C3G and IC-MPGN

C3G and IC-MPGN represent a group of chronic kidney disorders characterized by abnormal activation of the alternative complement pathway and typically manifest with proteinuria, hypertension, and progressively impaired kidney function.

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• The disease affects 1-2 per 10,000 individuals, and the annual incidence of newly diagnosed patients is 2 per million. The disease occurs in children and adults alike. The long-term prognosis of MPGN/C3G is poor, with a substantial fraction of patients progressing to end-stage kidney disease.
   

• The etiology of the disorder is complex and involves both genetic and immunological abnormalities. The disease group is classified according to histopathological, immunological and genetic criteria into individual disease entities, i.e. dense deposit disease (DDD), C3 glomerulonephritis (C3GN) and immune complex (IC)-MPGN.  
   

• Therapeutic options for the disorder are largely limited to non-specific immunosuppressants, which are often ineffective and can have serious side effects. Even kidney transplantation may not provide an ultimate ‘cure’ for the disease since C3G tends to recur in the transplant kidney, leading to early graft loss. Currently there is no approved specific therapy for C3G. 
   

The C3G awareness day is 25. September.