title: Outcomes of children with proliferative lupus nephritis: the role of protocol renal biopsy.
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 cyclophosphamide (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). administration 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 classification 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.
authors.affiliation: Department of Pediatrics, University of Alabama at Birmingham, 1600 7th Avenue South, ACC 516, Birmingham, AL, 35233, USA. daskenazi@peds.uab.edu
authors.forename: David
authors.initials: D
authors.lastname: Askenazi
authors.forename: Barry
authors.initials: B
authors.lastname: Myones
authors.forename: Ankur
authors.initials: A
authors.lastname: Kamdar
authors.forename: Robert
authors.initials: R
authors.lastname: Warren
authors.forename: Maria
authors.initials: M
authors.lastname: Perez
authors.forename: Marietta
authors.initials: M
authors.lastname: De Guzman
authors.forename: Anna
authors.initials: A
authors.lastname: Minta
authors.forename: M John
authors.initials: MJ
authors.lastname: Hicks
authors.forename: Arundhati
authors.initials: A
authors.lastname: Kale
mesh_terms: D001706:Biopsy; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007275:Injections, Intravenous; D007668:Kidney / Q000601:surgery*; D007958:Leukocyte Count; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D008181:Lupus Nephritis / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D008297:Male; D009504:Neutrophils / Q000187:drug effects; D020551:Pulse Therapy, Drug; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide