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Table 1 Standard therapeutic approach and escalation therapies

From: General principles and escalation options of immunotherapy in autoantibody-associated disorders of the CNS

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.
  1. §Treatment duration depending on the individual relapse risk in different diseases.
  2. *Consider re-infusion already by beginning repopulation. Intervals can be usually prolonged in case of sustained depletion and clinical stabilization in patients > 50 years old and/or after several years of rituximab therapy
  3. #Absolute dose depends on leucocyte nadir. Due to toxicity a lifetime cumulative dosage is limited. Accordingly intervals can be prolonged or therapy can be switched in case of clinical stabilization.