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.