Management of C3G

Management of C3G

 

The evidence base for treatments in C3G is limited. There was one randomised control trial in MPGN of high-dose steroids in a paediatric cohort. There was one retrospective observational study of MMF in C3G. The following table is adapted from the KDIGO recommendations that are based upon expert opinion only and may be applicable to C3G in native kidney and in recurrent disease following renal transplant.

 

All patients with C3G

Recommendation

  • Optimal blood pressure control include angiotensin converting enzyme inhibitors and angiotensin receptor blockers
  • Optimal nutrition for both normal growth in children and healthy weight in adults
  • Lipid control

 

Patients with Moderate disease

Defined by the following (despite supportive therapy )

  • Urine protein over 500 mg/24 h
  • Renal Biopsy showing Moderate inflammation
  • Recent increase in serum creatinine suggesting risk for progressive disease

Recommendation

  • Prednisone
  • Mycophenolate mofetil

 

Patients with Severe disease

Defined by the following (despite immunosuppression and supportive therapy)

  • Urine protein over 2000 mg/24h

  • Renal biopsy showing Severe inflammation represented by marked endo- or extracapillary proliferation with or without crescent formation

  • Increased serum creatinine suggesting risk for progressive disease at onset

Recommendation

  • Methylprednisolone pulse dosing as well as other anti-cellular immune suppressants have had limited success in rapidly progressive disease. This could include cyclophosphamide or plasma exchange.
  • Data are insufficient to recommend eculizumab as a first-line agent for the treatment of rapidly progressive disease