Background: Membranoproliferative glomerulonephritis (MPGN) is a relatively uncommon cause of progressive renal disease characterized by immune complex deposition resulting in mesangial proliferation and endocapillary inflammation with capillary wall thickening and double contour formation. Although a familial linkage has been reported in MPGN type II disease and less often in type I disease, a familial linkage in type III disease has not been reported previously.
Methods: We identified a family in which MPGN type III developed in a living-related donor 12 years later and recurred in the renal allograft of his son, whose primary disease was MPGN type III. We screened the members of the extended family, looking for evidence of hematuria and proteinuria. Renal biopsy specimens exhibited the findings of subendothelial deposits, subepithelial deposits, and complex glomerular basement membrane changes with C3 but not IgG seen on immunofluorescence.
Results: Screening identified eight affected family members (six biopsy proven) over three generations. The condition is inherited in an apparent autosomal dominant fashion.
Conclusion: This is the first description of familial MPGN type III. We hope that by studying the disease in this family group, we may learn more about the pathogenesis of the condition.
Copyright 2002 by the National Kidney Foundation, Inc.