P094 “Neonatal lupus”: a unique name for very different realities

Abstract

Abstract Background Neonatal lupus is a rare condition linked to the maternal-fetal transmission of maternal anti-SSA and/or anti-SSB antibodies, more rarely anti-U1-RNP. The most frequent clinical manifestations are cardiac and cutaneous, more rarely hematological (thrombocytopenia, leuco-neutropenia), hepatic (cholestasis), neurological (spastic paresis, lymphocytic meningitis) or renal. We report two observations of neonatal lupus: a classic form, and a rare form. Observations First case: A female newborn, premature of 37 weeks, from a first-degree consanguineous marriage, having a mother followed for systemic lupus erythematosus, with poor therapeutic compliance, presented at H1 of life with neonatal respiratory distress. The clinical examination revealed severe bradycardia at 67 Bpm. The electrocardiogram showed complete atrioventricular block, with a moderate pericarditis on the echocardiography, minimal tricuspid insufficiency, interatrial communication, and a 6 mm foramen oval with LR shunt. The immunological test had objectified positive antinuclear, anti-SSA and anti-SSB antibodies. An implementation of a pacemaker with inter-atrial communication ligation were performed successfully. The evolution was marked by the appearance of a malar erythema with generalized lesions of discoid lupus at the age of 14 months, treated with local corticosteroid therapy, with good outcomes. The control immunological workup was negative at 18 months. Second case: A 2-month-old boy from a non-consanguineous marriage with a mother followed for Sjögren's syndrome was admitted for a symptomatology dating back to birth marked by the appearance of diffuse petechial purpura. Clinically, the infant presented diffuse discoid macules throughout the body. The somatic examination, particularly cardiovascular, was normal. The biological workup showed haemolytic anaemia with a positive coombs test and severe thrombocytopenia. The electrocardiogram and echocardiography were normal. The immunological workup had objectified positive anti-SSA, anti-SSB and anti-nuclear antibodies. The infant was treated by oral corticosteroids (prednisolone) for 4 months, with good outcomes. The control immunological workup was negative at 6 months. Conclusion The clinical manifestations of neonatal lupus are polymorphic, and the only one that may expose to life threat is complete atrioventricular block. All mothers with known lupus or with positive anti-SSA autoantibodies should be monitored during pregnancy with repeated ultrasounds.