Infantile systemic lupus erythematosus with onset simulating congenital nephrotic syndrome

Two white female infants were seen with congenital nephrotic syndrome at age 6 weeks and 3 months, respectively. Both had hypocomplementemia, elevated antinuclear antibody and anti-double-stranded DNA titers, and diffuse proliferative glomerulonephritis with positive immunofluorescence in their initial renal biopsy samples. Although uncommon, infantile systemic lupus erythematosus should be considered in the evaluation of congenital nephrotic syndrome.