Intravenous immunoglobulin for treatment of neuromuscular disease.

Intravenous immunoglobulin (IVIg) has been widely used in the treatment of autoimmune neuromuscular diseases. Compared to other treatment modalities, such as corticosteroids and chemotherapy for autoimmune disorders, IVIg has relatively few side effects and favorable therapeutic outcomes in certain neuromuscular diseases. There is Class I evidence for IVIg as an initial treatment for patients with Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and multifocal motor neuropathy. It is as effective as plasma exchange in GBS and CIDP. In myasthenia gravis, IVIg is used for myasthenic crisis and exacerbations, though it is also helpful as maintenance therapy, particularly in patients with a suboptimal response or contraindications to prednisone or other immunosuppressive agents. IVIg has been demonstrated to be beneficial in placebo-controlled, double-blind, randomized studies in dermatomyositis and Lambert-Eaton syndrome. IVIg has also been beneficial in select patients with polymyositis and other autoimmune peripheral neuropathies. Clinical trials in amyotrophic lateral sclerosis, inclusion body myositis, and anti-myelin-associated glycoprotein neuropathy have been negative.