Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement
The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.
Recurrence of Neonatal Lupus Erythematosus in Siblings
Danayan1, KC, Jaeggi1, E., Tyrrell2, PN, Rogers1, A., Silverman3, ED.
Neonatal lupus erythematosus (NLE) is a model of passively acquired autoimmunity due to in utero exposure to maternal anti-Ro and/or anti-La antibodies. Manifestations of NLE include: congenital heart block (CHB), rash, hepatic or haematologic abnormalities, and macrocephaly. Much of the research to date has focused on the cardiac manifestations of NLE with little information on the risk of recurrence of NLE in subsequent pregnancies. The literature suggests a 15% recurrence risk of CHB developing in the subsequent sibling following the delivery of a child with CHB related to NLE. However, this recurrence rate is based on very few studies and none examined the risk of recurrence of any NLE manifestation following an index case with NLE. Our study was designed to address this clinical question.
A retrospective cohort study of 36 patients with confirmed NLE (index cases) and all 42 of their subsequent siblings followed at a tertiary paediatric hospital from March 1984 to April 2009. Detailed patient and maternal medical information was extracted from a rheumatology database and validated by chart review. The study cohort consisted of 42 consecutive children born to 36 anti-Ro and/or anti-La positive women. The women were identified following the delivery of a first-born child with confirmed NLE and all subsequent pregnancies were followed prospectively for the presence of fetal or neonatal manifestations of NLE. Patients were excluded if the NLE diagnosis was made retrospectively, essential patient information was missing, or the index NLE case was not seen initially at our institution.
Recurrence of NLE in a subsequent sibling after an index case of confirmed NLE occurred in 72.2% of families (26 out of 36 families) and 64.3% of subsequent siblings (27 out of 42 siblings). The outcome of NLE manifestations in index cases compared to any subsequent sibling is illustrated below. The rate of occurrence of CHB was 8.3% in families and 7.1% of all subsequent siblings after an index case of NLE, but 15.4% if the index case had CHB and 0% if the index case did not have CHB or rash as their NLE manifestation.
NLE recurred in at least one subsequent sibling in over two-thirds of families and the majority of subsequent siblings following an index case of NLE - however, it mainly presented as benign and transient non-cardiac features. Interestingly, when an index case had CHB, the risk of CHB in any subsequent sibling was higher (15.4%) compared to families where an index case presented with non-cardiac manifestations of NLE, similar to previously published recurrence rates. Further studies are needed to identify predictors of NLE recurrence of congenital heart block in order to help guide prospective monitoring of future pregnancies and siblings.
To cite this abstract, please use the following information:
Danayan, KC, Jaeggi, E., Tyrrell, PN, Rogers, A., Silverman, ED.; Recurrence of Neonatal Lupus Erythematosus in Siblings [abstract]. Arthritis Rheum 2009;60 Suppl 10 :1252