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Monday, October 19, 2009: 4:30 PM
204 B (Pennsylvania Convention Center)
Presentation Number: 1250
Purpose: The cutaneous manifestations associated with placental transport of maternal anti-Ro/La antibodies are generally transient and inconsequential. However, the impact of this manifestation of neonatal lupus on the risk of cardiac disease in a future pregnancy is critical for family counseling and powering of preventive trials. Methods: Of the 380 families enrolled in the Research Registry for Neonatal Lupus (RRNL) 57 met the following inclusion criteria: a) maternal anti-Ro or La antibodies, b) a child with classic annular or elliptical lesions on the face, scalp, trunk, or extremities which was verified by medical records and/or photographs, c) information on pregnancies subsequent to the child with rash. Maternal risk factors evaluated for recurrence of rash or emergence of cardiac manifestations included: age, race/ethnicity, antibody status, diagnosis, and use of non-fluorinated steroids during pregnancy. The following risk factors were also evaluated: gender of the child, season of birth, latitude of the city at birth, and breast feeding. Results: The majority (77%) of the 57 mothers were Caucasian. Of the 76 pregnancies following a child with rash, the overall recurrence rate for any manifestation of neonatal lupus was 50%. Specifically 14 (18%) were complicated by congenital heart block (CHB) (all 2nd/3rd degree, 9 accompanied by a rash and 2 with associated liver/hematological abnormalities), a nearly tenfold risk over the 2% rate reported without a previously affected child. Twenty-three (30%) of the subsequent children had a rash (3 accompanied by hematological and/or liver abnormalities). One child (1%) had isolated liver/hematological abnormalities and one neonate (1%) died of unknown reasons. Thirty-seven (49%) were healthy. In an attempt to limit potential referral bias of families with multiple affected children, a subset analysis was restricted to the 45 children who were born prospectively after the initial child was enrolled in the RRNL. In this analysis, the overall recurrence rate for neonatal lupus was 36%. Specifically, 5 (11%) developed CHB (a 6-fold higher risk for CHB), 10 (22%) developed a rash and one child (2%) developed isolated liver/hematological abnormalities. There were no significant differences in the following maternal risk factors for a having a subsequent child with either CHB or rash: age, race/ethnicity, anti-La status, diagnosis or use of non-fluorinated steroids during pregnancy. Fetal gender of the subsequent child did not influence the development of CHB or rash. Specifically with regard to the development of rash in a subsequent child, breast feeding, season of birth, or latitude of the city at birth did not affect risk. Conclusions: Based on data from this large cohort, the identification of cutaneous disease in an anti-Ro exposed infant is particularly important since it predicts increased risk for a subsequent child with CHB.
Keywords: heart disease, neonatal disorders and pregnancy
Disclosure: P. M. Izmirly, SLE Foundation, Inc., 2 ; C. Llanos, SLE Foundation, Inc., 2 ; J. P. Buyon, NIH Contract NO1-AR-4-2220 (Research Registry for Neonatal Lupus), 2 .