ORIGINAL ARTICLE Outcomes of children with proliferative lupus nephritis: the role of protocol renal biopsy David Askenazi & Barry Myones & Ankur Kamdar & Robert Warren & Maria Perez & Marietta De Guzman & Anna Minta & M. John Hicks & Arundhati Kale Received: 9 October 2006 / Revised: 5 January 2007 / Accepted: 16 January 2007 / Published online: 3 March 2007 # IPNA 2007 Abstract Outcomes in children with proliferate lupus nephritis (PLN) show 9–15% progress to end-stage renal disease (ESRD) at 5 years. Immunosuppression improves outcome, but significant side effects are possible. Clinical and laboratory analyses are poor predictors of class and progression in PLN. We describe 28 patients with systemic lupus erythematosus (SLE), between 1990 and 2005, whose initial biopsy (Bx1) showed PLN and who received nine monthly doses of intravenously administered cyclophos- phamide (CYP) (500–750 mg/m 2 up to 1 g to maintain their absolute neutrophil count (ANC) > 3,000). Continued therapy with additional quarterly intravenous (i.v). ad- ministration of CYP was dictated by repeat renal biopsy (Bx2). Bx1 was done 1±1.6 years after diagnosis of SLE. Bx2 showed histological improvement by WHO classifica- tion in 20/25 children; 3/25 were unchanged, 1/25 was categorized as new class V, and 1/25 was worse. Four patients (14%) had infectious complications requiring hospitalization (one of these died). Mean follow-up (f/u) after Bx2 was 3.5±2.3 years. At last follow-up, 26 patients had normal glomerular filtration rate (GFR), with a mean of 126±42.8 ml/min per 1.73 m 2 body surface area, one non- compliant patient had ESRD, and one had chronic renal failure. At last follow-up, most patients had minimal to no proteinuria. Clinical and biopsy results greatly improved after 9 monthly intravenously administered CYP pulses in most children with class IV PLN. Those who did not improve are at risk for flares and progression of disease. The tailoring of therapies based on findings from a biopsy after induction may improve outcomes. Keywords Lupus nephritis . Proliferative . Protocol biopsy . Cyclophosphamide . Outcome Background Families and physicians taking care of children with proliferative lupus nephritis (PLN) are faced with difficult decisions in balancing the impact of a chronic progressive disease and serious medications side effects. The best treatment for PLN remains very controversial. Based on the randomized control trials on adults published by the National Institutes of Health (NIH), most clinicians advocate the use of intravenous (i.v.) cyclophosphamide (CYP) for induction of PLN, as it has been shown to improve long- term renal outcomes [1, 2]. Long-term immunosuppression has been shown to improve renal survival and reduce the risk of renal flares [3, 4]; however, much controversy exists in regard to the drug, dose and length of treatment after the initial induction therapy for lupus nephritis. In children, very few data exist in regards to long-term management and outcomes of lupus nephritis. Many different treatment strategies have been proposed, including oral administration of CYP, i.v. infusion of CYP, cyclosporine, methylprednisolone, azathioprine, methotrexate and myco- phenolate mofetil (MMF). Current data are based on small Pediatr Nephrol (2007) 22:981–986 DOI 10.1007/s00467-007-0447-9 D. Askenazi (*) Department of Pediatrics, University of Alabama at Birmingham, 1600 7th Avenue South, ACC 516, Birmingham, AL 35233, USA e-mail: daskenazi@peds.uab.edu B. Myones : A. Kamdar : R. Warren : M. Perez : M. De Guzman : A. Minta : A. Kale Pediatric Rheumatology, Texas Children’ s Hospital, Houston, TX, USA M. J. Hicks Department of Pathology, Baylor College of Medicine, Houston, TX, USA