Characteristics of a benign subtype of dense deposit disease: comparison with the progressive form of this disease.
Pathologic, clinical and serum complement studies were performed on 18 patients with dense deposit disease (DDD). The patients were divided into 3 groups: group A (10 patients who developed end-stage renal failure within 2.9 +/- 1.0 years of onset) group B (3 patients who developed elevated serum creatinines within 6.8 +/- 4.8 years of onset) and group C (5 patients with no evidence of renal insufficiency after 11.0 +/- 1.4 years of follow-up). Renal biopsies from all patients showed intramembranous, electron-dense deposits in glomerular capillary basement membranes. Most group A and B patients presented with a nephritic and/or nephrotic syndrome and followed an active clinical course. In contrast, group C patients presented with either a nephrotic syndrome or asymptomatic proteinuria +/- hematuria and followed a benign course. Sera from group A and B patients contained reduced concentrations of C3 and factor B and large amounts of C3 nephritic factor (NeF) activity. In contrast, serum of group C patients contained normal amounts of C3 and factor B and low levels of C3 NeF activity. These studies show that some patients with DDD (group C) have a benign illness and that they can be identified by serum levels of C3, factor B and C3 NeF activity.
D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis; D003178:Complement C3 Nephritic Factor / Q000032:analysis; D011415:Complement Factor B / Q000032:analysis; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology / Q000648:ultrastructure*; D008297:Male; D008854:Microscopy, Electron; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000473:pathology*
D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D011415:Complement Factor B