NDT Advance Access originally published online on July 29, 2007
Nephrology Dialysis Transplantation 2007 22(12):3495-3500; doi:10.1093/ndt/gfm491
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Clinical presentation and outcome in a cohort of paediatric patients with membranous lupus nephritis*
1Pediatric Nephrology, Pediatric and Adolescent Medicine Department, Mayo Clinic, Rochester, MN 55905, 2Pediatric Nephrology, C.S. Mott Children's Hospital, Ann Arbor, MI 48109, 3Pediatric Nephrology, Department of Pediatrics, Children's Hospital of Michigan, Detroit, MI 48201 and 4Pediatric Rheumatology Department of Pediatrics, C.S. Mott Children's Hospital, Ann Arbor, MI 48109, USA
Correspondence to: C. H. Cramer II, Pediatric Nephrology, 200 First Street SW, Rochester, MN 55095, USA. Email: cramer.carl{at}mayo.edu
| Abstract |
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Background. Membranous glomerulopathy accounts for 28% of the biopsy-proven systemic lupus erythematosus nephritis in paediatric patients at the time of first biopsy, yet minimal data are available regarding outcomes in this population.
Methods. We present a retrospective analysis of 26 paediatric patients with World Health Organization class V lupus nephritis. Patients were subdivided based on renal biopsy findings into the following subclasses: 16 (63%) Va, 2 (9%) Vb, 7 (26%) Vc and 1 (4%) Vd. We evaluated outcomes of renal function and urine protein to creatinine ratio (UPr/UCr).
Results. Mean follow-up time was 38.6 (±22) months. Eight patients at presentation had a glomerular filtration rate (GFR) <90 ml/min/1.73 m2, six with Va and two with Vc. The initial presenting serum creatinine was predictive of renal function at last follow-up in class Va and Vb patients (P = 0.002). Twenty-one of the 26 patients had GFR
90 ml/min/1.73 m2 at last follow-up; the five patients with GFR <90 ml/min/1.73 m2 were all lupus class Va. Cyclophosphamide (CTX) therapy did not demonstrate a significant improvement in outcome. The following parameters failed to predict GFR at last follow-up visit: blood pressure, C3, C4 and UPr/UCr.
Conclusions. The initial creatinine in patients with classes Va and Vb lupus nephritis predicted follow-up renal function. We found no correlation with outcome based on therapy with CTX and/or mycophenolate mofetil.
Keywords: membranous nephropathy; paediatrics; systemic lupus erythematosus
*This manuscript was presented in abstract form at the 2005 Pediatric Academic Societies Meeting in Washington D.C.
Received for publication: 21. 3.07
Accepted in revised form: 28. 6.07