NDT Advance Access published online on September 15, 2009
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfp481
Severe paediatric systemic lupus erythematosus nephritis—a single centre experience
1 Pediatric Nephrology, Helen DeVos Children's Hospital and Michigan State University College of Human Medicine, Grand Rapids, MI 2 Pediatric Resident Physicians, University of Minnesota, Minneapolis, MN 3 Department of Family Medicine, Michigan State University College of Human Medicine, East Lansing, MI 4 Pediatric Rheumatology, Helen DeVos Children's Hospital, Grand Rapids, MI, USA
Correspondence and offprint requests to: Timothy E. Bunchman; E-mail: timothy.bunchman{at}devoschildrens.org
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Abstract |
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Background. Paediatric patients with systemic lupus erythematosus (SLE) often have severe presentations including lupus nephritis (LN). Few paediatric studies have evaluated the anticardiolipin antibody (aCL) and renal histology. The purpose of this study was to evaluate clinicopathologic features, including aCL, short-term clinical and renal histologic outcomes of paediatric patients with new-onset SLE nephritis.
Methods. We conducted a single centre, retrospective inception cohort study. Charts were reviewed at presentation (initial renal biopsy), 6-month (follow-up biopsy) and 12-month follow-up.
Results. The population consisted of 21 patients (median age, 14.5 years): 19/21 were female, 6/21 African American, 3/21 Asian, 9/21 Caucasian and 3/21 Hispanic. At presentation, 19/21 had elevated aCL, 15/21 hypertensive, 12/21 nephrotic and 7/21 required haemodialysis (HD)—2/7 HD patients had thrombotic microangiopathy, 1/7 crescentic glomerulonephritis. Two patients had thromboembolism: both had aCL, were taking oral contraceptives and required HD, one was nephrotic and the other had elevated lupus anticoagulant. Initial biopsies revealed 6/21 ISN/RPS class II nephritis, 3/21 class III, 7/21 class IV and 5/21 class V. Treatment consisted of methylprednisolone, corticosteroids, cyclophosphamide or mycophenolate mofetil. Follow-up biopsies revealed 12/13 to have improved histology. Indication for a follow-up biopsy was severe illness at presentation. At 12-month follow-up, no patients were nephrotic (P < 0.001) or required HD (P < 0.001), and 3/14 had elevated aCL (P < 0.001).
Conclusion. Elevated aCL, hypertension, nephrotic syndrome and need for HD were common presentations among our paediatric SLE nephritis population. Renal histology and aCL were helpful in the therapeutic management.
Keywords: anticardiolipin antibody; dialysis; paediatrics; systemic lupus erythematosus
Received for publication: 10. 3.09
Accepted in revised form: 19. 8.09