, liver enzyme abnormalities and anaemia/thrombocytopenia. Additional transient electrocardiographic abnormalities (sinus bradycardia, QT interval prolongation) have been reported. Such clinical and laboratory manifestations are included in the so-called neonatal lupus syndromes, independently whether the mother is suffering from a systemic autoimmune disease or is totally asymptomatic. The prevalence of the congenital heart block is around 2%, of neonatal rash around 20%, while laboratory abnormalities in asymptomatic babies can be detected in up to 40% of cases. The risk of recurrence of complete heart block is almost 10 times higher in the following pregnancies. Most of the mothers are asymptomatic at delivery and are identified only by the birth of an affected child. Their long-term outcome is generally more reassuring than previously assumed. Serial echocardiograms and obstetric sonograms, performed at least every 2 weeks, starting from 16 weeks gestation, are recommended in anti-Ro/SSA positive pregnant women: the goal is to detect early fetal abnormalities, that might precede complete atrioventricular block and that might be a target for preventive therapy. Transplacental passage of maternal anti-SSA/Ro -SSB/La IgG is thought to be pivotal in inducing tissue damage. However, the discordant appearance of the syndrome in twins does suggest a role also for fetal or environmental factors.
Cimaz
R
R
Département de Pédiatrie, Hôpital Edouard-Herrio, 69437 Lyon cedex 03, France. roland.cimaz@chu-lyon.fr
Duquesne
A
A
D006801:Humans; D007231:Infant, Newborn; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D011379:Prognosis