IVIg in idiopathic autoimmune neuropathies: analysis in the light of the latest results

Abstract

High-dose intravenous immunoglobulin (IVIg) is effective in the treatment of idiopathic autoimmune neuropathies including Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating neuropathy (CIDP) and multifocal motor neuropathy (MMN), representing a useful option or, as in MMN, the gold standard for their treatment. In GBS, two randomised, controlled trials (RCT) showed that IVIg is at least as effective as plasma exchange (PE). IVIg may however be preferred due to its low number of contraindications and complications and the fact that it can be administered at any time, in any department, including patients with contraindications to PE, or in intensive care units. In CIDP, at least four RCTs have demonstrated the efficacy of IVIg in over 60% of CIDP patients, while two additional RCTs have shown a comparable effect to steroids and PE as initial treatment. As with PE, the effects of IVIg usually last a few weeks meaning that the majority of patients require periodic maintenance infusions. The lower cost and easier administration of oral steroids compared to IVIg may be partly compensated by the safer long-term profile of IVIg over steroids. In MMN, almost 80% of patients improve with IVIg, the efficacy of which has been confirmed by four RCTs, making of IVIg the first-choice therapy in MMN, for which steroids and PE are ineffective or even detrimental. Also in these patients, IVIg induces a rapid improvement that usually lasts only a few weeks and has to be maintained with periodic IVIg infusions for long periods of time, if not indefinitely.