Targeting Complement Activation in Antineutrophil Cytoplasmic Autoantibodies (ANCA)-Vasculitis - Eculizumab

Inclusion Criteria:• Patients with active Antineutrophil Cytoplasmic Autoantibodies (ANCA)
glomerulonephritis and/or small vessel vasculitis with de novo or relapsing disease
(BVAS≥5).

- Patients must have a current or a history of positive ANCA by the ELISA technique.

- De novo or relapsing disease requiring immunosuppression.

- Patients must have evidence of active glomerulonephritis as evidenced by the presence
of glomerular hematuria (dysmorphic Red Blood Cells (RBCs) or RBC casts) with or
without an increase in serum creatinine.

- Patients will be eligible within 10 days of commencing induction therapy (i.e., they
may have already received pulse methylprednisolone and first dose of
cyclophosphamide).

Exclusion Criteria:• Pregnancy or lactation, or women of child bearing potential who are
not willing or able to comply with 2 contraceptive methods.

- Patients with severe renal failure: creatinine > 6 mg/dL or receiving hemodialysis
and/or receiving plasmapheresis therapy.

- Patients with severe pulmonary hemorrhage requiring ventilation and/or plasmapheresis
therapy.

- Patients with active bacterial or viral infection.

- Absolute neutrophils count < 1000/mm^3 to minimize the risk of infections

- Hemoglobin < 8.5 g/dL

- Prior therapy with a monoclonal antibody (for example rituximab)within the previous 6
months. Peripheral CD-20 B-cells count <= 1% due to rituximab even longer than 6
months.

- Severe coexisting conditions precluding immunosuppressive therapy or conditions
requiring intravenous antibiotic therapy.

- History of infection with Hepatitis B virus (HBV), Hepatitis C virus (HCV), HIV,
tuberculosis or syphilis.