SYSTEMIC LUPUS-ERYTHEMATOSUS PRESENTING AS CONGENITAL NEPHROTIC SYNDROME

A male Caucasian infant developed nephrotic syndrome at 10 weeks of ag e. He had high titres of antinuclear antibody (ANA) and anti-double-st randed DNA antibody, with hypocomplementaemia, antiplatelet antibodies and anticardiolipin antibodies. There were no detectable antibodies t o extractable nuclear antigens (ENA). His mother was consistently sero negative for anti-ENA (anti-Re) antibodies and ANA. He developed sever e, progressive multisystem involvement, however renal function has rem ained stable. Immunosuppression has been the mainstay of therapy in th is rare presentation of systemic lupus erythematosus.