Table 1.

Standard therapeutic approach and escalation therapies

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.

§Treatment duration depending on the individual relapse risk in different diseases.

*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

#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.