Original Articles
Neonatal lupus erythematosus: results of maternal corticosteroid therapy

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Abstract

Objective: To assess the possibility of preventing cardiac or cutaneous manifestations of neonatal lupus erythematosus or treating the fetus with congenital heart block by administering corticosteroid therapy to the mother.

Methods: Eighty-seven offspring of 40 anti-Ro/SSA-positive mothers, followed up from 1979 to 1996, were evaluated. Autoantibodies against Ro/SSA and La/SSB antigens were detected by immunodiffusion and enzyme-linked immunosorbent assay.

Results: None of 26 neonates whose mothers received corticosteroid maintenance therapy initiated before 16 weeks’ gestation demonstrated congenital heart block, whereas 15 of 61 neonates whose mothers received no corticosteroids during pregnancy or began receiving steroid therapy after 16 weeks’ gestation had congenital heart block. Complete congenital heart block, once developed, did not respond to corticosteroid treatment in utero. Four infants whose mothers received steroid treatment before 16 weeks’ gestation had skin lesions of neonatal lupus erythematosus.

Conclusion: Once established, complete congenital heart block was irreversible and maternal corticosteroid therapy did not effectively prevent cutaneous lupus erythematosus. However, prenatal maintenance therapy with prednisolone or betamethasone given to the mother starting early in pregnancy (before 16 weeks’ gestation) might reduce the risk of developing antibody-mediated congenital heart block in the offspring.

Section snippets

Materials and methods

Since 1979, we screened sera from patients with symptoms of connective tissue diseases for precipitating autoantibodies against extractable nuclear antigens. Forty mothers who were positive for anti-Ro/SSA antibodies were included in this study. Twenty-eight women had been followed at our hospitals, and seven were referred for care after fetal bradycardia was diagnosed. In the other five cases (two infants with congenital heart block and three infants with cutaneous lupus lesions), serum

Results

Results of clinical and serologic studies as well as neonatal outcomes in anti-Ro/SSA-positive mothers are given in Table 1. Fourteen mothers were positive for both anti-Ro/SSA and La/SSB antibodies, and 26 mothers had only anti-Ro/SSA antibodies. As reported previously,13, 15 there was no specific antibody profile unique to mothers of children with neonatal lupus erythematosus compared with anti-Ro/SSA-positive mothers with asymptomatic children. However, mothers with only a low titer of

Discussion

Neonatal lupus erythematosus was originally described in children born to mothers with systemic lupus erythematosus and Sjögren’s syndrome, but recent studies have shown that in many mothers, the symptoms of connective tissue disease are absent or mild at the time of delivery of the affected infants.4, 15, 16 Permanent cardiac and transient cutaneous disease are two major manifestations of neonatal lupus erythematosus.17, 18 According to previous findings5, 17 as well as those of the present

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