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