A 10-year-old girl with distal renal tubular acidosis (RTA) for 4 year
s (adequately treated for 3 years) developed clinical features suggest
ing systemic lupus erythematosus (SLE) with supportive laboratory evid
ence. She had heavy proteinuria and a decreased creatinine clearance (
C-Cr). Renal biopsy showed diffuse proliferative and sclerosing glomer
ulonephritis with severe tubulointerstitial changes. Following treatme
nt with corticosteroids and cyclophosphamide, she had a clinical remis
sion, an increase in C-Cr and recovery from systemic acidosis. It is L
ikely that distal RTA in this patient was a manifestation of SLE.