Treatment

Regimen

First-line immunotherapy

Methylprednisolone

1 g daily, for 3 - 5 days

Intravenous immunoglobulin

2 g/kg over 5 days (400 mg/kg/day)

Plasma exchange

1 session every other day for 5 - 7 days

Second-line immunotherapy

Rituximab

375 mg/m² weekly intravenous infusion for 4 weeks

Cyclophosphamide

750 mg/m² monthly for 3 - 6 months

Alternative treatments

Tocilizumab

Initially 4 mg/kg, followed by an increase to 8 mg/kg monthly based on clinical response

Low-dose interleukin-2 (aldesleukin)

1.5 million IU/day, 4 subcutaneous injections with 3-week interval

Steroid-sparing agents used for maintenance therapy

Azathioprine

Initially 1 - 1.5 mg/kg once daily or divided twice daily, target 2 - 3 mg/kg/day

Mycophenolate mofetil

Initially 500 mg twice daily, target 1000 mg twice daily.