Lupus eritematoso neonatal

Abstract

Neonatal lupus erythematosus (NLE) is an uncommon disease caused by transport of maternal autoantibodies against Ro, La and/or ribonucleoprotein (RNP) into the fetal circulation. These IgG antibodies cross the placenta and can potentially damage fetal tissue and cause the clinical manifestations of NLE.

NLE is more common in girls, has no racial association and can affect multiple organs. Cutaneous manifestations are present in 50% of patients and are clinically similar to the lesions of subacute lupus erythematosus. Patients with NLE have a higher risk of congenital heart block, a potentially fatal complication. Other, less frequent manifestations are hepatic and hematologic. Approximately half of all patients have cutaneous manifestations and the other half cardiac manifestations. Approximately 10% of patients have both cutaneous and cardiac manifestations. Cutaneous, hematological and hepatic manifestations are transient, healing at 6 months of age, due to the clearance of maternal autoantibodies. Congenital heart block is a permanent manifestation that usually requires pacemaker implantation.

Pregnant women with autoimmune diseases or anti-Ro or anti-La antibodies should be followed-up by ultrasound during pregnancy to detect any manifestations susceptible to treatment. Currently, there is no consensus on preventive treatment in high-risk patients or in those with a risk of recurrence. Equally, there is insufficient data to confirm the efficacy of currently available preventive treatments. What seems clear is that early detection of congenital heart block should be treated with fluorinated corticosteroids.