Neonatal lupus syndromes

Abstract

The congenital heart block (CHB) was the first reported neonatal lupus syndrome. CHB can occur in association with structural heart disease, such as atrioventricular septal defects, left atrial isomerism, and abnormalities of the great arteries with tumors, such as mesotheliomas, or as an isolated defect. It is found that maternal sera contained antibodies to SSA/Ro ribonucleoproteins. Other abnormalities affecting the skin, liver, and blood elements were reported to be associated with anti-SSA/Ro-SSB/La antibodies in the maternal and fetal circulation and are now grouped under the heading of neonatal lupus syndromes (NLS). Neonatal lupus was so termed because the cutaneous lesions of the neonate resembled those seen in system lupus erythematosus. The transient hematologic abnormalities and skin disease of the neonate reflect the effect of passively acquired autoantibodies on those organ systems that have the capacity of continual regeneration. In contrast, these regenerative processes apparently do not occur in cardiac tissue; complete block is irreversible to date. Curiously, the transient manifestations of this passively acquired autoimmune syndrome closely mimic the disease manifestations observed in adolescents or adults with systemic lupus erythematosus, whereas while heart block is rarely, if ever, present in these same patients.