Typical clinical presentation of infants with neonatal lupus erythematosus

Typical clinical presentation of infants with neonatal lupus erythematosus and what is the pathogenesis. What treatment options exist?

SkinSubacute SLE rash a
LiverElevation of liver function tests/hepatosplenomegaly
HematologicCytopenias/hemolytic anemia/thrombocytopenia
CardiacHeart block/endocardial fibroelastosis

Most noncardiac manifestations clear in approximately 6 months as this is when the antibodies degrade.

a Some patients with NLE and skin rash have ribonucleoprotein antibodies only.

Congenital heart block (CHB) is the most common cardiac manifestation of NLE, and the accompanying myocarditis accounts for the major morbidity and mortality.

The conduction defect is due to maternal IgG anti-Ro (SS-A) more than anti-La (SS-B) antibodies that cross the placenta and bind to fetal cardiocytes and heart conducting cells, eliciting an inflammatory injury during the second trimester of pregnancy. Clinical manifestations of NLE occur in 5% to 20% of offspring of mothers with high circulating levels of these antibodies. The risk of CHB is 2% in the offspring of seropositive mothers.

After a mother has one child with CHB, the risk of recurrent heart bock in the fetus during another pregnancy is 15% to 20%. In some retrospective observational studies, hydroxychloroquine use was associated with a decreased rate of recurrent CHB in subsequent pregnancies. 


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