The Efficacy of Intravenous Pulse Cyclophosphamide in the Treatment of Severe Lupus Nephritis in Children
KANCHANA TANGNARARATCHAKIT, M.D.*, CHALEOMSRI TAPANEYA-OLARN, M.D.*, WIWAT TAPANEYA-OLARN,M.D.*
Affiliation : * Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok I 0400, Thailand.
Objective : To evaluate the clinical efficacy, renal function, renal outcome and com- plications of IPC therapy in children with severe lupus nephritis.
Method : Prospective study.
Patients : Children with severe lupus nephritis have been followed-up for at least 6 months. Treatment regimen: Intravenous pulse cyclophosphamide 0.5-0.75 gfm2 given monthly for 6 months with subsequent doses given at 2-3 months interval up to 3 years and combined with low dose oral prednisolone therapy.
Results :Thirty-one children (mean age: 12.31 ± 2.03 years; female : male= 24:7) with severe lupus nephritis received IPC therapy. 24 out of 28 patients (85. 7%) had diffuse pro- liferative glomerulonephritis. After 3 months of treatment, most patients were clinically improved as evidenced by significant improvements in 24-hour urine protein, creatinine clearance, serum creatinine, BUN, serum albumin and c3 level. These improvements were sustained up to 18 months and were accompanied by a significant reduction in prednisolone dosage. Renal outcome at the last follow-up (range = 6-76 months) demonstrated that twelve patients (38. 7%) had complete remission, 18 patients (58.0%) still had significant proteinuria and only one had serum creatinine of 1.6 mg/dl at 42 months. One child progressed to end stage renal diseases during IPC therapy. Five patients had severe infections during the treatment resulting in one death. Hemorrhagic cystitis and malignancies were not found.
Conclusion : Treatment of severe lupus nephritis in children with intravenous pulse cyclophosphamide is associated with favorable short term results. Severe infections are the major complications.
Keywords : Lupus Nephritis, Children, Pulse Cyclophosphamide, Treatment
