Clinical presentation and outcome in a cohort of paediatric patients with membranous lupus nephritis

Abstract

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 m(2), 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 > or = 90 ml/min/1.73 m(2) at last follow-up; the five patients with GFR <90 ml/min/1.73 m(2) 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.