Familial lobular glomerulopathy

A family with an unusual lobular glomerulopathy is described. Renal tissue from three males and one female in two successive generations was available for review. The glomerulopathy was characterized by a marked lobular accentuation with only a modest increase in mesangial cellularity. Immunofluorescence in two patients showed focal or diffuse staining with immunoglobulins G, A, M, and C3 in the mesangium and along the glomerular capillary basement membranes. Ultrastructural study showed amorphous granular subendothelial material distending capillary loops and mesangial regions. This material accounted for the pronounced lobular accentuation. The patients in this family presented with proteinuria, hematuria, and hypertension. Three of the four patients have sustained cerebral vascular accidents and two have died. This family is compared with a previously reported family that showed similar glomerular pathology.