Cardiac manifestations of neonatal lupus erythematosus: guidelines to management, integrating clues from the bench and bedside.
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.
Buyon
Jill P
JP
Division of Rheumatology, New York Medical College, New York, NY, USA. jbuyonic@aol.com
Clancy
Robert M
RM
Friedman
Deborah M
DM
D001323:Autoantibodies / Q000097:blood; D004562:Electrocardiography; D005260:Female; D005315:Fetal Diseases / Q000188:drug therapy / Q000209:etiology; D006327:Heart Block / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007074:Immunoglobulin G / Q000008:administration & dosage; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D007262:Infusions, Intravenous; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D011247:Pregnancy; D012261:Ribonucleoproteins / Q000276:immunology; D012307:Risk Factors; D000099207:SS-A Antigen
D001323:Autoantibodies; D007074:Immunoglobulin G; D012261:Ribonucleoproteins; D000099207:SS-A Antigen