C3 Glomerulopathy Highlights

C3G is a rare disease. There are two major sub-types - DDD and C3GN.

C3G affects both sexes equally.  In general, within 10 years of diagnosis about half of persons progress to end-stage renal disease. 

The diagnosis of C3G 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 C3G should see an eye doctor.

The cause of C3G is related to uncontrolled systemic activation of the alternative pathway of the complement cascade. 

Most current treatments for C3G are ineffective.  However, some patients have an elevated complement biomarker called soluble C5b-9. For these patients, Eculizumab may slow the progression of disease.

Genetic testing, autoantibody testing, and complement studies should be obtained on all patients with C3G.  

For reviews of C3G 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.
PDF iconkidneeds_nester_and_smith_clin_neph.pdf

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.
PDF iconkidneeds_c3g_sem_throm_and_hemo.pdf