Update on Membranoproliferative GN

Abstract

Membranoproliferative GN represents a pattern of injury seen on light microscopy. Historically, findings on electron microscopy have been used to further subclassify this pathologic entity. Recent advances in understanding of the underlying pathobiology have led to a proposed classification scheme based on immunofluorescence findings. Dysregulation of the complement system has been shown to be a major risk factor for the development of a membranoproliferative GN pattern of injury on kidney biopsy. Evaluation and treatment of this complex disorder rest on defining the underlying mechanisms.

Clinical features and pathogenesis of membranoproliferative glomerulonephritis: a nationwide analysis of the Japan renal biopsy registry from 2007 to 2015

“…Membranoproliferative glomerulonephritis (MPGN), also termed as mesangiocapillary glomerulonephritis, is a morphological pattern of injury characterized by mesangial hypercellularity, endocapillary proliferation, capillary wall remodeling, double contour formation, and duplication of basement membranes on light microscopy [1,2]. In the clinical setting, MPGN is one of the most common causes of nephrotic syndrome in both children and adults, and accounts for approximately 2-10% of all cases of biopsy-confirmed glomerulonephritis [3][4][5][6].…”

“…In the clinical setting, MPGN is one of the most common causes of nephrotic syndrome in both children and adults, and accounts for approximately 2-10% of all cases of biopsy-confirmed glomerulonephritis [3][4][5][6]. Traditionally, MPGN has been classified based on the findings of electron microscopy as primary MPGN type I (MPGN I), MPGN II, MPGN III, or secondary MPGN [1,2]. MPGN I, the most common form, is characterized by subendothelial deposits, and MPGN III has both subepithelial and subendothelial deposits, whereas MPGN type II is characterized by dense deposits in the glomerular basement membrane (dense deposit disease [DDD]) [1,2].…”

“…Traditionally, MPGN has been classified based on the findings of electron microscopy as primary MPGN type I (MPGN I), MPGN II, MPGN III, or secondary MPGN [1,2]. MPGN I, the most common form, is characterized by subendothelial deposits, and MPGN III has both subepithelial and subendothelial deposits, whereas MPGN type II is characterized by dense deposits in the glomerular basement membrane (dense deposit disease [DDD]) [1,2]. Together with DDD, this group of C3-positive immunoglobulinnegative glomerular diseases has been labeled as C3 glomerulopathies [7,8].…”

Clinical and histological features of patients with membranoproliferative glomerulonephritis classified by immunofluorescence findings

“…In other study, MPGN with none deposition seen on IF was introduced with other group and could be secondary to membrane reactions, as in thrombotic microangiopathy. 6 In this new classification the use of electron microscopy is mandatory in cases of exclusive C3 deposition, in order to differentiate DDD and C3 glomerulonephritis. 5 The new classification proposed by Sethi and Fervenza 5 emphasizes the various mechanisms involved in the pathogenesis of MPGN, drawing distinctions between MPGN mediated by immune complexes, which had immunoglobulin deposition, and MPGN resulting from abnormalities of the alternative complement pathway with C3 deposition only.…”

Membranoproliferative Glomerulonephritis Preceding Non-Hodgkin Lymphoma Recurrence: A Case Report

“…Membranoproliferative GN (MPGN) accounts for approximately 7%-10% of biopsy-confirmed GN [1]. Clinical presentation varies between mild hematuria/proteinuria and nephrotic syndrome.…”