P440 Cadiac neonatal lupus

Background Neonatal lupus erythematosus (NLE) is an uncommon acquired autoimmune disease caused by transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. The most common effects of NLE include cutaneous involvement and congenital heart block (CHB). The death rate of complete congenital heart block (CCHB), which is the most severe clinical manifestation, is as high as 20% to 30%. That’s why, early recognition of infants at risk is important. Objectives To investigate the clinical features and long-term prognosis of cardiac NLE. Methods A retrospective descriptive study of nine cases with cardiac events of neonatal lupus over a 15-year period from January 2003 to December 2018. The diagnostic criteria required the presence of clinical symptoms and positive autoantibodies both in the mother and her child. Results There were 9 patients (male: female ratio of 6:3). Sjogren syndrome was the most common maternal involvement. CCHB was detected in 7 patients of whom 2 patients had an antenatal diagnosis. Two other patients were presented with a cardiomyopathy with immediate heart failure in one case. Antenatal treatment with glucocorticoids and/or hydroxychloroquine was used in 3 cases (maternal connective disease in 2 cases and CCHB in 1 case). After birth, 2 patients were treated with isoprenaline and 6 of the 8 patients diagnosed with the CHB received single-chamber pacemaker implants. Immediate evolution was favorable for all patients. During the follow-up, two infants died. A cardiomyopathy appeared in one patient with CCHB one year after pace maker implanting. Conclusions CCHB is the most serious manifestation of the NLE associated with significant morbidity and mortality. Early diagnosis can lead to early treatment avoiding in some cases the onset of CCHB.