Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

Membranous nephropathy

Membranous nephropathy is well characterized histomorphologically, pathophysiologically, and clinically. Histomophological findings include immunoglobulin (IgG4) and C3 deposits that that impose a diffuse thickening of the glomerular basement membrane. In electron microscopy these deposits are electron dense. Pathophysiologically autoantibodies against podocyte antigens (PLA2R1) and the lectin pathway play a role. Clinically, the patients present with severe nephrotic syndrome.

Classification

Two forms can be distinguished the primary or idiopathic form comprises 75% of cases. The secondary form is due to diseases such as lupus erythematoides, hepatitis B, drugs, or malignant tumors.

Epidemiology

Incidence is 1.7:100,000 with male predominance (2:1).[Error: Macro 'ref' doesn't exist]

Pathogenesis

Autoantibodies against podocyte antigens play crucial rote in pathogenesis, and polymorphisms in the PLA2R1 und HLA-DQA1 genes are strongly correlated to the development of the disease.

Systematic

Glomerulonephritis
C3 glomerulopathy
CFHR5 Nephropathy
Goodpasture syndrome
Lupus erythematosus nephritis
Membranoproliferative glomerulonephritis (MPGN)
Membranous nephropathy
HLA-DQA1
PLA2R1
Mesangioproliferative glomerulonephritis

References:

1.

Stanescu HC et al. (2011) Risk HLA-DQA1 and PLA(2)R1 alleles in idiopathic membranous nephropathy.

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2.

Lv J et al. (2013) Interaction between PLA2R1 and HLA-DQA1 variants associates with anti-PLA2R antibodies and membranous nephropathy.

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3.

Debiec H et al. (2014) Immunopathogenesis of membranous nephropathy: an update.

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4.

Smyth LJ et al. (2014) Genetic and epigenetic factors influencing chronic kidney disease.

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5.

None (2015) Anti-phospholipase A2 Receptor Antibody and Immunosuppression in Membranous Nephropathy: More Evidence for Pathogenicity of Anti-phospholipase A2 Receptor Autoantibodies.

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6.

Lai WL et al. (2015) Membranous nephropathy: a review on the pathogenesis, diagnosis, and treatment.

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7.

Li ZQ et al. (2015) Efficacy and safety of tacrolimus vs. cyclophosphamide for idiopathic membranous nephropathy: A meta-analysis of Chinese adults.

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8.

Ronco P et al. (2015) Membranous nephropathy: A fairy tale for immunopathologists, nephrologists and patients.

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9.

Ronco P et al. (2015) Pathophysiological advances in membranous nephropathy: time for a shift in patient's care.

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10.

Ruggenenti P et al. (2015) Anti-Phospholipase A2 Receptor Antibody Titer Predicts Post-Rituximab Outcome of Membranous Nephropathy.

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11.

Francis JM et al. (2016) Membranous Nephropathy: A Journey From Bench to Bedside.

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12.

Gentile G et al. (2016) Novel Biomarkers for Renal Diseases? None for the Moment (but One).

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13.

Hihara K et al. (2016) Anti-Phospholipase A2 Receptor (PLA2R) Antibody and Glomerular PLA2R Expression in Japanese Patients with Membranous Nephropathy.

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14.

Li Y et al. (2016) Single-nucleotide polymorphisms in the PLA2R1 gene are associated with systemic lupus erythematosus and lupus nephritis in a Chinese Han population.

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15.

Radice A et al. (2016) Clinical usefulness of autoantibodies to M-type phospholipase A2 receptor (PLA2R) for monitoring disease activity in idiopathic membranous nephropathy (IMN).

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16.

Sinico RA et al. (2016) Immunology of membranous nephropathy: from animal models to humans.

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17.

Wuttke M et al. (2016) Insights into kidney diseases from genome-wide association studies.

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18.

Short CD et al. (1984) Familial membranous nephropathy.

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19.

Debiec H et al. (2002) Antenatal membranous glomerulonephritis due to anti-neutral endopeptidase antibodies.

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20.

Bockenhauer D et al. (2008) Familial membranous nephropathy: an X-linked genetic susceptibility?

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21.

Debiec H et al. (2011) PLA2R autoantibodies and PLA2R glomerular deposits in membranous nephropathy.

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22.

Tomas NM et al. (2014) Thrombospondin type-1 domain-containing 7A in idiopathic membranous nephropathy.

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23.

Maisonneuve P et al. (2000) Distribution of primary renal diseases leading to end-stage renal failure in the United States, Europe, and Australia/New Zealand: results from an international comparative study.

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24.

OMIM.ORG article

Omim 614692 [^]
25.

Orphanet article

Orphanet ID 97560 [^]
26.

Wikipedia article

Wikipedia EN (Membranous_glomerulonephritis) [^]
Update: April 29, 2019