C3 Glomerulopathy (C3G) and Immune Complex Membranoproliferative Glomerulonephritis (IC-MPGN)

Understanding C3G and IC-MPGN

C3G and IC-MPGN are rare kidney diseases. In both diseases, an important part of the immune system known as the complement cascade is overactive, which results in the excessive breakdown of a protein called C3.1 These C3 breakdown products become trapped in the kidney, causing inflammation and damage to the organ.1

Although IC-MPGN is a distinct disease from C3G, the underlying cause and progression of the two diseases are remarkably similar.2,3 The signs and symptoms of C3G and IC-MPGN include blood in the urine (hematuria); dark foamy urine due to the presence of protein (proteinuria); edema (swelling), often in the legs, although any part of the body can be affected; high blood pressure; and decreased urine output.4

There are no medicines currently approved for C3G or IC-MPGN, so there is a substantial need for medicines that target the cause of the diseases. C3G and IC-MPGN can lead to kidney failure within five to 10 years of diagnosis, requiring these patients to go on dialysis or get a kidney transplant.5

Pegcetacoplan (APL-2) in C3G and IC-MPGN

Pegcetacoplan is a targeted C3 inhibitor designed to regulate uncontrolled or excessive complement activation. We believe it may have the potential to target the underlying disease processes of C3G and IC-MPGN.

Interested in our clinical trials in C3G?

For clinical trial information, please email us at clinicaltrials@apellis.com.

Resources

References

  1. Genetics Home Reference. Available at: https://ghr.nlm.nih.gov/condition/c3-glomerulopathy. Accessed November 27, 2019.
  2. Noris M, Donadelli R, Remuzzi G. Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy. Pediatr Nephrol. 2019 Aug;34(8):1311-1323.
  3. Cook HT. Evolving complexity of complement-related diseases: C3 glomerulopathy and atypical haemolytic uremic syndrome. Curr Opin Nephrol Hypertens. 2018 May;27(3):165-170.
  4. Complement 3 Glomerulopathy (C3G). National Kidney Foundation Website. https://www.kidney.org/atoz/content/complement-3-glomerulopathy-c3g. Accessed November 21, 2019.
  5. C3 glomerulopathy. National Institute of Health, Genetics Home Reference. https://ghr.nlm.nih.gov/condition/c3-glomerulopathy#resources. Accessed November 21, 2019.