KMID : 0388220140210030156
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Journal of the Korean Rheumatism Association 2014 Volume.21 No. 3 p.156 ~ p.161
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An Unusual Case of Severe Neonatal Lupus Mimicking Neonatal Sepsis and Literature Review
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Park Hye-Min
Jun Hong-Gil Park Ju-Hwan Choi Seung-Won Oh Ki-Won Oh Ji-Seon
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Abstract
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Neonatal lupus is an uncommon autoimmune disease that results from transplacental passage of the maternal an-ti-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 neo-natal illnesses, such as infection, hematologic disease, or hepatobiliary disease. Here, we report an unusual case of a premature newborn with severe neonatal lupus man-ifested with skin rash, fever, pancytopenia, and severe jaundice with abnormal liver function tests. His mother had been clinically asymptomatic before delivery; how-ever, she revealed peripheral edema, bilateral pleural effu-sion, and ascites after delivery and diagnosed with sys-temic lupus erythematosus based on positive anti-nuclear and anti-cardiolipin antibodies, proteinuria, and serositis. The newborn and his mother had anti-SSA/Ro and an-ti-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 im-munoglobulin, and he gradually recovered completely with the treatment. His corticosteroid was stopped at 5 months of age without relapse or complication.
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KEYWORD
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Neonatal lupus, Severe jaundice, Pancytopenia, Steroid
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