Neonatal lupus erythematosus

Neonatal lupus erythematosus (NLE) is associated with cutaneous lesions, congenital heart block (CHB), hepatic disease and thrombocytopenia. IgG antibodies to Ro and/or La cross the placenta and participate in the development of the clinical manifestations. Mothers of babies with NLE are likely to develop collagen vascular diseases with time. Infants with NLE are at risk to develop other autoimmune diseases during childhood or adolescence.

Neonatal lupus erythematosus (NLE) was likely first reported in 1928 in two siblings with congenital heart block (CHB) born to a mother with Sjogren's syndrome. In 1954, the link was suggested between the mother's autoimmune disease and the cutaneous lupus lesions in her newborn. This was latter found to be associated with maternal anti-Ro antibodies, then with anti-La antibodies; also with congenital heart block.

Neonatal lupus skin lesions often appear in the first few weeks of life, but may be present at birth. They are scaly lesions on the face, scalp and extremities, but nonscarring.

One to two-thirds of mothers of babies with NLE will develop rheumatic disease within 5 years. A Canadian study followed mothers of babies with cutaneous NLE and those with CHB for a mean of 7 years. A much greater percentage (75%) of mothers of babies with cutaneous disease developed symptomatic rheumatic disease; 56% of mothers of infants with CHB remained asymptomatic.

There have been case reports of infants with NLE going on to develop SLE as adolescents. Five of 57 patients reviewed in the NLE registry developed autoimmune syndromes within 6 years: autoimmune thyroiditis, juvenile rheumatoid arthritis, Raynaud's disease, and systemic lupus erythematosus.

About 1 in 200 women is positive for anti-Ro antibodies. The risk for mothers who are anti-Ro antibody positive to have an affected infant is about 0.5%. If the mother has a previously affected infant, the risk increases to about 20%, regardless of whether CHB or cutaneous NLE was present.

NLE appears to occur in about 1 in 20,000 live births and can affect all ethnic groups. Females are affected by NLE preferentially 2:1 for cutaneous lupus, but males and females are equally affected with congenital heart block (CHB); 70% to 80% of babies with CHB had NLE.

One percent of NLE patients develop hepatic disease. Thrombocytopenia or neutropenia is observed in about 10% of cases. Both usually resolve within weeks of birth.