Intravenous cyclophosphamide combined with steroids in pediatric onset severe lupus nephritis

Int Urol Nephrol. 2013 Oct;45(5):1301-8. doi: 10.1007/s11255-012-0331-9. Epub 2012 Dec 7.

Abstract

Background: Intravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN) for many years. Because of the wide variety of manifestations of the condition and the long-term nature of the disease, outcomes vary widely.

Objective: To evaluate and compare the immediate and long-term results of IVCY in pediatric onset severe LN and between patients with normal and abnormal initial renal function.

Methods: Patients aged <18 years who attended the Department of Pediatrics, Prince of Songkla University, diagnosed with severe LN, and who were given a 36-month IVCY course, were included. Comparison of overall survival between the two groups was assessed using Kaplan-Meier survival curves.

Results: 108 patients with a mean age of 12.6 ± 2.7 years were studied, with a mean follow-up time of 5.7 ± 4.3 years. 48 patients completed the IVCY course. 36 patients had abnormal renal function and 72 patients had normal renal function at the start of therapy. Both groups responded well initially to treatment; proteinuria reduced to normal levels after 1 and 2 treatments in the normal and abnormal groups, respectively, while creatinine clearance returned to normal levels after 8 treatments in the abnormal group. Overall survival was not different between the two groups; however, the abnormal renal function group had a higher crude mortality rate than the normal group (13/36 vs 10/72, p value = 0.02). At the time of analysis, some patients who had completed their IVCY course still required other therapy to control their disease activity.

Conclusion: Three years of IVCY treatment provided similar outcomes in both normal and abnormal renal function groups. Immediate outcomes were favorable but long-term remission was not promising.

MeSH terms

  • Administration, Intravenous
  • Adolescent
  • Anti-Inflammatory Agents / therapeutic use*
  • Child
  • Child, Preschool
  • Creatinine / blood
  • Creatinine / urine
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / therapeutic use*
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Kaplan-Meier Estimate
  • Lupus Nephritis / complications
  • Lupus Nephritis / drug therapy*
  • Lupus Nephritis / physiopathology*
  • Male
  • Methylprednisolone / therapeutic use*
  • Prednisolone / therapeutic use*
  • Proteinuria / etiology
  • Recurrence
  • Retrospective Studies
  • Severity of Illness Index
  • Survival Rate
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Prednisolone
  • Creatinine
  • Methylprednisolone