University of Colorado School of Medicine, Denver Health Medical Center, Denver, Colorado, USA

Correspondence: Lela A. Lee, Denver Health Medical Center, Mail Code 4000, 777 Bannock St., Denver, CO 80204, USAEmail: lela.lee@uchsc.edu

Received 16 September 2002; Revised 10 November 2002; Accepted 13 January 2003.

Abstract

Neonatal lupus erythematosus is an uncommon disease associated with maternal autoantibodies to proteins of the Ro/La (SSA/SSB) family. The clinical findings most often reported are third- degree heart block and cutaneous lupus lesions, but a significant number of children have cardiomyopathy, hepatobiliary disease, or hematologic cytopenias. The consistent presence of maternal autoantibodies and the transient nature of the disease implicate maternal autoantibodies as the cause of the disease, and developing animal models support the concept that the autoantibodies are pathogenic. Only a minority of babies exposed to the autoantibodies develop disease, however, and mothers and their babies have different disease manifestations. Thus, additional factors are likely to be important in determining disease expression.