Membranoproliferative Glomerulonephritis

Braun, Michael C.; Licht, Christoph; Strife, C. Frederic

doi:10.1007/978-3-540-76341-3_32

Cited by 4 publications

(3 citation statements)

References 93 publications

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“…Complement perturbation in type III MPGN is thought to be related to a slow-acting nephritic factor that stabilizes a properdin dependent C5-convertase, (Cb3)2BbP, activating the terminal pathway; hence, the term nephritic factor of the terminal pathway (NeFt) [ 14 ]. This nephritic factor has not been reported in healthy subjects, unlike C3NeF.…”

Section: Pathogenesismentioning

confidence: 99%

Membranoproliferative glomerulonephritis

2009

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Membranoproliferative glomerulonephritis is an uncommon kidney disorder characterized by mesangial cell proliferation and structural changes in glomerular capillary walls. It can be subdivided into idiopathic and secondary forms, which are differentially diagnosed by a review of clinical features, laboratory data, and renal histopathology. Three types—I, II, and III—have been defined by pathologic features. All three types are associated with hypocomplementemia, but they manifest somewhat different mechanisms of complement activation. Type II, also known as “dense deposit disease”, is associated with the presence of C3-nephritic factor. Membranoproliferative glomerulonephritis primarily affects children and young adults, with patients presenting with nephrotic or nephritic syndrome or with asymptomatic renal disease. This type of glomerulonephritis often progresses slowly to end-stage renal disease, and it tends to recur after renal transplantation, especially type II. The efficacy of various forms of treatment remains controversial; however, long-term steroid treatment seems to be effective in children with nephrotic-range proteinuria. Improvement in renal outcomes largely relies on the evaluation of more selective agents in carefully controlled studies.

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Section: Pathogenesismentioning

confidence: 99%

Membranoproliferative glomerulonephritis

2009

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“…The finding of immune complexes with cellular inflammatory infiltrate suggests an immune mediated process. In some cases, a genetic basis of the diseases is evident, particularly in families [26] with inherited defects of the complement system [27–29]. Only a few cases have been reported with C4 deficiency [18–21,30] and one with isolated C4B deficiency [19].…”

Section: Discussionmentioning

confidence: 99%

Familial C4B deficiency and immune complex glomerulonephritis

Soto

Ortiz

et al. 2010

Clinical Immunology

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Homozygous complement C4B deficiency is described in a Southern European young female patient with Membranoproliferative Glomerulonephritis (MPGN) type III characterized by renal biopsies with strong complement C4 and IgG deposits. Low C4 levels were independent of clinical evolution or type of immunosuppression and were found in three other family members without renal disease or infections. HLA typing revealed that the patient has homozygous A*02, Cw*06, B*50 at the class I region, and DRB1*08 and DQB1*03 at the class II region. Genotypic and phenotypic studies demonstrated that the patient has homozygous monomodular RCCX in the HLA class III region, with single long C4A genes coding for C4A3 and complete C4B deficiency. Her father, mother, son and niece have heterozygous C4B deficiency. The patient's deceased brother had a history of Henoch-Schönlein Purpura (HSP), an immune complex-mediated proliferative glomerulonephritis. These findings challenge the putative pathophysiological roles of C4A and C4B and underscore the need to perform functional assays, C4 allotyping and genotyping on patients with persistently low serum levels of a classical pathway complement component and glomerulopathy associated with immune deposits.

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“…The term membranoproliferative glomerulonephritis (MPGN) defines a heterogeneous group of kidney diseases that frequently lead to kidney failure [1]. Histologically, it is characterized by mesangial interposition and the duplication of glomerular basement membranes that are associated with immune deposits, all of which give an appearance of hyperlobulated glomeruli.…”

Section: Introductionmentioning

confidence: 99%

A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl

et al. 2013

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C3 glomerulopathy defines a subgroup of membranoproliferative glomerulonephritis (MPGN) characterized by complement 3 (C3)-positive, immunoglobulin-negative deposits in immunofluorescence microscopy. It comprises 3 clinical conditions: dense deposit disease, C3 glomerulonephritis, and complement factor H-related 5 (CFHR5) nephropathy. Mutations in genes encoding regulatory proteins of the alternative complement pathway have been described. A 16-year-old girl was admitted to the hospital due to periorbital edema. Nephrotic syndrome accompanied by low C3 level was diagnosed. Renal biopsy showed MPGN in light microscopy, only C3 deposits in immunofluorescence microscopy, and subendothelial electron dense deposits and capillary basement membrane thickening with double contour formation in electron microscopy. C3 nephritic factor and anti complement factor H antibody were negative. Complement factor H level was normal. Genetic screening showed a novel heterozygous p.Cys269Arg variation in the CFHR5 gene without any mutation in CFH and CFI genes. Eculizumab therapy was started but was unsuccessful at 10 months of follow-up. We have identified a novel heterozygous variation in CFHR5-related nephropathy presenting with nephrotic syndrome and persistently low C3 level, thus expanding the genetic and phenotypic spectrum of the disease. Eculizumab seems to be ineffective in this subtype.

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Membranoproliferative Glomerulonephritis

Cited by 4 publications

References 93 publications

Membranoproliferative glomerulonephritis

Membranoproliferative glomerulonephritis

Familial C4B deficiency and immune complex glomerulonephritis

A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl

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