An Unusual Case of Severe Neonatal Lupus Mimicking Neonatal Sepsis and Literature Review

ABSTRACT

Neonatal lupus is an uncommon autoimmune disease that results from transplacental passage of the maternal anti-SSA/Ro and/or anti-SSB/La antibodies. Pancytopenia or severe jaundice is a rare manifestation of neonatal lupus, respectively, and could be misdiagnosed with other neonatal illnesses, such as infection, hematologic disease, or hepatobiliary disease. Here, we report an unusual case of a premature newborn with severe neonatal lupus manifested with skin rash, fever, pancytopenia, and severe jaundice with abnormal liver function tests. His mother had been clinically asymptomatic before delivery; however, she revealed peripheral edema, bilateral pleural effusion, and ascites after delivery and diagnosed with systemic lupus erythematosus based on positive anti-nuclear and anti-cardiolipin antibodies, proteinuria, and serositis. The newborn and his mother had anti-SSA/Ro and anti-SSB/La antibodies. His pancytopenia and jaundice were progressively aggravated, and his illness was confused with neonatal sepsis or biliary obstruction. We decided to treat with high dose of corticosteroid and intravenous immunoglobulin, and he gradually recovered completely with the treatment. His corticosteroid was stopped at 5 months of age without relapse or complication.