A clinicopathological study of lupus nephritis in children

To assess clinical characteristics, pathological findings, and therapeutic response in children with lupus nephritis (LN), we retrospectively studied 25 children under 16 years of age with LN at the Abozar children's hospital from 1995 to 2006. The study included 13(65%) girls and 7(35%) boys. The mean age at the time of diagnosis of SLE was 10.2 (+/- 4.8) years. Eighteen patients (90%) were more than 8 years old. Sixty percent of the patients presented as nephritic-nephrotic syndrome. All the patients underwent percutaneous renal biopsy and were followed up for at least 36 months. The clinical and serologic parameters at the time of renal biopsy were recorded. Twenty patients were treated with the following regimens: one (class I) with low dose prednisone, 7 (class II, III) with high-dose of prednisone, 12 (class IV) with high-dose prednisone plus 13 intermittent intravenous cyclophosphamide (CTX) pulses (monthly for 6 months and then every 3 months), followed by mycophenolate mofetil (MMF) as maintenance therapy. Remission was achieved in 17 (85%) cases; one required hemodialysis and 2 died due to renal failure and central nervous system involvement. Among 12 cases with class IV, 11 responded to prednisone and intravenous CTX pulses. We conclude that i.v. pulses of CTX induced clinical remission of renal disease in the majority of children with severe LN. MMF maintenance therapy was effective after induction of remission in refractory cases. However, this study was performed in a small number of subjects, further studies to confirm the long-term efficacy and safety of CTX pulse therapy on larger numbers of patients are warranted.