Treatment of Autoimmune Neuromuscular Diseases with High-Dose Intravenous Immune Globulin

Abstract

ABSTRACT. The majority of autoimmune neuromuscular RATIONALE FOR USE O F IVIG --diseases fall into three groups: 1 ) The autoimmune neuropathies, which include the acute inflammatory demyelinating polyneuropathy (Guillain-Barri. 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 abovementioned 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. (Pediatr Res 33 (Suppl): S95-S100,1993) Abbreviations IVIG, intravenous immune globulin AChR, acetylcholine receptor CIDP, chronic inflammatory demyelinating polyneuropathy DM, dermatomyositis

Prospective study of children with Guillain-Barre syndrome

“…21,22 Although the precise mechanism of immunomodulation by IVIG is unknown, it probably directly inactivates myelin specific antibodies and indirectly inhibits its production, hastening recovery leading to improvement in nerve conductions. 21 Other mechanisms include solubilization of immune complexes, possible action on natural killer or suppressor cells and immunomodulation by Fc receptor blockade. 21 The halflife of IVIG is usually 3 weeks.…”

“…21 Other mechanisms include solubilization of immune complexes, possible action on natural killer or suppressor cells and immunomodulation by Fc receptor blockade. 21 The halflife of IVIG is usually 3 weeks. 22 In case the disease activity persists beyond the half-life of IVIG, relapse sets in.…”

High‐dose immunoglobulin therapy for Guillain−Barré syndrome in Japanese children

“…Patients with GBS were recruited from 35 centers in Japan. Inclusion criteria for patients were: able to fulfill the criteria for acute GBS 11,12 ; under 15 years of age ; moderate or severe GBS (grade 3, 4, or 5) on the Hughes’ functional grade (FG) scale; and onset of neuropathic symptoms in the prior 4 weeks. Exclusion criteria were: presence of a serious other pre‐existing condition or contraindication to IVIg.…”

Intravenous Immune Serum Globulin (IVIG) Therapy in Patients with Antibody Immune Deficiency

“…83 The expression of ICAM-1 is increased on the endomysial blood vessels and muscle cells, which further facilitates the infiltration of inflammatory cells, mainly CD4 + T cells and some B cells. 83,84 The efficacy of IVIG treatment in adult DM has been demonstrated in a doubleblinded, placebo-controlled study by Dalakas and colleagues. 82,85 Smaller, uncontrolled studies and case reports suggest that IVIG may also be efficacious in juvenile DM.…”