Molekulargenetische Diagnostik
Praxis Dr. Mato Nagel

Membranöse Glomerulonephritis

Die membranöse Glomerulonephritis ist eine histologisch, pathophysiologisch und klinisch gut abgrenzbare Erkrankung. Histologisch finden sich Immunglobulin- (IgG4) und C3-Ablagerungen vornehmlich subendothelial, die die glomerulären Basalmembran diffus verdickt erscheinen lässt. In der Elektronenmikroskopie erweisen sich die subendothelialen Ablagerungen als elektronendicht. Pathophysiologisch scheinen zumindest bei einem Teil der Patienten Autoantikörper gegen Podozytenantigene insbesondere das PLA2R1 eine Rolle zu spielen. Die Komplementaktievierung erfolgt wahrscheinlich über den Lectin-Weg. Klinisch zeigen die Patienten meist ein ausgeprägtes nephrotisches Syndrom.

Einteilung

Es werden zwei Formen unterschieden die primäre oder idiopathische Form (75%) und die sekundäre form infolge anderer Erkrankungen: systemischer Lupus erythematodes, Hepatitis B, Medikamente oder bösartige Tumoren.

Epidemiologie

Die Inzidenz beträgt 1,7:100.000. Es sind überwiegend Männer betroffen (2:1).[1]

Pathogenese

Autoantikörper gegen podozytäre Antigene spielen eine wichtige pathogenetische Rolle. Ihre Entstehung korreliert sehr stark mit Polymorphismen in den Genen PLA2R1 und HLA-DQA1.

Gliederung

Glomerulonephritis
C3 Glomerulopathie
CFHR5 Nephropathie
Goodpasture-Syndrom
Lupus erythematodes Nephritis
Membranoproliferative Glomerulonephritis (MPGN)
Membranöse Glomerulonephritis
HLA-DQA1
PLA2R1
Mesangioproliferative Glomerulonephritis

Referenzen:

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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