Table 1.

Summary of immunomodulatory therapy aspects in SPSD.

IVIg is considered the first-line treatment where available Corticosteroids and plasmapheresis are other options of first-line treatment In spite of a negative trial, Rituximab is useful in some cases aHSCT may be a treatment option in treatment refractory cases FcRn-targeted therapies, interleukin-6-receptor antagonists, plasmablast- and plasma cell-targeting therapies and the proteasome inhibitor bortezomib are used in other neuroimmunological diseases and might be possible treatment options in the future There is an unmet need for biomarkers to evaluate treatment response Further studies are needed to determine the best approaches to treatment initiation, escalation and maintenance