Idiopathic membranous nephropathy, associated with HLA-DRw3 and not related to monocyte-phagocyte system Fc receptor dysfunction, in father and son

Familial idiopathic membranous nephropathy, an immune-complex-associated glomerulopathy, has not been previously reported in father and son, despite its striking immunogenetic correlation, especially with HLA-DR3. As a dysfunction of the monocyte-phagocyte system (MPS), it has been observed linked t...

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Published inNephron (2015) Vol. 58; no. 3; p. 320
Main Authors Mezzano, S, Rojas, G, Ardiles, L, Caorsi, I, Bertoglio, J C, Lopez, M I, Kunick, M, Elgueta, S
Format Journal Article
LanguageEnglish
Published Switzerland 1991
Subjects
Adolescent
Adult
Antigen-Antibody Complex - blood
Enzyme-Linked Immunosorbent Assay
Glomerulonephritis, Membranous - blood
Glomerulonephritis, Membranous - genetics
Glomerulonephritis, Membranous - immunology
Glomerulonephritis, Membranous - physiopathology
HLA-DR3 Antigen - immunology
Humans
Male
Monocytes - physiology
Monocytes - ultrastructure
Phagocytes - physiology
Phagocytes - ultrastructure
Phagocytosis - physiology
Receptors, Fc - physiology
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Summary:Familial idiopathic membranous nephropathy, an immune-complex-associated glomerulopathy, has not been previously reported in father and son, despite its striking immunogenetic correlation, especially with HLA-DR3. As a dysfunction of the monocyte-phagocyte system (MPS), it has been observed linked to DR3 antigen, so we studied the MPS Fc receptor function in a father and his son with a histologically proven membranous nephropathy, associated with the haplotype A9-B35-DR3-DQw2. The Fc receptor function of the MPS was examined by measuring the clearance of IgG-sensitized, 51Cr-labeled erythrocytes and by measuring the ability of isolated monocytes to ingest autologous red blood cells coated with IgG anti-Rh (D) antibody. Immune clearance and in vitro phagocytosis was normal in both patients and not related to their levels of immune complexes (as measured by ELISA C1q and Conglutinin solid-phase binding assay). This report suggest that genetic factors may play an important role in the development of membranous nephropathy, and it seems not to be related to a dysfunction of MPS as measured by these tests.
ISSN:1660-8151
2235-3186
DOI:10.1159/000186444