Table 4.

Therapeutic agents used in PACNS.

Treatment	Regimen
Acute therapy
Corticosteroids	oral prednisone at 1 mg/ kg/day or methylprednisolone pulse IV (1000 mg daily for 3–5 days)
Cyclophosphamide	daily oral dose (2 mg/kg/day) or by monthly intravenous pulse dose (e.g. starting at 750 mg/m2)
Maintenance therapy
Azathioprine	1–2 mg/kg daily
Methotrexate	20–25 mg/week
Mycophenolate mofetil	1–2 g daily
Biological agents
Rituximab	375 mg/m2/week for 4 weeks or 2 IV doses of 1 g each, administered 2 weeks apart
Tumour necrosis factor-α blockers
Infliximab	single IV infusion (5 mg/kg)
Etanercept	25 mg twice weekly for 20 months, then 25 mg/kg, once weekly for 8 months

IV, intravenous; PACNS, primary angiitis of the central nervous system.