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 (AE22) 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 ¼ 0.002). Twenty-one of the 26 patients had GFR !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.

Clinical characteristics of children with membranous lupus nephritis: the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry

“…This statistic highlights the risk of poor renal outcome for patients with pure class V LN, which has been reported elsewhere. 2,9,[12][13][14][15] This risk may explain the use of more-aggressive treatment for patients with pure MLN, even in the absence of nephrotic-range proteinuria.…”

“…9 Although outcomes for pure class V LN are generally thought to be favorable, renal insufficiency and end-stage renal disease (ESRD) do occur, with reported rates of ESRD ranging from 3% to 28% across adult studies and 0% to 25% in pediatric studies. 2,4,9,[11][12][13][14][15][16][17][18][19] Reliable predictors of ESRD for patients with pure class V LN have not been identified.…”

Pediatric lupus nephritis: more options, more chances?

“…23 Membranous LN (class V) is a less common form of LN, but some studies have found that its impact is almost as high as diffuse proliferative LN. 24,25 Treatment Most therapies for LN are based on trials conducted in adults rather than in children, as more adults are affected and thus available for study. To date there are no large and comprehensive singlecenter studies in children with LN because of the relatively small sample sizes available.…”

Immunoadsorption: A New Strategy to Induce Remission in Membranous Lupus Nephritis

“…MN can manifest in a clinical setting of lupus nephritis, hepatitis B, Epstein Barr virus and others [1, 6, 7, 8]. Other rare causes have been described including C4 complement deficiency and antitubular basement membrane antibodies [9].…”