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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.

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

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2.

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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3.

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

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4.

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

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5.

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

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6.

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

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7.

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

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8.

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

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9.

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

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10.

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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11.

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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12.

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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13.

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

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14.

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

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15.

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

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16.

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

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17.

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

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18.

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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19.

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

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20.

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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21.

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

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22.

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

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23.

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

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24.

OMIM.ORG article

Omim 614692 external link
25.

Orphanet article

Orphanet ID 97560 external link
26.

Wikipedia article

Wikipedia EN (Membranous_glomerulonephritis) external link
Update: Aug. 14, 2020
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