Original Articles
Incidence and spectrum of neonatal lupus erythematosus: A prospective study of infants born to mothers with anti-Ro autoantibodies,☆☆

https://doi.org/10.1067/mpd.2003.233Get rights and content

Abstract

Objective Neonatal lupus erythematosus (NLE) is characterized by complete congenital heart block (CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with autoantibodies directed against SSA/Ro, SSB/La, or both. We carried out a prospective study to determine the incidence of individual NLE features. Study design The study was performed in two centers: Toronto, Canada, and Milano, Italy. Mothers had been referred for the presence of anti-SSA/Ro autoantibodies, regardless of their diagnosis. All the children were seen at least once within the first 6 months of life for clinical evaluation and laboratory testing. The study group consisted of 128 infants born from 124 pregnancies in 112 women with anti-Ro antibodies with or without anti-La antibodies. Results There were two cases of CCHB for an overall percentage of 1.6%. Twenty-one children (16%) developed cutaneous NLE. Laboratory testing showed hematologic abnormalities in 27% of the babies and elevation of liver enzymes in 26%. Conclusions Mothers with autoimmune diseases and anti-Ro antibodies are at risk of delivering a child with NLE but at a low risk of delivering a child with CCHB. Infants born to mothers with anti-Ro or anti-La antibodies should be monitored for other features of NLE in addition to CCHB. (J Pediatr 2003;142:678-83)

Section snippets

Materials and methods

The study group consisted of 128 infants born in two different centers (Toronto, Canada, and Milano, Italy) to mothers who were positive for anti-Ro with or without anti-La antibodies at the time of pregnancy. All mothers were contacted during pregnancy. Both hospitals are tertiary referral centers for NLE, and the study group was composed of patients born during the periods 1987 to 2000 (Toronto) and 1996 to 2000 (Milano). Mothers were referred, and their children subsequently enrolled in this

Results

There were 128 live births and 124 pregnancies. Only 10 of the 128 babies (7.8%) were born at a gestational age of <36 weeks. The Figure shows the incidence of each of the individual manifestations of NLE.

Figure. Occurrence of the individual features of NLE in our series. Numbers above each bar indicate the number of patients evaluated.

Complete congenital heart block was detected in only two of the 128 births (1.6%). Both of these infants with CCHB were born to mothers who had previously

Discussion

Autoimmune diseases frequently occur in women during their childbearing age and may affect the developing fetus and the infant. NLE is a disease of the developing fetus and neonate which is associated with maternal anti-Ro, anti-La, and, less frequently, anti-U1RNP antibodies. The clinical syndrome consists of cardiac abnormalities, most commonly CCHB; cutaneous manifestations; abnormalities of liver function; and hematologic abnormalities. NLE has been reported to occur in approximately 1% to

Acknowledgements

We acknowledge Prof Pierluigi Meroni for referral of several mothers in this study, performance of laboratory testing, and continuous support and discussions.

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  • Cited by (0)

    Supported in part by a grant from the March of Dimes (to Drs Silverman and Hornberger).

    ☆☆

    Reprint requests: Rolando Cimaz, MD, Pediatric Department, ICP, Via Commenda 9, 20122 Milano, Italy. E-mail: [email protected] .

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