Objective: To determine the efficacy of various intravenous immunoglobulin (IVIG) protocols used in the treatment of autoimmune and chronic inflammatory disorders.
Data sources: Literature retrieval was accessed through MEDLINE (November 1984-March 2007) and a search was conducted using the term intravenous immunoglobulin. References cited in the selected articles were also reviewed.
Study selection and data extraction: Inclusion criteria for studies were (1) English language, (2) randomized controlled trials, (3) defined protocols, (4) a minimum of 15 patients, and (5) objective criteria provided to assess clinical outcomes and course.
Data synthesis: The therapeutic efficacy of IVIG therapy is well established, and defined protocols exist for treatment of Kawasaki disease, immune thrombocytopenic purpura, Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and autoimmune mucocutaneous blistering diseases. In the absence of a defined protocol, studies have demonstrated that IVIG therapy is effective in the treatment of myasthenia gravis, dermatomyositis, stiff person syndrome, antineutrophil cytoplasmic antibody positive systemic vasculitides, Graves' ophthalmopathy, and certain forms of systemic lupus erythematosus. It might also be of benefit in some patients with relapsing-remitting multiple sclerosis. The outcomes are variable in these studies. In toxic epidermal necrolysis and Stevens-Johnson syndrome, use of IVIG has dramatically influenced clinical response and reduced mortality.
Conclusions: The cumulative evidence suggests that the clinical outcomes observed are significantly influenced by the use of a defined protocol. There is a need for multicenter trials approved by the Food and Drug Administration to better define the role of IVIG in many disease states. Such studies would be able to establish the indications for use, optimal dose, frequency of infusions, duration of therapy, and need for gradual withdrawal versus sudden cessation. Defined protocols resulting from the study of a large cohort of patients often convince insurance companies to create policies that provide access to IVIG therapy.