Membranous glomerulonephritis: treatment response and outcome in children

Abstract

The aim of this study was to characterize clinical features, treatment response, and outcome of idiopathic membranous glomerulonephritis (MGN) in a single-center cohort of children. A retrospective review of biopsy-proven idiopathic MGN in 12 children (mean age 11.9 years) was undertaken. Presentation was nephrotic syndrome (NS) (75%), hematuria/proteinuria (17%), and asymptomatic proteinuria (8%). Ten patients (83%) with NS and nephrotic range proteinuria (NRP) were treated with prednisone, and two patients with non-NRP were not treated with immunosuppressive medications. Steroid response in the treated patients was complete (10%), partial (40%), and absent (50%), respectively. Oral cyclophosphamide was used in seven patients of whom five were steroid resistant, one was steroid dependent, and one was partially responsive. At the mean follow up of 27 months, outcome parameters included an estimated glomerular filtration rate of 128 cc/min per 1.73 m(2), albumin of 4.2 gm/dL, and a urine protein/creatinine ratio of 0.87 [median 0.16 (range 0.02-6.52)]. Remission was complete in 75% of the patients and partial in 17%. One patient (8%) with chronic kidney disease (stage 2) was unresponsive to therapy. Complete remission was significantly associated with the absence of chronic histological changes (p = 0.03). In conclusion, children with NS and/or NRP associated with MGN appear to have a good prognosis when treated with a combination of corticosteroids and cyclophosphamide.

Clinicopathological analysis of idiopathic membranous nephropathy in young adults

“…In most cases, patients were elderly men with continuous massive proteinuria, and the pathological stages were mainly identified as stage III or IV accompanied by renal dysfunction at disease onset, and the progression of tubulointerstitial lesions, as well as poor prognosis associated with FSGS (Wasserstein, 1997;Valentini et al, 2009;Ponticelli and Passerini, 2010b;Praga and Rojas-Rivera, 2012). In the present study, pathological stages were identified as stage I in 16 cases, stage II in 6 cases, and stage III in 1 case.…”

“…Data indicate that a third of IMN patients gradually progress to end-stage renal disease (Valentini et al, 2009), but the factors affecting prognosis are varied in literature. In most cases, patients were elderly men with continuous massive proteinuria, and the pathological stages were mainly identified as stage III or IV accompanied by renal dysfunction at disease onset, and the progression of tubulointerstitial lesions, as well as poor prognosis associated with FSGS (Wasserstein, 1997;Valentini et al, 2009;Ponticelli and Passerini, 2010b;Praga and Rojas-Rivera, 2012).…”

Membranous nephropathy: not just a disease for adults

“…In a pediatric population, there is always the added concern of inducing infertility, however the use of a limited course of low-dose cyclophosphamide can avoid cumulative doses that would be concerning for gonadal toxicity. One option that provides a relatively low exposure to cyclophosphamide (total dose of < 200 mg/kg) involves a 12 week regimen using daily cyclophosphamide (2 mg/kg/day) with alternate-day steroids and was used successfully in a small uncontrolled study [61]. Calcineurin inhibitors may also be effective but, based on experience in adults, they require 6–12 months of therapy with a slow taper to avoid relapse.…”

Chapter 7: Idiopathic membranous nephropathy

“…301, 302, 303 This may also apply to other types of primary GN associated with severe nephrotic syndrome; the evidence base, however, is lacking. There have been no RCTs of prophylactic anticoagulation in IMN with nephrotic syndrome.…”

“…There have been no RCTs of prophylactic anticoagulation in IMN with nephrotic syndrome. 301, 302, 303 …”