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 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.
Mezzano
S
S
Renal Division, School of Medicine, Universidad Austral, Valdivia, Chile.
Rojas
G
G
Ardiles
L
L
Caorsi
I
I
Bertoglio
J C
JC
Lopez
M I
MI
Kunick
M
M
Elgueta
S
S
D000293:Adolescent; D000328:Adult; D000936:Antigen-Antibody Complex / Q000097:blood; D004797:Enzyme-Linked Immunosorbent Assay; D015433:Glomerulonephritis, Membranous / Q000097:blood / Q000235:genetics / Q000276:immunology* / Q000503:physiopathology; D015803:HLA-DR3 Antigen / Q000276:immunology*; D006801:Humans; D008297:Male; D009000:Monocytes / Q000502:physiology / Q000648:ultrastructure*; D010586:Phagocytes / Q000502:physiology / Q000648:ultrastructure*; D010587:Phagocytosis / Q000502:physiology; D011961:Receptors, Fc / Q000502:physiology*
D000936:Antigen-Antibody Complex; D015803:HLA-DR3 Antigen; D011961:Receptors, Fc