First-line therapies | |
Methylprednisolone | 1000 mg/day for 5 days, if needed with oral tapering |
 Intravenous immunoglobulin | 0.4 g/kg/day over 5 days |
 Plasma exchange or immunoadsorbtion | 5–7 cycles |
 +Tumor therapy in paraneoplastic cases as soon as possible! | |
Escalation immunotherapies§ | |
 Rituximab | Initially 500–2000 mg IV, followed by 250–1000 mg every 6 months or depending on B-cell repopulation* |
 Cyclophosphophamide | Induction with 750–1000 mg/m2 of BSA (e.g. 300–350 mg/m2/d over 3 days), followed by 500–750 mg/m2 of BSA every 4 weeks# |
Further long-term immunotherapies§ | |
 Intravenous immunoglobulin | 1 g/kg body weight every 4–6 weeks IV, alternatively subcutaneously in equivalent dose (home setting) |
 Oral immunosuppressive drugs alone or in combination with prednisolone | |
 Azathioprine | 2–3 mg/kg/d |
 Methotrexate | 7.5–20 mg/week |
 Mycophenolate mofetil | 1000–2000 mg/kg/d |
Reserve therapies in refractory disease course | |
 Tocilizumab | 8 mg/kg every 4 weeks |
 Bortezomib | 1–2 cycles with 1.3 mg/m2/cycle s.c., administered on days 1, 4, 8, 11, followed by other long-term therapy. |