Systemic lupus erythematosus presenting as congenital nephrotic syndrome.
A male Caucasian infant developed nephrotic syndrome at 10 weeks of age. He had high titres of antinuclear antibody (ANA) and anti-double-stranded DNA antibody, with hypocomplementaemia, antiplatelet antibodies and anticardiolipin antibodies. There were no detectable antibodies to extractable nuclear antigens (ENA). His mother was consistently seronegative for anti-ENA (anti-Ro) antibodies and ANA. He developed severe, progressive multisystem involvement, however renal function has remained stable. Immunosuppression has been the mainstay of therapy in this rare presentation of systemic lupus erythematosus.
Dudley
J
J
Department of Paediatric Nephrology, Southmead Hospital, Westbury-on-Trym, Bristol, UK.
Fenton
T
T
Unsworth
J
J
Chambers
T
T
MacIver
A
A
Tizard
J
J
D000974:Antibodies, Antinuclear / Q000032:analysis; D004247:DNA / Q000276:immunology; D005920:Glomerular Mesangium / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology
D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; D004247:DNA