Six Japanese children with severe lupus nephritis received prompt initiatio
n of methylprednisolone pulse therapy (MPT). After three courses of MPT, or
al prednisolone combined with a 12-week course of oral cyclophosphamide was
given and prednisolone was tapered. At presentation, urine protein excreti
on and histological indices of the mean activity index and the mean chronic
ity index in the patients were 2.2+/-1.5 g/day, 10.3+/-2.0, and 2.8+/-1.2,
respectively. Urine protein excretion and the activity index decreased sign
ificantly at the second renal biopsies obtained at a mean interval of 8 mon
ths after the first [0.1+/-0.1 g/day and 3.5+/-1.4 (P<0.05), respectively],
while the chronicity index did not change. At the latest observation (mean
interval 53 months), all showed clinical and serological improvement. Comp
lete remission was achieved in two patients, and no patient showed renal im
pairment. Although this case series is without controls, our treatment prot
ocol may be of benefit to Japanese children with severe lupus nephritis.