Familial IgM mesangial nephropathy: a morphologic and immunogenetic study of three pedigrees.
Eight patients belonging to 3 unrelated families had biopsy-proven IgM mesangial nephropathy. In the first family, the mother and the 2 daughters were affected; in the second, the mother and the son; in the third, 2 sisters and the brother. Two additional sisters of the third family showed a clinical picture consistent with chronic glomerulonephritis. The clinical picture was that of hematuria and/or proteinuria. No patients had nephrotic syndrome. Genealogic investigation enabled us to discover 2 additional affected members in the kindred of the first family (1 with IgA nephropathy, 1 with clinical glomerulonephritis) and 3 other affected members in the pedigree of the third family (1 with IgA nephropathy, 1 with sclerosing glomerulonephritis, 1 with clinical glomerulonephritis). Immunogenetic studies showed the recurrence of an extended haplotype bearing DR beta 11-DQ beta 3B-DQ alpha 2-C4A3-C4B1-BfS in 9 of 10 affected members. Our data suggest that genetic factors may be involved in the mechanism of the disease and support the hypothesis that IgM nephropathy is a distinct disease entity.
Scolari
F
F
Institute of Nephrology, University and Spedali Civili, Brescia, Italy.
Scaini
P
P
Savoldi
S
S
Prati
E
E
Sacchi
G
G
Amoroso
A
A
Mazzola
G
G
Canale
L
L
Borelli
I
I
Cristinelli
L
L
D000293:Adolescent; D000328:Adult; D000368:Aged; D015139:Blotting, Southern; D002648:Child; D005260:Female; D005455:Fluorescent Antibody Technique; D005920:Glomerular Mesangium / Q000276:immunology; D015432:Glomerulonephritis, Membranoproliferative / Q000235:genetics*; D006683:HLA-DQ Antigens / Q000235:genetics; D006684:HLA-DR Antigens / Q000235:genetics; D006239:Haplotypes; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010375:Pedigree
D006683:HLA-DQ Antigens; D006684:HLA-DR Antigens; D007075:Immunoglobulin M