Peter Špalek
Over the past years we have observed considerable advances in the treatment of autoimmune-mediated neuromuscular disorders. Many immune-modulating therapies are available, including steroids, an increasing number of immunosuppressive drugs and plasmapheresis. Part of this development was the introduction of high-dose polyclonal intravenous immunoglobulins (IVIg). IVIg has become a commonly used therapy as it is well tolerated, easily administered, and is often efficacious with a relatively rapid action. On the basis of randomised, placebo controlled, double-blind clinical trials, IVIg has became a first-line therapy for Guillain-Barré syndrome, multifocal motor neuropathy and some cases of chronic inflammatory demyelinating polyneuropathy. Furthermore, IVIg plays an important role in treating myasthenic crises and exacerbations in myasthenia gravis, and IVIg may play a role in selected chronic treatment-refractory cases of polymyositis, dermatomyositis, Lambert-Eaton myasthenic syndrome and acquired neuromyotonia. Due to excellent safety profile, IVIg has been used very liberally for most diseases with suspected autoimmune pathology or where there is no treatment available. This, and the strict licensing requirements, has led to a shortage in IVIg supply and dramatic increase in costs. Therefore, IVIg should principally be used according to available data from controlled clinical trials. Here, we review the clinical applications and recommendations for the use of IVIg in autoimmune neuromuscular diseases.