Treatment of chronic inflammatory neuropathies

This thesis focuses on the efficacy of existing and alternative treatments in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) and explores predictors of treatment response in patients with CIDP treated with corticosteroids.
The efficacy of intravenous immunoglobulin (IVIg) in CIDP and MMN was confirmed in meta-analyses. In CIDP, IVIg efficacy was similar to the efficacy of plasma exchange, prednisolone and intravenous methylprednisolone. Subcutaneous immunoglobulin may be an alternative treatment in patients with MMN needing maintenance IVIg treatment.
In CIDP we compared remission rates with pulsed high-dose dexamethasone treatment with remission rates with standard oral prednisolone. There was no difference in remission rates, but remissions were achieved quicker in the dexamethasone treated patients. Long-term remission was achieved in about a quarter of CIDP patients after one or two courses of pulsed dexamethasone or 8-month daily prednisolone. Surprisingly, almost a quarter of patients deteriorated soon after starting corticosteroid treatment. There were no clinical determinants of poor treatment response, but an electrophysiological profile with predominantly motor involvement with focal demyelination was found significantly more often in patients with early deterioration.
Based on the results of this thesis and recent literature, there is probably enough evidence to conclude that IVIg is superior to steroids in achieving improvement faster in CIDP patients. However, corticosteroids, and especially high dose pulse therapy, are superior to IVIg because much longer relapse-free periods can be induced. In MMN, IVIg remains the only proven initiation treatment.