Delayed dilated cardiomyopathy as a manifestation of neonatal lupus: case reports, autoantibody analysis, and management.

Abstract

Neonatal lupus erythematosus (NLE) is a transient disease consisting of one or more of the following findings: congenital complete heart block with or without congestive heart failure, photosensitive erythematous macular cutaneous lesions, thrombocytopenia, and cholestatic liver disease.1 In almost all cases, mothers of infants who have NLE have anti-Ro/Sjogren's Syndrome A antigen (Ro/SSA) autoantibodies of the immunoglobulin G (IgG) class. NLE is believed to be caused by the placental transmission of these maternal autoantibodies. The heart block typically begins during the second or third trimester. Although the disease process of NLE is transient, the atrioventricular node may be replaced by scar tissue, resulting in permanent heart block. In those infants who have heart block accompanied by heart failure, pacemaker placement is necessary and is usually sufficient to resolve symptoms. However, at least 10% of infants who have heart block due to NLE have heart failure that does not respond adequately to pacing, and it has been presumed that the cardiac muscle may also be affected by the disease process.1,2 In these most severely affected infants, heart failure is typically apparent at birth or very shortly after birth. We describe two infants who were unique because of the late onset of cardiomyopathy. The infants had congenital complete heart block and pacemaker placement in the perinatal period and did well for the first few months of life. Symptoms of heart failure developed at 8 and 4 months of age, respectively, and they were noted to have dilated cardiomyopathy. We confirm the persistence of substantial quantities of maternal anti-Ro/SSA antibodies in the infants' sera at the time of development of the dilated cardiomyopathy and speculate that the prolonged presence of high levels of maternal autoantibodies in the sera facilitated the unusually prolonged disease course in these children. ### Case 1 The patient …