Treating C3 Glomerulopathy

Medications

  • ACE Inhibitors – these are a class of drugs that may benefit in two ways. They help to lower blood pressure and also work to reduce the amount of protein that leaks into the urine. They are recommended in general in the majority of patients with kidney disease.

  • Immunosuppressive agents eg.  Prednisolone, MMF, cyclophosphamide, Rituximab. There are a few published reports that this group of medications may be helpful in some patients with with C3G. They work by suppressing the body’s immune response. 

 

Plasma exchange

  • Your physician may carry out a procedure known as plasma exchange. The purpose of this is to remove the plasma from your body that contains the faulty complement proteins and replace it with fresh plasma containing non-faulty proteins

  • This procedure normally lasts a few hours and you may need a line to be inserted into one of your larger blood vessels for it to be carried out.

 

Dialysis

  • Around 50% of patients with C3G develop end-stage kidney disease within 10 years and require some form of renal replacement therapy.

  • Dialysis is the most common and easily available form of renal replacement therapy.

  • If your kidney doctors think you may need dialysis they and their team will discuss all available options with you well in advance.

 

Transplantation

  • Patients with end-stage kidney disease resulting from C3G can be assessed for their suitability for a kidney transplant.

  • Unfortunately a kidney transplant does not cure the underlying fault in the complement system that caused the damage so the disease may re-occur in the transplanted kidney.

  • Around 50% of patients will get recurrence of the disease in the transplanted kidney – this may lead to failure of the transplanted kidney. In DDD this recurrence rate may be even higher.

 

Eculizumab

  • Eculizumab is currently licensed for use in two other diseases caused by abnormal complement activation – Atypical Haemolytic Uraemic Syndrome (aHUS) and Paroxysmal Nocturnal Haemoglobinuria (PNH).

  • Unlike in aHUS and PNH, Eculizumab is not licensed for C3G. However, there are some reports of the successful use of Eculizumab in certain cases of C3G.

  • In February 2017 NHS England agreed to fund the use of Eculizumab for treatment of recurrence of C3G in the kidney transplant in England.

  • NHS England have made it very clear that  Eculizumab can only be funded if a number of criteria have been met.

  • Your local physician will need to contact the NRCTC if they feel you might benefit from Eculizumab.

  • The full guidance can be read below

  https://www.england.nhs.uk/wp-content/uploads/2017/02/clin-comms-policy-16054p.pdf

  • It should be noted that if Eculizumab is funded for use, it would be for 4 months only.