[Lupus nephritis in children and adolescents: therapy, clinical course and prognosis]

In 53 children and adolescents (47 males, 6 females) with lupus nephritis, clinical features at the time of renal biopsy were analyzed and correlated with pathohistological findings. Therapeutic regimens used and the renal status at the end of follow-up are presented, and factors significantly associated with adverse outcome are analyzed. The mean age at the time of diagnosis of systemic lupus was 12.9 +/- 2.6 (SD) years, the mean ages at the time of diagnosis of lupus nephritis and renal biopsy were 13.5 +/- 2.6 and 13.6 +/- 2.5 years, respectively. The patients having WHO classes I and II of lupus nephritis were treated with prednisone and/or azathioprine; those with WHO classes IV and III were treated with cyclophosphamide (25) or with corticosteroids (5) or with corticosteroids and azathioprine (4), five of them were also treated with plasmapheresis; the patients with WHO class V were treated with prednisone and azathioprine or cyclosporine. Repeated renal biopsies were performed in 12 patients: worsening of morphological lesions was found in four patients, improvement in two and no change was observed in six patients (all with WHO class IV). At the end of follow-up lasting from 0.1 to 14.6 years (mean 4.8 +/- 3.2 years) 80% of patients were in complete (49%) or partial (30%) remission, in 8% of patients the renal disease was clinically active, and in 13% of patients the adverse outcome was noted: one patient died in the first month after diagnosis from extrarenal complications, two patients were in preterminal and the other four in terminal renal failure. Adverse outcome was significantly associated with the presence of nephrotic syndrome at the time of biopsy and with class IV nephritis. The five-year patient's survival rate was 98.1%. The five-year kidney survival rate was 88.6% and 82.4% for the whole group and for the subgroup of patients having classes III and IV nephritis, respectively.