Update membranous nephropathy

Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA R1 and THSD7A have been identified as target antigens in 70-80 % and 2-3 % of patients, respectively. The detection of PLA R1- and THSD7A-antibodies in the blood an...

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Published in:Deutsche medizinische Wochenschrift (1946) Vol. 145; no. 20; p. 1481
Main Authors: Hoxha, Elion, Huber, Tobias B
Format: Journal Article
Language:German
Published: Germany 01.10.2020
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ISSN:1439-4413, 1439-4413
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Abstract Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA R1 and THSD7A have been identified as target antigens in 70-80 % and 2-3 % of patients, respectively. The detection of PLA R1- and THSD7A-antibodies in the blood and staining of renal biopsies for the respective antigens allow the correct diagnosis of PLA R1- and THSD7A-associated membranous nephropathy, respectively, in practically 100 % of cases. Measurement of PLA R1- and THSD7A-antibodies is helpful in order to further individualize the decision whether to perform a renal biopsy in patients with suspected membranous nephropathy. PLA R1-antibody level is a strong predictor for remission of proteinuria, loss of renal function and relapse of disease. Moreover, treatment decisions in patients with PLA R1-associated MN are increasingly based on the PLA R1-antibody levels. Rituximab was shown to be non-inferior to ciclosporine A to induce remission of proteinuria after 12 months. After 24 months rituximab was superior to ciclosporine A for the same endpoint. Development of novel treatment strategies, focusing on disease pathogenesis, remains highly relevant for these patients.
AbstractList Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA R1 and THSD7A have been identified as target antigens in 70-80 % and 2-3 % of patients, respectively. The detection of PLA R1- and THSD7A-antibodies in the blood and staining of renal biopsies for the respective antigens allow the correct diagnosis of PLA R1- and THSD7A-associated membranous nephropathy, respectively, in practically 100 % of cases. Measurement of PLA R1- and THSD7A-antibodies is helpful in order to further individualize the decision whether to perform a renal biopsy in patients with suspected membranous nephropathy. PLA R1-antibody level is a strong predictor for remission of proteinuria, loss of renal function and relapse of disease. Moreover, treatment decisions in patients with PLA R1-associated MN are increasingly based on the PLA R1-antibody levels. Rituximab was shown to be non-inferior to ciclosporine A to induce remission of proteinuria after 12 months. After 24 months rituximab was superior to ciclosporine A for the same endpoint. Development of novel treatment strategies, focusing on disease pathogenesis, remains highly relevant for these patients.
Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA2R1 and THSD7A have been identified as target antigens in 70-80 % and 2-3 % of patients, respectively. The detection of PLA2R1- and THSD7A-antibodies in the blood and staining of renal biopsies for the respective antigens allow the correct diagnosis of PLA2R1- and THSD7A-associated membranous nephropathy, respectively, in practically 100 % of cases. Measurement of PLA2R1- and THSD7A-antibodies is helpful in order to further individualize the decision whether to perform a renal biopsy in patients with suspected membranous nephropathy. PLA2R1-antibody level is a strong predictor for remission of proteinuria, loss of renal function and relapse of disease. Moreover, treatment decisions in patients with PLA2R1-associated MN are increasingly based on the PLA2R1-antibody levels. Rituximab was shown to be non-inferior to ciclosporine A to induce remission of proteinuria after 12 months. After 24 months rituximab was superior to ciclosporine A for the same endpoint. Development of novel treatment strategies, focusing on disease pathogenesis, remains highly relevant for these patients.Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA2R1 and THSD7A have been identified as target antigens in 70-80 % and 2-3 % of patients, respectively. The detection of PLA2R1- and THSD7A-antibodies in the blood and staining of renal biopsies for the respective antigens allow the correct diagnosis of PLA2R1- and THSD7A-associated membranous nephropathy, respectively, in practically 100 % of cases. Measurement of PLA2R1- and THSD7A-antibodies is helpful in order to further individualize the decision whether to perform a renal biopsy in patients with suspected membranous nephropathy. PLA2R1-antibody level is a strong predictor for remission of proteinuria, loss of renal function and relapse of disease. Moreover, treatment decisions in patients with PLA2R1-associated MN are increasingly based on the PLA2R1-antibody levels. Rituximab was shown to be non-inferior to ciclosporine A to induce remission of proteinuria after 12 months. After 24 months rituximab was superior to ciclosporine A for the same endpoint. Development of novel treatment strategies, focusing on disease pathogenesis, remains highly relevant for these patients.
Author Huber, Tobias B
Hoxha, Elion
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Snippet Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA R1 and THSD7A have been...
Membranous nephropathy is an autoimmune disease caused in most cases by binding of circulating antibodies to antigens on podocytes. PLA2R1 and THSD7A have been...
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StartPage 1481
SubjectTerms Autoantibodies - blood
Glomerulonephritis, Membranous - diagnosis
Glomerulonephritis, Membranous - immunology
Glomerulonephritis, Membranous - pathology
Glomerulonephritis, Membranous - therapy
Humans
Immunologic Factors - therapeutic use
Kidney Glomerulus - immunology
Kidney Glomerulus - pathology
Proteinuria
Receptors, Phospholipase A2 - immunology
Rituximab - therapeutic use
Thrombospondins - immunology
Title Update membranous nephropathy
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