Cardiac manifestations of neonatal lupus erythematosus: guidelines to management, integrating clues from the bench and bedside

Nat Clin Pract Rheumatol. 2009 Mar;5(3):139-48. doi: 10.1038/ncprheum1018.


One of the strongest clinical associations with autoantibodies against components of the SSA/Ro-SSB/La ribonucleoprotein complex is the development of congenital heart block in an offspring, an alarming prospect facing 2% of primigravid mothers with these reactivities. This risk is increased tenfold in women who have had a previous child with congenital heart block. Accumulated evidence suggests that anti-SSA/Ro and anti-SSB/La antibodies are necessary but insufficient for fetal disease. Basic and clinical research is heavily focused on identifying fetal and environmental factors that convert disease susceptibility to disease development. A disturbing observation that has emerged from current research efforts is the rapidity of disease progression, with advanced heart block and life-threatening cardiomyopathy being observed less than 2 weeks after detection of a normal sinus rhythm. Once third-degree block is unequivocally identified, reversal has never been achieved, despite dexamethasone treatment. Accordingly, strategies aimed at preventing disease before irrevocable scarring ensues assume a high priority. One approach has been the implementation of serial echocardiography to monitor for a prolonged PR interval. Intravenous immunoglobulin is being evaluated as a potential prophylactic approach in mothers who have previously had an affected child.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Autoantibodies / blood
  • Electrocardiography
  • Female
  • Fetal Diseases / drug therapy
  • Fetal Diseases / etiology
  • Heart Block / congenital*
  • Heart Block / diagnosis
  • Heart Block / therapy
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / etiology*
  • Heart Defects, Congenital / therapy
  • Humans
  • Immunoglobulin G / administration & dosage
  • Infant, Newborn
  • Infant, Newborn, Diseases / diagnosis
  • Infant, Newborn, Diseases / etiology
  • Infant, Newborn, Diseases / therapy
  • Infusions, Intravenous
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / immunology
  • Pregnancy
  • Ribonucleoproteins / immunology
  • Risk Factors


  • Autoantibodies
  • Immunoglobulin G
  • Ribonucleoproteins
  • SS-A antigen