Isolated antinuclear antibody-negative lupus nephropathy in young children

Systemic lupus erythematosus (SLE) is a multisystem disease in which the kidneys are frequently involved. The clinical and histopathologic diagnosis of SLE is based on the American Rheumatism Association (ARA) and the World Health Organization classification, respectively. A few adults who qualify as SLE by the ARA criteria but are antinuclear antibody (ANA)-negative have been reported. We found only one report of a child with SLE where renal disease was the only manifestation but who had a positive ANA. This report describes 3 children who presented with the nephrotic syndrome and in whom the renal histology is highly suggestive of SLE. Short-term follow-up as failed to demonstrate other systemic involvement and the ANA has continued to be negative.