|Agnieszka Zmysłowska, Elżbieta Smolewska, Anna Charubczyk, Henryka Brózik, Elżbieta Zielińska|
Neonatal lupus erythematosus, characterized by a wide spectrum of clinical symptoms, is an rare antibody-mediated disorder due to placentally transmitted maternal autoantibodies to Ro/SSA and/or La/SSB. The sera of the neonatal lupus mothers and their infants are positive for a few autoantibodies, which are relevant for prognosis and the clinical course of the disease. Manifestations of neonatal lupus erythematosus are variable. Affected tissues commonly include skin, heart and blood cells, in a single or combined setting. The most severe symptoms of the disease are the cardiac abnormalities, as the complete atrioventricular block, which often leads to death within first days of life.
We report the clinical and diagnostic course of a case of multiorgan manifestation of neonatal lupus erythematosus in a 2-months-old boy with a few characteristic symptoms and atypical cardiac signs. Physical examination revealed skin lesions, hepatosplenomegaly and profound anaemia. There were multiple episodes of tachycardia, hypertension, persistent foramen oval as confirmed in echocardiography and urinary tract disorders of vesicoureteral reflux type. The high titter of antinuclear antibody and anti-Ro/SSA (1:1280) were found. Simultaneously in maternal blood antinuclear antibody and anti-Sm antibody were detected. Mother of the child had no clinical signs. Immunosupressive and anti-inflammatory treatment led to complete resolution of the skin lesions.
Alergia Astma Immunologia, 2001, 6(3), 164-168
keywords: toczeń noworodkowy, przeciwciała anty-Ro/SSA, przeciwciała anty-La/SSB, przeciwciała przeciwjądrowe, postać skórna, postać sercowa, neonatal lupus, anti-Ro/SSA antibodies, anti-La/SSB antibodies, antinuclear antibodies, skin lesions, cardiac abnormalities
pages: from 164 to 168
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