The majority of autoimmune
neuromuscular diseases fall into three groups: 1) The autoimmune neuropathies, which include the
acute inflammatory demyelinating polyneuropathy (
Guillain-Barré syndrome), the chronic inflammatory demyelinating
polyneuropathy, the paraproteinemic
polyneuropathies, and the anti-GM1-associated motor neuropathies with conduction block; 2) the
inflammatory myopathies, which include the
dermatomyositis and
polymyositis complex; and 3) the autoimmune neuromuscular junction defects, which include
myasthenia gravis, and the
Lambert-Eaton myasthenic syndrome. Laboratory and clinical evidence suggests that circulating
antibodies or sensitized lymphocytes are operating in the pathogenesis of these conditions. Current
immunotherapies include treatment with
plasmapheresis, high-dose
steroids, or immunosuppressive drugs. Although all of these
therapies are effective in a number of patients and for some period of time, they often result in serious side effects that necessitate their discontinuation. The need for safer and more effective
therapies in the treatment of these conditions prompted the use of high-dose i.v.
immune globulin (
IVIG). A number of small trials and a few reports suggest that
IVIG is safe and effective in the treatment of patients with autoimmune neuropathies,
inflammatory myopathies, and
myasthenia gravis unresponsive to conventional
therapies. We will present current experience with
IVIG in the above-mentioned autoimmune
neuromuscular diseases, and we will stress the need for long-term controlled studies. The possible immunomodulatory action of
IVIG in these conditions will also be discussed.