Dense Deposit Disease Highlights
DDD a rare disease. It is a type of C3 glomerulopathy (C3G). C3G has two major subtypes - DDD and C3 glomerulonephritis (C3GN).
DDD affects both sexes equally. Within 10 years of diagnosis, about half of persons progress to end-stage renal disease.
The diagnosis of DDD requires renal biopsy. The biopsy will show electron dense deposits in the glomerular basement membrane on electron microscopy. Eye problems can develeop and so persons with DDD should see an eye doctor.
The cause of DDD is related to uncontrolled systemic activation of the alternative pathway of the complement cascade.
Most current treatments for DDD are ineffective. However, some persons with DDD have mutations in a gene called Factor H (CFH). For these patients, plasma exchange can control complement activation and prevent end stage renal disease. Some patients also have an elevated biomarker called soluble C5b-9. For these patients, Eculizumab may slow the progression of disease.
Genetic testing and biomarker profiling should be offered to all patients with DDD.
For reviews of C3G focused on DDD and C3GN please see
Nester CM, Smith RJ. Diagnosis and treatment of C3 glomerulopathy. Clin Nephrol 2013 Sep 2 [Epub ahead of print]; 80:395-403, 2013.
Xiao X, Pickering MC, Smith RJH. C3 Glomerulopathy: The genetic and clinical findings in Dense Deposit Disease and C3 Glomerulonephritis. Semin Thromb Hemost 2014 May 5 [Epub ahead of print]; 40(4):465-71, 2014.