1643 LONG-TEEM OUTCOME OF DIFFUSE PROLIFERATIVE LUPUS NEPHRITIS

Abstract

We reviewed our experience with diffuse proliferative Lupus Nephritis (D.P.L.N.) in 22 children. Aggressive steroid and ant metabolite therapy prevented end stage renal disease (E.S.R.D.) in 10 patients, but 2 patients died of sepsis. 12 patients have reached E.S.R.D. in a mean of 30 months from diagnosis.

Dialysis: 12 patients received hemodialysis from 3 to 23 months (mean 13 mos.). No mortality was recorded while on dialysis but a high rate of morbidity was noted. The 12 Lupus patients accounted for 60% of all hospitalizations compared to 51 non Lupus children on dialysis who accounted for 40% of hospitalization (p<.001). One child was able to discontinue dialysis after a period of 9 months.

Transplant: 9 children received 12 transplants (1 patient had 4 transplants). One patient died of sepsis during the 2nd month after transplantation. Graft survival rates at 1 and 4 years were 80 and 65% respectively. Patients with a successful transplant spent a mean of 25 days in hospital post transplant compared to a mean of 112 days in hospital during dialysis (p<.05). One patient had clinical and histological recurrence within 6 months after a live related transplant. Our study concludes:

1) majority of D.P.L.N. patients will reach E.S.R.D. in 3 years;

2) mortality is due to therapy associated sepsis; 3) dialysis is safe but has a high morbidity and 4) successful transplant has the best quality of life but the risk of recurrence in live related donors is a concern.

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